Step by Step Echocardiography in Congenital Heart Diseases

Step by Step
Echocardiography in
Congenital Heart Diseases
Step by Step
Echocardiography in
Congenital Heart Diseases
IB Vijayalakshmi
MD, DM, FICC, FIAMS, FIAE, FICP
President, Cardiological Society of India, Karnataka Chapter
Convenor, National Policy for Congenital Heart Disease
Former Senate Member of
Rajiv Gandhi University of Health Sciences
Head of Paediatric Cardiology Department
Children’s Heart Care Centre
Sri Jayadeva Institute of Cardiology
Bangalore, Karnataka
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Step by Step Echocardiography in Congenital Heart Diseases
© 2006, IB Vijayalakshmi
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First Edition: 2006
ISBN 81-8061-743-2
Typeset at JPBMP typesetting unit

Printed at Gopsons Paper Ltd, Noida
To
My parents
Smt & Shri Ishwarappa Gurusiddappa Balekundri,
who are always a source of strength and encouragement,
who have always guided and shown me the
right path in life
Prologue
It gives me great pleasure to write this

Prologue for the book Step by Step
Echocardiography in Congenital Heart
Diseases. This is a “state of the art” book,
which will be of great educational value
to all echocardiographers, as well as
adult and pediatric cardiologists. Dr Vijayalakshmi, who
has authored this book, is Chief of Pediatric Cardiology at
Sri Jayadeva Institute of Cardiology at Bangalore. The book
is divided into eight chapters: Basics and Technical
Background, Normal Heart, Terms Used to Describe
Cardiovascular Segments and Connections, Segmental
Approach to CHD, Acyanotic Congenital Heart Diseases,
Cyanotic Congenital Heart Diseases, Rare Congenital Heart
Diseases, and Foetal Echocardiography. In addition a DVD
ROM has been prepared to complement the material listed
in the book. There is a great need for a book of this type
and Dr Vijayalakshmi’s present work goes a long way in
fulfilling this need.
Navin C Nanda, MD
Professor of Medicine and Director
Heart Station/Echocardiography Laboratories
The University of Alabama at Birmingham
President, International Society of Cardiovascular
Ultrasound
Editor-in-Chief, Echocardiography Journal
Prologue
A precise and complete diagnosis of

congenital heart disease has always
been a very challenging problem.
Prior to the echo-Doppler era, the
physician/cardiologist had to exercise
their clinical skills for evaluation of
this subset of cardiovascular disease.
Although it was useful but in several instances the details
could not be unmasked even after a meticulous clinical
examination by a superb clinical cardiologists. Cardiac
catheterization had to be resorted to in all cases prior to
surgery.
The access to echo-Doppler has enabled the cardiologist
to delineate the pathoanatomy and pathophysiology of
congenital heart disease with great ease and perfection and
a major chunk of this population is now submitted directly
to surgery/catheter interventions bypassing haemo-
dynamic evaluation. The availability of portable echo has
paved the way for evaluation of suspected cases with
congenital heart diseases in far flung areas too, enabling
their timely referral to a tertiary centre.
The intraoperative echocardiography and echocardio-
graphy during catheter interventions has also proved very
gratifying. The key to success in congenital heart disease
is to proceed in a much planned systematic way. The book
x STEP BY STEP ECHOCARDIOGRAPHY IN CHD
entitled Step by Step Echocardiography in Congenital Heart

Diseases by Dr IB Vijayalakshmi is so elegantly written and
illustrated that it will automatically pave the way for a
correct and complete diagnosis of congenital heart disease.
The book will continue to vibrate you from beginning
to end and going through the book is a pleasure. The
author of the book, Dr IB Vijayalakshmi is an unmatched
pediatric cardiologist who has excelled not only in clinical
cardiology but also in echocardiography and catheter
interventions.
I am sure the book will find a permanent place on the
desk of all echocardiographers interested in congenital
heart disease. I congratulate Dr Vijayalakshmi for this
wonderful endeavor and wish the book and her great
success.
PC Manoria
President: CSI, India
Preface
There are many systematic text-

books on Echocardiography but
this book is different. It is designed
for students, general physicians,
sonographers, technicians and
cardiologists as a handbook for a
quick reference and recognition of
both common and rare congenital
heart diseases that one may encounter during one’s daily
practice.
Why is this book necessary? Today ECHO facility is
available in many peripheral centres. Further, ECHO is
reported by technicians and clinicians who are otherwise
good but have less exposure to ECHO in CHD. The eyes
do not see what the mind does not know is very true about
the ECHO in CHDs. As a result, simple ASD can be missed.
Since complete cure is possible in nearly 65 per cent of
CHDs with surgery or with transcatheter interventions,
early detection and referral is very important. In this
direction this book will assist, guide and help detect and
diagnose CHDs. The usual apex up imaging simulates the
anatomy of a patient who is standing on their head and
facing away from the examiner. Therefore apex down
imaging not only displays echocardiographic data in a
more anatomical format, but also allows us to communicate
more effectively with our colleagues. But unfortunately,
xii STEP BY STEP ECHOCARDIOGRAPHY IN CHD
apex down imaging is not familiar to many Echocardio-

graphers in India, hence I have shown both apex up as
well as some apex down images for the benefit of those
who are not familiar with apex down image.
This book will also appeal to general practitioners
wanting to know about the ECHO which is a simple, safe,
cost effective, excellent noninvasive diagnostic tool, that is
easily available for the common man with CHD. I hope the
readers will be richly benefited by this book.
IB Vijayalakshmi
Contents
1. Basics and Technical Background ........................... 1

2. Normal Heart .............................................................. 15
3. Terms Used to Describe Cardiovascular
Segments and Connections ...................................... 19
4. Segmental Approach to CHD .................................. 29
5. Acyanotic Congenital Heart Diseases .................... 45
6. Cyanotic Congenital Heart Diseases ...................... 93
7. Rare Congenital Heart Diseases ........................... 133
8. Foetal Echocardiography ......................................... 171
Index ............................................................................. 199
DVD Contains
Echo pictures and videos of
various Congenital Heart
Diseases
Introduction
Historically, the emergence of echocardiography (ECHO)

is viewed as a milestone in the diagnostic approach to the
Congenital Heart Disease (CHD). In 1924 when ECHO was
not available, Joseph K Perloff said in his gospel of CHD,
“The diagnosis of Congenital Heart Disease represents the
epitome of applied clinical logic when correct inferences
are drawn from accurate observations, diagnosis are made
with gratifying frequency.” Since then the management of
CHD both by surgery and by transcatheter interventions
have made spectacular advancements. Hence there is a
need for precise diagnosis, which can be like a road map
to the cardiac surgeons and cardiologists. Today, ECHO
is a window to the heart and every minute details of the
heart, both structural and functional can be obtained by
ECHO. As the information that one gets from ECHO is so
complete, that the introduction and development in ECHO
and Doppler modalities enable definitive diagnosis of CHD
without cardiac catheterization. For open heart surgery
without invasive investigations, atrial septal defect (ASD),
aortic and pulmonary stenosis, sub aortic membrane, supra
aortic stenosis, cardiac tumours and isolated valve
disorders like Ebstein’s anomaly are ‘classic’ candidates.
Recent experience indicated that ‘selected’ cases of
complete AV canal defect, tetralogy of Fallot, truncus
arteriosus, total anomalous pulmonary venous connection
(TAPVC) and transposition of the great arteries (TGA), may
safely undergo primary repair without cardiac
catheterization in sick and moribund infants.
Chapter 1
Basics and
Technical
Background
2 STEP BY STEP ECHOCARDIOGRAPHY IN CHD
The ECHO gives complete information about the anatomy

and physiology of the heart and great vessels. It can detect
diseases of endocardium, myocardium, pericardium,
valves, anomalies in the arteries and the pulmonary veins.
The ECHO study comprises of two-dimensional ECHO, M-
mode ECHO, Doppler study and colour flow mapping and
tissue harmonics. There is some disagreement and/or
confusion about image orientation, usually centers on the
apical and subcostal transducer positions. Almost all
congenital cardiologists display these images using
American Society of Echocardiography (ASE) options,
apex down format. This approach again displays the heart
as if the examiner is looking at a patient in a classical
anatomic format and is consistent with the conventions
used for parasternal sagittal, horizontal and coronal plane
scans. Therefore apical 4 chamber images taken in a
patient with dextrocardia should be displayed with apex
down and left-sided structures to the right. This consistency
of presentation is vital to understanding the complex
anatomy found in some patients with congenital heart
disease. Secondly, the nonechocardiographer, especially
cardiac surgeon, has difficulty in understanding anatomy
when it is displayed in a non-anatomic format. The usual
apex up imaging simulates the anatomy of a patient who
is standing on their head and facing away from the
examiner. Therefore apex down imaging not only displays
echocardiographic data in a more anatomical format, it also
allows us to communicate more effectively with our
colleagues.
BASICS AND TECHNICAL BACKGROUND 3
2-
2-DD ECHO
In 2-D ECHO, various views like, parasternal long axis
(Figure 1.1A), Parasternal short axis, Apical four chamber
view (Figure 1.1B), subcostal abdominal, subcostal cardiac
view (Figure 1.1C), short axis (Figure 1.1D), suprasternal
views, High parasternal short axis (Figure 1.1E) and ductal
views are studied. Emphasis is placed on certain views that
are particularly rewarding. For example, the subcostal
approaches identify the interatrial septum, atrial septal
defect and the relationships of the atrial and ventricular
septum to the atrioventricular valves. Suprasternal views
are good for examination of the great vessels and the aortic
arch. All views obviously must be utilized. In small
FIGURE 1.1A: ECHO picture in parasternal long axis view

FIGURE 1.1B: Diagrammatic representation and echo

picture in apical four-chamber view
FIGURE 1.1C: ECHO picture in subcostal cardiac view

FIGURE 1.1D: Echo picture in parasternal

short axis view
FIGURE 1.1E: ECHO picture in high

parasternal short axis view
children, lack of attenuation from the rib cage permits

routine imaging with high frequency transducers such as
5 to 12 MHz.
The LV is circular, with the interventricular septum acts
as part of LV and the RV is crescent like. Atrioventricular
concordance with RA to TV to RV and LA to MV to LV.
NORMAL
The aorta looks like a “circle” posteriorly and right
ventricular out flow tract (RVOT), pulmonary valve (PV),
Main pulmonary artery (MPA) appears anteriorly like a
“sausage”.
M-mode is used for timing events within the heart and
for measuring cardiac dimensions. It can also detect
pericardial effusion, and bacterial endocarditis (Figure 1.2).
DOPPLER ULTRASOUND
The Doppler study provides information on velocity. By
convention, velocities in a direction towards the probe (A)
are displayed above the line and away from the probe (B)
are displayed below the line (Figures 1.3 and 1.4).
CONTINUOUS WAVE (CW) DOPPLER

A continuous wave probe consists of two crystals: one trans-
mitting continuously and the other receiving continuously.
The CW Doppler is used to estimate the severity of valve
stenosis or absolute pressure (Figures 1.5A and B).
FIGURE 1.2: M-mode in 12 years old girl with Ebstein’s anomaly

shows dilated RV, increased excursion of the anterior tricuspid
leaflet and delayed closure of the tricuspid valve
FIGURE 1.3: Diagrammatic representation of Doppler effect

FIGURE 1.4: Diagrammatic representation of velocity display,

in a direction towards the probe (A) are displayed above the
line and away from the probe (B) are displayed below the line
FIGURE 1.5A: Diagrammatic representation of CW Doppler

FIGURE 1.5B: Suprasternal CW Doppler, 14 years old boy with

bicuspid aortic valve with critical aortic stenosis shows 232
mm of Hg gradient across AV
The shape of the wave form and the density of the

signal give an approximate measure of the severity of
regurgitation (Figure 1.6) .
PULSE DOPPLER
It can record velocity information from a relatively small
region. For example, in a patient with mild aortic coarc-
tation, the pulsed sample volume is moved from the aortic
arch to the site of the coarctation and into the descending
aorta. A progressive increase in flow velocity can be seen
(Figures 1.7A to C).
FIGURE 1.6: CW Doppler in a case of TOF with absent pul-

monary valve, shows both pulmonary stenosis and pulmonary
regurgitation
FIGURE 1.7A: Diagrammatic representation of

pulse wave Doppler
FIGURE 1.7B: PW Doppler of inferior vena cava
FIGURE 1.7C: PW Doppler in descending aorta in CoA

COLOUR FLOW MAPPING

The colour flow system calculates mean blood velocity and
direction of flow at multiple points. Hence colour flow
mapping is used as a screening tool for abnormal blood
flow with which even as small as 2 mm VSD can be
detected (Figures 1.8A to C). Such small shunts can go
undetected without colour flow mapping and develop
bacterial endocarditis if not put on bacterial endocarditis
prophylaxis. It also can give a semiquantitative estimate
of the severity of regurgitation.
FIGURE 1.8A: Small VSD seen in short axis view,

below the pulmonary valve
FIGURE 1.8B: Colour comparison in short axis view

shows a small subpulmonic VSD by colour Doppler
FIGURE 1.8C: Colour comparison in parasternal

long axis shows a small VSD
Chapter 2
Normal Heart
It is very pertinent to know the normal heart to detect the

abnormality in the heart. The normal heart consist of four
chambers: (a) right atrium—RA, (b) right ventricle—RV, (c)
left atrium—LA, (d) left ventricle—LV, the two intact
septum—interatrial septum (IAS) and interventricular
septum (IVS). The characteristics of right atrium (RA) are
inferior vena cava (IVC) and superior vena cava (SVC) drain
into it. It has a wide spade-like RA appendage. The right
ventricle (RV) is identified by its characteristic morphology,
triangular shape, coarse trabeculations, three papillary
muscles, moderator band and the tricuspid valve which
FIGURE 2.1: Apical four chamber view shows interventricular

septum with fine trabeculations smooth on LV side, coarse
trabeculations on RV side with moderator band and the
tricuspid valve which is attached slightly lower than the mitral
valve indicates RV
NORMAL HEART 17
FIGURE 2.2: Parasternal short axis shows long fingerlike

LA appendage with narrow neck
FIGURE 2.3: Parasternal long axis view shows mitral to

aortic continuity
is attached slightly lower than the mitral valve (Figure

2.1).The left atrium (LA) is recognized by all the four
pulmonary veins draining into it and long fingerlike LA
appendage with narrow neck (Figure 2.2). The left ventricle
(LV) is elliptical in shape, has smooth fine trabeculations,
two papillary muscles and Mitral valve is attached at a
slightly higher level. In the parasternal long axis the mitral
to aortic continuity is seen in the normal heart (Figure 2.3).
Chapter 3
Terms Used to
Describe
Cardiovascular
Segments and
Connections
It is very essential to know the terms used to describe the

cardiovascular segments and understand the precise
meaning of these terms, so that the anomalies can be
understood and reported appropriately.
SEGMENT
A section of the cardiovascular system (i.e. great veins or
the ventricles).
CONNECTIONS
The junction between two cardiovascular segments.
OVER-RIDING
A function of a valve annulus and a ventricular septal
defect. The term describes an annulus that crosses the
plane of a VSD and is therefore “over”more than one
ventricle. Any of the cardiac valves can potentially be
described as over-riding. Most often it is the aorta which
over-rides as in case of tetralogy of Fallot (Figure 3.1) or
Double outlet RV (DORV) as in Figure 3.2.
STRADDLING
A function of the chordae tendineae of an atrioventricular
valve and a VSD. The term describes chordae which cross
a VSD and have their myocardial attachments within the
opposite ventricle, as in the case of complete AV canal
defect. This can create difficulties for the surgeon trying to
close a VSD. The ECHO can delineate straddling better
than angiogram (Figure 3.3).
TERMS USED TO DESCRIBE CARDIOVASCULAR SEGMENTS 21
Figure 3.1: Parasternal long axis view shows large

subaortic VSD with over-riding of aorta
Figure 3.2: Subxyphoid echo images, Large subaortic VSD,

more than 75% of aorta arising from dilated right ventricle,
larger than LV
FIGURE 3.3: Diagrammatic representation of types of

straddling and over-riding
Point to remember: An atrioventricular valve can both

straddle and over-ride, but one should remember that a
semi-lunar valve cannot straddle since it does not have
chordae.
CONCORDANT
This refers to a normal connection between segments. For
example, when the RA connects to the RV, the connection
is described as concordant (Figure 3.4).
FIGURE 3.4: Diagrammatic representation of concordant

and discordant connections of chambers
DISCORDANT
This refers to the opposite of the normal connection. For
example, when the LV connects to the pulmonary artery
and RV connects to aorta as in case of ventricular inversion
in corrected transposition of great vessels (CTGV) the
connection is discordant (Figure 3.5).
UNIVENTRICULAR HEART
A special form of atrioventricular connection in which both
atria are committed to only one functional ventricle as in
case of single ventricle or tricuspid atresia or mitral atresia
(Figures 3.6A to C).
FIGURE 3.5: Apical four-chamber view shows LA with pulmonary

veins, connected to trabeculated RV through tricuspid valve
attached at lower level than mitral valve, in a 6 years old boy
of CTGV with ASD, VSD, PH
FIGURE 3.6A: Apex down image shows both left atrium and
right atrium connected to single ventricle (double inlet SV)
FIGURE 3.6B: Apical four-chamber view shows a dense band

of echoes at the site normally occupied by the tricuspid valve
FIGURE 3.6C: Apex down image shows mitral valve atresia,

hence both the atria draining into single ventricle
TRANSPOSITION
The prefix “trans-” means across, this means “across the
septum.” Therefore transposition refers to the semilunar
valves only and occurs when the great arteries are on the
opposite side of the ventricular septum relative to normal,
e.g. aorta on the morphologically right ventricular side of
the septum and pulmonary artery on the morphologically
LV side (Figures 3.7A and B).
FIGURE 3.7A: Aorta is seen arising from

trabeculated morphological RV
MALPOSITION
This term also refers to the semilunar valves and great
arteries. It is applied to any position or connection of the
great arteries to the ventricles that is not normal and not
transposition (Figure 3.8). For example, the great arteries
FIGURE 3.7B: Branching pulmonary artery is seen arising

from smooth walled ellipsoid morphological LV
FIGURE 3.8: Parasternal long axis view shows aortic root and
pulmonary trunk run in parallel courses and arise from RV
are always malpositioned in double outlet right ventricle

(DORV) and the term transposition should never be used
to a double outlet right ventricle, because the great arteries
are on the same side of the septum and not “across” it as
in TGA.
Chapter 4
Segmental
Approach to CHD
Two-dimensional echocardiography is ideally suited

for the evaluation of congenital heart diseases (CHD)
because of its ability to visualize cross-sections of complex
cardiac anatomic structures. Visualizing heart walls,
chambers, and valves, is, in many ways, superior to
angiography for revealing complex spatial morphologic
information. All CHDs are potentially complex. Such a
statement should not be frightening, as it only reflects the
fact that the presence of one lesion increases the possibility
of another lesion. Hence to obtain a complete under-
standing of even the most straight forward congenital
cardiac malformations a standardized method of assess-
ment is important. In the setting of very complicated
cardiovascular anomalies, like the heterotaxy syndromes,
it becomes essential.
The method that is most useful and most widely used
is the “segmental approach.” This approach recognises
that situs (position) is important to the understanding of
the anomaly and considers the cardiovascular system to
be composed of four major segments and the connections
between them can be assessed, as it is anatomy that is
demonstrated. Then their connections and relations are
defined. Later the associated lesions are evaluated at each
level and haemodynamics and the function of the
structures within each segment are assessed. This
segmental approach makes the understanding and
diagnosis of even the most complex cardiac lesions very
easy. For example, start with the situs in subcostal and
abdominal view (Figure 4.1).
SEGMENTAL APPROACH TO CHD 31
FIGURE 4.1: Situs solitus—aorta(red) to the left and

inferior vena cava (blue) to the right, confirmed with the
Doppler study
SITUS OR SIDEDNESS
This concept applies to structures or organ systems that
are not bilaterally symmetric. It describes the position of
the organs in the system and usually has three possible
arrangements:
Normal or solitus, inverted or inversus (mirror image
of normal), and ambiguous (something else).
It is important to know the visceral situs in congenital
heart disease (Figure 4.2A).
Solitus or Normal
Liver and caecum to the right, stomach and spleen to the
left. The aorta to the left and IVC to the right.
FIGURE 4.2A: Diagrammatic representation of visceral situs
Inversus or Inverted
Liver and caecum to left, stomach and spleen to right. The
aorta to right and IVC to the left.
Ambiguous
Any other patterns. Frequently the liver is bilateral and there
is intestinal malrotation. Gastric position is variable (the
spleen, when present, is always posterior to the stomach).
FIGURE 4.2B Diagrammatic representation of normal,

mirror-image, right isomerism, left isomerism of atria
ATRIAL SITUS OR SIDEDNESS

It can also be referred to as cardiac situs or sidedness and
is determined by the position of the morphologic right and
left atria (Figure 4.2B).
Solitus or Normal
Morphologic LA is posterior and to the left of RA.
Inversus or Inverted
Morphologic LA is posterior and to the right of RA.
Ambiguous
Confident assignment of morphologic LA and RA cannot
be made, usually in common atrium with multiple venous
connection abnormalities. Sometimes this term is used to

describe the atrial situs found in heterotaxy syndromes
(atrial isomerism).
The most reliable determinant of atrial morphology is
the atrial septum. The thin valve of the Oval Fossa (labeled
VOF in the Figure 4.2B) is always positioned to the same
side as the left atrium. The thicker, superior limbus of the
atrial septum (labeled limb in the Figure 4.2B) is associated
with the right atrium (Figure 4.3).
FIGURE 4.3: The IAS profiled subcostal views shows thin

valve of the oval fossa in the left atrium. The thicker, superior
limbus of the atrial septum is with the right atrium
The atrium to which the coronary sinus and hepatic

veins connect will usually be the right atrium. The left
atrium will not receive any of these vessels, unless the
coronary sinus is unroofed or absent. Pulmonary venous
connections do not reliably predict atrial type. The shape
of the atrial appendage has often been used to assign atrial
situs (RAA looks broad and triangular and the LAA is thin
and finger-like). However, the appendages are unreliable
because in patients with left atrial dilation, the appendage
can also enlarge and change its shape.
CARDIAC ORIENTATION
It represents the orientation of the base to apex axis of the
heart, not its position within the mediastinum (although
the two usually go together).
Cardiac position—represents the gross position of “most”
of the heart relative to the midline.
Levocardia—base to apex axis is “pointed” from upper

right to lower left.
Levoposition—most of the cardiac mass is to the left of

midline.
Dextrocardia—base to apex axis is “pointed” from the
upper left to the lower right.
Dextroposition—most of the cardiac mass is the right of

midline.
Mesocardia—base to apex axis is “pointed” nearly directly

from superior to inferior and usually is in the midline.
Mesoposition—the heart is evenly distributed around the
midline. Then identify the cardiac position in subcostal
cardiac view by identifying the ventricles and the apex.
The anatomy, function and physiology (i.e. shunts,
stenoses, etc.) of each segment should be described. In
addition, and sometimes most importantly, the connection
between segments must be described. Connections can be
concordant (normal) or discordant (opposite of normal, i.e.
LA connected to a morphologic RV in corrected trans-
position of great arteries (CTGV). In some situations other
designations are needed for connections that are not
normal but are also not the opposite of normal (i.e. DORV
and univentricular AV connection).
Venous segment—includes both the systemic, pulmonary

and coronary veins. The great veins are usually considered
to include:
• Systemic—SVC, IVC and hepatic veins.

• Coronary—the coronary sinus.
• Pulmonary—the 5 major pulmonary veins (one from
each lobe of the lungs).
• Veno-atrial connection—how and to what the great
veins connect.
ATRIAL MORPHOLOGY AND ATRIAL SEGMENT

The atrial segment includes the RA, LA, atrial septum and
the atrial appendages and any anomalies. In order to
distinguish a morphologic RA from a morphologic LA a
variety of anatomic landmarks can be used. The most

reliable is the anatomy of the atrial septum. The thicker
limbus of the foramen ovale is always located on the same
side of the septum as the morphologic RA. Conversely, the
thin valve of the foramen ovale is always on the same side
as the morphologic LA. Unfortunately, patients with large
atrial septal defects do not have this landmark. In others
with suboptimal image quality, one may not be able to
resolve the septum well enough to make this distinction.
In these situations, we rely on the connection of the
suprahepatic portion of the IVC, the size and shape of the
atrial appendages, and the pattern of the atrial wall
(muscular or not) to determine RA or LA. The suprahepatic
portion of the IVC (upstream from the entrance of the
hepatic veins) will almost invariably connect to the
FIGURE 4.4: Subcostal abdominal view images suprahepatic

portion of the IVC (upstream from the entrance of the hepatic
veins) connecting to the morphologic right atrium
morphologic RA (Figure 4.4). The only time this finding

becomes difficult to interpret is when the IVC connects to
one side of an atrium and an independently connecting
hepatic vein connects to the other. In our experience this
most often represents atrial situs ambiguous with bilateral
morphologic RAs.
ATRIAL-VENTRICULAR CONNECTION
The AV valves assessment includes not only the con-
nection, but also the function of the valves (stenosis,
regurgitation, straddling, AV discordance, univentricular
connection, Ebstein’s anomaly—the list is nearly endless).
Figures 4.5A and B for types of connections and straddling
that can occur.
FIGURE 4.5A: Diagrammatic representation of atrioventricular

and ventriculoarterial discordance. The great arteries simulta-
neously run parallel to each other (1) and do not cross each
other as in normal ventriculoarterial connections (2)
FIGURE 4.5B: Apex down image with colour Doppler

shows ventriculoarterial concordance
INTERNAL CARDIAC CRUX IN CONCORDANT AND

DISCORDANT AV CONNECTION
The morphologically tricuspid valve is always attached to
the ventricular septum in a slightly lower (more apical
position), than the septal attachment of the mitral valve at
the internal crux. Other features differentiating the
tricuspid from the mitral valve: tricuspid valves have .
multiple septal attachments, mitral valves do not attach to
the septum, unless they have a cleft. Mitral valves have
large well-developed papillary muscles (usually 2),
tricuspid valves have many small papillary muscles
scattered throughout the apex of the RV.
Point to Remember
The AV valves and ventricles go together. So, if you know
that a valve has tricuspid morphology, then the ventricle
it connects to will be a morphologically RV, even if it is
the left-sided ventricle. This is the best way to determine
ventricular morphology, even though it has to do with the
valves.
Normally mitral to aortic continuity is seen. In TGA
there is ventriculoarterial discordance. The pulmonary
artery arises from LV and aorta arises from RV. In
Transposition of great arteries (TGA), the great arteries
typically appear as “double circles”( owl’s eyes) and aorta
is anterior and to the right of the main pulmonary artery
(Figures 4.6 and 4.7A and B).
FIGURE 4.6: Parasternal long axis view shows

mitral to aortic discontinuity in TGA
FIGURES 4.7A and B: In TGA, the great arteries typically appear

as “double circles” (owl’s eyes) and aorta is anterior and to
the right of the main pulmonary artery
FIGURES 4.8A: Apical four-chamber view shows the

RV hypertrophy and IVS is seen acting as part of
RV in a case of TGA as RV is pumping blood into
aorta
FIGURES 4.8B: Modified view shows aorta arising

from RV, indicating ventriculoarterial discordance
Normally IVS acts as a part of LV. But since IVS acts

as a part of ventricle which pumps blood into the systemic
circulation, in TGA RV hypertrophy (RVH) is there in
absence of Pulmonary stenosis (PS) or pulmonary hyper-
tension (PH) (Figures 4.8A and B).
Points to Remember
Usually the artery that branches is pulmonary artery.
MPA branches out into right and left pulmonary artery.
The artery that arches and gives rise to cephalic vessels
is aorta.
The anterior aortic root and posterior pulmonary trunk
when visualized simultaneously run parallel to each other
and do not cross each other as in normal heart.
IMPORTANCE OF SEGMENTAL APPROACH TO DIAGNOSIS OF

RARE COMBINATION OF COMPLEX CONGENITAL ANOMALIES
Master S 18 months male child presented with history of
repeated respiratory infection, breath holding spells,
excessive sweating, feeding difficulties. O/E grade III
malnourishment, weight 6.5 kg (Expected wt—12 kg) mild
cyanosis. BP 96/30 mm of Hg. First admission was at the
age of 3 months. Detailed Echo and Doppler evaluation
by segmental approach revealed congenital cyanotic heart
disease, situs solitus, ASD, Cor-triatriatum , TAPVC , PDA
with PH. For the understanding of congenital cardiac
lesions the ”segmental approach” is recommended. This
means that one identifies echocardiographically each
cardiac segment independently, i.e atria, ventricles, great

arteries and veins and then define their connections and
relations methodically. The morphological right atrium is
identified by the entry of inferior and superior caval veins
and the characteristic appearance of the appendage (broad
and blunt). The left atrium is identified by the narrow and
curved appendage. Entry of pulmonary veins to the left
atrium is taken as a criteria for identification, but is not
constant as in case of TAPVC. As in this case, they were
draining into superior vena cava. One should not be
content with identifying just ASD, but look for other
associated lesions. Otherwise rare anomalies like cor-
triatriatum will be missed.
Chapter 5
Acyanotic
Congenital Heart
Diseases
The acyanotic CHDs can be grouped into:

1. Left to right shunts
2. Non-shunt lesions
ATRIAL SEPTAL DEFECT

Among the L to R shunts the Atrial septal defect (ASD)
is the most common CHD (30%). Defects in the atrial
septum are traditionally divided into four types according
to their location: ostium secundum, primum, sinus venosus
and coronary sinus defects.
The Echo visualizes the atrial septal defect and
establishes its location, number as well as physiologic
consequences . The subcostal view generally permits the
most reliable and comprehensive picture as the ultrasonic
beam is perpendicular to the atrial septum in this view
(Figure 5.1). In the apical four chamber view, the ostium
secondum ASD is represented by echo-free space in the mid
portion of the septum with the bright echogenic tips ( ‘T’
artifact). The colour Doppler confirms the diagnosis (Figure
5.2). The left to right shunt in colour Doppler is seen during
midsystole, early diastole (largest peak in late systole). The
sinus venosus defect of the superior vena caval type lies
near the orifice of the superior vena and RA junction,
whereas a sinus venosus defect of the inferior vena caval
type lies just beyond the rim of the IVC. An unroofed
coronary sinus is identified by the dilated sinus and a
partition defect between the coronary sinus and left atrium.
A dilated, hyperkinetic right ventricle and paradoxical
ventricular septal motion are visualized in parasternal
short, long axis and M-mode.
ACYANOTIC CONGENITAL HEART DISEASES 47
FIGURE 5.1: Subcostal view shows the whole

length of IAS and fossa ovalis
FIGURE 5.2: Apex down image in apical four-chamber view

showing ostium secundum ASD with good superior and
inferior rims. The colour Doppler shows left to right shunt
FIGURE 5.3: Apical four-chamber view shows RA, RV dilated

intact but interatrial septum appears intact in sinus venosus
ASD
But in sinus venosus or unroofed coronary sinus type

of ASD, the interatrial septum (IAS) appears intact in
apical four-chamber view (Figure 5.3). The only clue is the
right ventricle and right atrium are enlarged significantly
because of the increased volume overload due to significant
left to right shunt and on M-mode there is a paradoxical
septal movement and RV dimension is considerably
increased (Figure 5.4).
Sinus Venosus Defects

Sinus venosus defects are surprisingly difficult to detect
as they are located superiorly on the interatrial septum
near its junction with the superior vena cave. Traditional
FIGURE 5.4: M-mode shows paradoxical septal movement

and considerably increased RV dimension
subcostal views are frequently unrewarding and other non-

conventional views must be attempted as in Figure 5.5. It
is important to note the type of ASD, as each type is
associated with other anomalies and may require different
approaches of management. For example, ostium primum
ASD (Partial AV canal defect) is associated with cleft
mitral with mitral regurgitation or inlet VSD, which
requires repair and sinus venosus ASD is associated with
Partial anomalous pulmonary venous connection (PAPVC)
which requires rerouting (Figure 5.6).
Normally, the inter atrial septum is relatively fixed in
position and moves passively with the movement of the
entire heart. Occasionally, a hypermobile septum may be
seen as. an aneurysm of the interatrial septum. In this
FIGURE 5.5: The apex down image shows high sinus

venosus type ASD
FIGURE 5.6: Modified view with colour comparison shows high

ASD with right pulmonary vein draining into right atrium
setting the septum is usually thin and may be fenestrated,

resulting in variable degrees of interatrial flow. In fact,
when such a finding is noted by echo, the majority of
patients show small degrees of interatrial shunting by
saline microbubbles techniques (Contrast ECHO). The
degree of shunting is rarely hemodynamically significant.
It should be noted, however, that recent evidence has
indicated some link between septal aneurysm and the
presence of embolic stroke in older age population (Figure
5.7A).The transthoracic echo (TTE) and sometimes trans-
esophageal echo play an important role in detecting septal
aneurysm, rims around the defect (Figure 5.7B) and
suitability for device closure. Nowadays transesophageal
echo (TEE) and intracardiac ECHO are being used and
FIGURE 5.7A: Septal aneurysm bulging into RA

FIGURE 5.7B: TEE shows deficient aortic rim, which

makes the device closure difficult
FIGURE 5.7C: Subcostal abdominal view shows Amplatzer

septal occluder with normal flow in IVC
play a major role in balloon sizing of ASD and positioning

of AMPLATZER device during the transcatheter procedure
(Figure 5.7C). The echo is also used in the post-surgical
period.
FIGURE 5.7D: In the parasternal long axis view the dilated

coronary sinus is visualized in a case of persistent left superior
vena cava in CTGV, ASD, VSD, PS
The coronary sinus (CS) is very well visualized on Echo

when it is dilated (Figure 5.7D), either due to intracardiac
TAPVC, persistent left superior vena cava (LSVC) draining
into it or coronary AV fistula.In apical four-chamber view
the dilated coronary sinus gives rise to echo drop out
(Figure 5.8A) which should not be mistaken for ostium
primum ASD.The ostium primum ASD or incomplete AV
canal defect has both the AV valves at the same level and
associated with cleft mitral valve.When there is a doubt
regarding the diagnosis, one should carefully look for the
level of attachment of AV valves (in apical 4-chamber view)
and pulmonary vein connection, in parasternal long axis
view also (Figure 5.8B).
At times ASD is not visualized, but RA, RV, PA are
dilated. In order to distinguish primary pulmonary artery
FIGURE 5.8A: The dilated RA, RV, coronary sinus and small
LA, LV, with no pulmonary veins draining into LA indicate
intracardiac TAPVC
FIGURE 5.8B: Parasternal long axis view shows dilated RV

compressing LV. All the four pulmonary veins are seen draining
into dilated coronary sinus
FIGURE 5.9: Contrast echo shows the microbubbles

enter LA from the patent foramen ovale
hypertension (PPH) from high ASD, contrast echo is done.

If it is sinus venosus ASD, the microbubbles enter LA from
SVC and RA junction, but if it is PPH then the microbubbles
enter LA from the patent foramen ovale and at times the
bubbles appear in LA only on Valsalva maneuver (Figure
5.9). If the microbubbles enter LA after 3 to 4 cardiac cycles
it indicates pulmonary AV fistula, in whom the cardia is
normal despite the patient having clubbing and cyanosis.
In children the windows in subcostal and abdominal
view are very good obviating the need for transesophageal
Echo (TEE).
Point to Remember
ASDs are best viewed from subcostal view. Dilated
coronary sinus should not be mistaken for ostium primum
ASD or the IVC type of sinus venosus ASD.
Special views must be tried to visualize sinus venosus
type of ASD when unexplained RA, RV dilatation are seen
and associated PAPVC should not be missed.
VENTRICULAR SEPTAL DEFECT (VSD)

The ventricular septal defect is the second most common
CHD accounting for 20 per cent of all the left to right
shunts. The incidence is 2.5 per 1000 live births. The VSD
can occur in any of the four components of the inter-
ventricular septum (IVS), namely the membranous, outlet
septum, inlet septum, trabecular septum. The VSD in
membranous septum is located directly beneath the aortic
valve, hence called as ‘subaortic’ (Figure 5.10). This is the
FIGURE 5.10: Apex down image shows

large subaortic VSD
most common type of VSD (about 80%). It is also called as

‘perimembranous’ as it may also extend into adjacent
portions of the septum. Trabecular septal defects (5-20%),
also called as ‘muscular’ VSD vary from small to large and
from single (Figure 5.11A) to multiple. Sometimes the
septum may be transformed into honeycombed or sieve like
fenestration called”swiss cheese” type of defects burrow
through the muscular septum in a spiral or oblique fashion
(Figure 5.11B). The multiple small muscular defects have
the same net functional effect as a single large communi-
cation Multiple VSDs. The defect in inlet portion of septum
are uncommon (8%), are called as “Inlet VSD” or canal or
inlet VSD, typically large, laying beneath both atrio-
ventricular valves but primarily under the septal leaflet of
FIGURE 5.11A: Apex down image shows large single mid-

muscular VSD
FIGURE 5.11B: Apex down image shows multiple small Swiss

cheese defects burrow through the muscular septum in a
spiral or oblique fashion
the tricuspid valve. The MV and TV are attached to the crux

at the same level and best seen in apical 4-chamber view.
Often seen in patients with Down’s syndrome.
The VSD in outlet septum are beneath both the aorta
and pulmonary artery. The right coronary cusps or non-
coronary cusp of the aorta or both may prolapse causing
aortic regurgitation.
2-D Echo along with Doppler study is the best diag-
nostic means of establishing the location of VSD. As the
septum is a curved structure, varieties of views are
required. Multiple rotational views improve the diagnostic
yield .The echo can detect as small as 2 mm VSD (Figure
5.12). Some times bright images that originate from the
margins of the defect (‘T’ artifact) can indicate the presence
of true defect. But the colour Doppler is the most reliable
means to distinguish the presence of a true defect from false
FIGURE 5.12: Colour compare in short axis shows 2 mm

VSD just below the pulmonary valve (subpulmonic VSD)
FIGURE 5.13: Parasternal long axis view shows bright images

at the margins of the defect (‘T’ artifact) the colour Doppler
confirms the defect
artifact (Figure 5.13). The colour Doppler is very important
because without it many small VSDs will be missed.
The ventricular septal aneurysms that occur often tend

to reduce the size of the defect or close the perimembranous
VSD spontaneously. The echo can detect the septal
aneurysms accurately (Figure 5.14A). The colour Doppler
and CW Doppler enable to assess the size of the VSD
(Figures 5.14B and C). The real-time image can show the
systolic bulging of the septal aneurysm into the right
ventricular out flow tract (RVOT) and diastolic realignment
with the septum.
In short-axis view of a subarterial outlet defect entering
the right ventricle just below the pulmonic valve is sub
pulmonic. During surgery, such a defect would be very
difficult to approach through the tricuspid valve as it is
far away for proper closure. In addition, these types of
FIGURE 5.14A: Apical four-chamber view shows large peri-

membranous VSD with septal aneurysm bulging into RV
FIGURE 5.14B: Apical four-chamber view with colour

Doppler shows a small VSD
FIGURE 5.14C: The CW Doppler shows the trans-VSD gradient

of 104 mm of Hg indicating small VSD in 4 years old girl
defects may, over the time, allow for prolapse of the right
coronary cusp of the aortic valve into the defect resulting
in aortic insufficiency (Figures 5.15A and B). So what
could have been a simple VSD closure will require either
aortic valve repair or rarely valve replacement at a
considerable cost and morbidity. Hence it is very important
to do the echo at a regular interval in small VSDs to detect
the aortic regurgitation in time.
Inlet defects are visualized in a subcostal or apical four
chamber view. These windows also serve to identify
straddling or overriding. It is important to measure the size
of VSD and the size of Aorta. Any VSD that is approxi-
mately equal to or greater than the aortic valve orifice or
> 1.0 cm2/m2, with virtually no resistance to the flow of
blood is considered as a large VSD. Such a large VSD is
FIGURE 5.15A: Parasternal long axis view clearly shows

the prolapse of the aortic cusp
FIGURE 5.15B: Parasternal long axis view with colour

Doppler shows severe aortic regurgitation
unlikely to close. In fact such a patient can develop

pulmonary hypertension sooner or later, leading to
bidirectional or right to left shunt (Figures 5.16A and B).
The gradient across the VSD enables to calculate the
presence or absence of pulmonary hypertension. If the
trans- VSD gradient is more than 64 mm of Hg, it means
small VSD. The cuff BP minus the gradient across the VSD
is the RV and the PA pressure (in absence of pulmonary
stenosis). For example, if the systolic blood pressure of the
child is 90 mm of Hg and the trans VSD gradient is 20 mm
of Hg, then 90 minus 20 is 70 mm of Hg and that is the
RV and pulmonary artery pressure. This indicates that
pulmonary artery pressure is more than 2/3rd of systemic
pressure hence needs early surgery. If the gradient across
VSD is less than 10 mm of Hg, it means the systolic
FIGURE 5.16A: Apical four-chamber view shows large VSD

and large ASD with dilated LV
FIGURE 5.16B: Apical four-chamber view with colour

Doppler shows large VSD with bidirectional shunt
FIGURE 5.17: Parasternal long axis view shows bacterial

endocarditis, with vegetation closing the VSD
pressures in both ventricles, aorta and pulmonary artery

are essentially same. Such a child will be in failure requires
emergency medical and surgical management. The
proportion of blood going into two circulations is directly
governed by relative resistance of two vascular beds,
resulting in bidirectional shunt. In small VSD with normal
PVR, echo shows trans-VSD gradient > 64 mm Hg as VSD
< 0.5 cm2/m2 offers large resistance to flow, has normal
PA pressure and the L to R shunt is < 1.5 l/min, hence
imposes little burden. But such a small VSD with high
velocity jet across the defect have the danger of aortic valve
prolapse due to Venturi effect or develop bacterial
endocarditis, leading to vegetation closing the defect rarely
(Figure 5.17).
Today the role of echo extends beyond the diagnosis
of size, number of VSDs and haemodynamics. Infact it is
extensively used during the non surgical and surgical

closure of VSD and also for follow-up and assessing the
residual shunt after surgery (Figures 5.18A to C).
FIGURE 5.18A: Apical four-chamber view shows the

Amplatzer septal occluder in situ
FIGURE 5.18B: Apical four-chamber view with the colour

Doppler shows, device in situ, no residual shunt, no mitral/
tricuspid/aortic regurgitation
FIGURE 5.18C: Echo with colour Doppler shows residual

shunt with echogenic surgical patch used for closing the VSD
CORRECTED TRANSPOSITION OF GREAT VESSELS (CTGV)

CTGV refers to a malformation in which ventricles are
inverted (LV to the right and RV to the left) therefore, atrial
and ventricular morphologies are discordant, and hence
ventricle is discordant with the great artery that arises from
it. The double discordance: atrioventricular and ventriculo-
arterial. Thus serves to counteract or “correct” the physio-
logic abnormality. Accordingly, desaturated blood from a
morphologic right atrium reaches the pulmonary artery, but
through the mitral valve and morphological LV and
oxygenated blood from LA reaches the aorta, but through
the tricuspid valve and morphologic right ventricle. That
means the blood flows from RA →LV→PA and
LA→RV→AO. The 2-D Echo establishes atrioventricular
and ventriculoarterial discordances and allows recognition
of associated lesions like VSD, pulmonary stenosis, etc.

ECHO criteria for determining ventricular chamber and AV
valve morphology include the relative levels of attachments
of each AV valve to the ventricular septum, configuration
of AV valve leaflet (MV- bileaflet or TV-trileaflet), the
papillary muscle arrangement, the presence or absence of
chordal attachments to the inlet septum (tricuspid valve)
or ventricular free valve (mitral valve), the type of
ventricular trabeculations (Figure 5.19).
FIGURE 5.19: Apical four-chamber view reveals LA with

pulmonary veins connected to morphological RV (triangular in
shape with moderator band) through the tricuspid valve at a
lower level on the left side, the RA draining into ellipsoid
morphological LV with mitral valve on right side, indicating,
atrioventricular discodance.The VSD with septal aneurysm is
also seen
PATENT DUCTUS ARTERIOSUS (PDA)

The patent ductus arteriosus represnts abnormal persis-
tence of a normal foetal vascular channel between the
pulmonary artery and aorta. In the past, patients with
continuous murmur were taken up for surgical ligation and
the chest used to be closed on not finding the PDA on the
operation table. Today echo can detect and differentiate
PDA, aorto-pulmonary calaterals, AP-window, Coronary
AV fistula and RSOV.The catheterization is required only
to know the pulmonary artery pressure and pulmonary
vascular resistance and close the PDA by coils or
Amplatzer duct occluder.
FIGURE 5.20: Parasternal short axis at the base of the heart

shows the aorta (as a circle)identified from left coronary artery
(CA) arising from it, Dilated main pulmonary artery (MPA),
branching into right pulmonary artery (RPA), left pulmonary
artery and the PDA (shown by an arrow) entering the LPA
2-D echo along with colour and CW Doppler study

establishes ductal morphology, patency, size, and shunt.
The PDA is best visualized in the parasternal short axis
at the base of the heart and the colour Doppler shows
turbulence in pulmonary artery.CW Doppler shows
continuous wave signals (Figures 5.20; 5.21A and B).
The high (Ductal view) and suprasternal notch view
can avoid superimposition of ductus and the aortic
isthmus. This is the best view to measure the narrow
pulmonary end, wide aortic end (Ampulla), the length of
the PDA.Colour Doppler shows flow from ampulla to
FIGURE 5.21A: Parasternal short axis at the base of the

heart with the colour Doppler shows PDA
FIGURE 5.21B: The CW Doppler shows continuous signals
narrow PDA and PA (Figure 5.22A). In contrast, in large

tubular PDA with pulmonary hypertension there is a
laminar flow from aorta to PDA and PA (Figure 5.22B). It
is important to note that many a times PDA with severe
pulmonary hypertension is missed, because there is no
turbulence on colour Doppler.
The echocardiography with colour and CW Doppler
not only establishes the diagnosis of PDA, it also enables
to detect the associate lesions like coarctation of aorta
(COA). It is impotant to note whether the COA is before
the duct (Pre-ductal) or after the duct (post-ductal). Because
in many centres the child with PDA is sent for surgical
multiple ligation with the echo confirmation. It is
disastrous if the post ductal COA is missed and the patient
is sent for surgery. Similarly preductal COA is known to
FIGURE 5.22A: High ductal view with colour Doppler shows

flow from ampulla to narrow PDA and PA
FIGURE 5.22B: Parasternal short axis at the base of the heart

with the colour Doppler in a 4-month old 4 kg infant shows
large tubular PDA with laminar flow, dilated PA with pulmonary
regurgitation
surgeon prior, the plan for surgery is altered. The Doppler

signals vary from aorta to descending aorta to PDA (Figures
5.23A and B).
To-day echo plays a major role in detecting the position
of coils or Device used for closure and also to detect any
residual shunt and obstruction to left pulmonary artery or
aorta (Figures 5.24A and B) and follow-up of the patient
to know whether residulal shunt has disappeared or not.
The bacterial endocarditis can occur in PDA especially
small PDAs with high velocity jet.The vegetations
invariably occur in pulmonary end of PDA (Figure 5.25).
Therefore one should look for fuzzy, mobile, echogenic
vegetations in suspected cases of bacterial endocarditis in
cases of PDA with fever of more than 3 weeks duration
FIGURE 5.23A: Suprasternal notch view with

colour Doppler shows COA
FIGURE 5.23B: Suprasternal notch view with PW Doppler

shows significant gradient across COA
FIGURE 5.24A: Short axis views show the coil used to close
the small PDA and the device used to close the large PDA
in situ
FIGURE 5.24B: The colour Doppler in short axis shows

Amplatzer device not causing obstruction to left pulmonary
artery
FIGURE 5.25: Parasternal short axis view at the level of aortic

valve show fuzzy echoes in main pulmonary artery at the
junction of PDA and LPA
AORTOPULMONARY WINDOW (AP- WINDOW)

The AP window is a less common lesion than PDA and
consists of a round or oval window like communication
between adjacent portions of the ascending aorta and
pulmonary artery. Though the PDA and AP window are
clinically indistinguishable, the Echo with Doppler study
can distinctly distinguish between them.It is very important
to differentiate them because the pulmonary hypertension
develops in AP window much earlier than in PDA and
management is entirely different. The PDA is treated with
multiple ligation, (a closed heart surgery which is safe and
less expensive), whereas AP window is treated with open
heart surgery and closed with the patch like in VSD. 2-D
echo and the colour Doppler in Parasternal Short axis view
at the level of aortic valve shows the communication
between the aorta (AO) and the main pulmonary artery
(Figure 5.26A). The co-existing anomalies are rare, but
should be looked for PDA and COA in echo (Figure 5.26B).
Though echo can diagnose aorto-pulmonary window,
catheterization is required to know the pulmonary artery
pressure and pulmonary vascular resistance and confirm
the other associate lesions.
Rupture of Sinus of Valsalva (RSOV)

The congenital absence of media in the aortic wall behind
the sinus of valsalva leads to aneurysmal dilatation and
may enlarge over a number of years and rupture even-
tually. The most commonly involved sinuses are the right
and non-coronary, especially the right. The involvement
FIGURE 5.26A: The colour Doppler in parasternal short axis

view at the level of aortic valve show a large communication
between the aorta (AO) and the main pulmonary artery
FIGURE 5.26B: Short axis shows, large AP-Window with

COA in 6 months infant
of left coronary sinus is extremely rare.The aortic sinuses

are almost entirely intracardiac. Hence when they rupture,
invariable it is into cardiac chambers, very rarely into
pericardium.
The 2 D Echo plays an important role in diagnosing
ruptured or unruptured sinus of Valsalva aneurysms. The
short axis view images all these sinuses, hence aneurysm
can be identified together with the pathway of rupture into
the right side of the heart (Figure 5.27). In parasternal long
axis view, the two most relevant sinuses—right and
noncoronary sinuses are imaged. The colour Doppler in
this view can help to distinguish RSOV above the aortic
cusp (Figure 5.28A) from VSD which is below the aortic
cusp (Figures 5.28B and C). The rupture can be confirmed
by continuous Doppler signals.
FIGURE 5.27: Short axis shows aneurysm of

sinus of Valsalva
FIGURE 5.28A: Parasternal long axis view shows ROSV
FIGURE 5.28B: Colour Doppler comparison shows RSOV

to RV is above the aortic cusp. In contrast colour Doppler
in VSD is below the aortic cusp
FIGURE 5.28C: Parasternal long axis view shows

sub-aortic membrane obstructing VSD
CORONARY ARTERIOVENOUS FISTULAS

Coronary arteriovenous fistula is the abnormal fistulous
connection between coronary artery and one of the great
veins entering the heart.Whereas congenital coronary-
Cameral fistula involves an abnormal fistulous connection
between coronary artery and a cardiac chamber. Approxi-
mately half of the coronary AV fistula arise from right
coronary artery (RCA), about 45 per cent from Left
coronary artery (LCA) some 5% arise from both the
coronaries.Rarely all the three major coronaries are
involved.It is more important to detect the drainage site of
fistula than the origin of coronary artery, because surgical
or non surgical coil or device closure is done at this site.
Almost 90 per cent drain into systemic venous side of
circulation, less than 3 per cent drain into the left ventricle.
2-D Echo in parasternal short axis view at the level of

aortic valve reveals the dilated proximal portion of the
coronary artery that feeds the fistula (Figures 5.29A and
B). The fistulous track itself can be traced with the help of
colour Doppler.The Doppler study establishes the site of
entry because the fistula typically generates a continuous,
turbulent, systolic and diastolic flow pattern (Figure 5.30).
The image of dilated coronary sinus is conspicuous when
it receives the fistula.But unlike the other left to right shunts
the RA or RV are not dilated, as less than 10 per cent of
cardiac output shunts through the fistula.If the shunt is
sizeable with grossly dilated coronary artery, then
dilatation of RA or RV occurs, but there is no paradoxical
septal movement.
FIGURES 5.29A and B: Parasternal short axis view shows

(A)dilated right coronary artery and (B)shows aneurysmally
dilated fistulous tract
FIGURE 5.30: Doppler shows continuous, turbulent,

systolic and diastolic flow pattern
Point to Remember
Always ECHO must be seen with the clinical back-
ground. Most of the left to right shunts have common
symptoms like repeated respiratory infections, chest
retractions, feeding difficulties, failure to thrive, excessive
sweating, precordial bulge, bilateral Harrison sulcus. The
exceptions are coronary AV fistula and Anomalous left
coronary artery from pulmonary artery.
AORTIC STENOSIS
Congenital aortic stenosis could be at the level of valve/
supravalvar/subvalvar. However good a clinician is, the
most accurate diagnosis is possible only with echocar-
diography (Figures 5.31 to 5.33).
FIGURE 5.31: Parasternal long axis view shows gross

hypertrophy of LV almost obliterating the LV cavity
FIGURE 5.32A: M-mode shows hypertrophied septum

and posterior wall with reduction in LV cavity
FIGURE 5.32B: M-mode shows eccentric aortic orifice

with cusp separation of 4 mm
FIGURE 5.33: CW Doppler shows transaortic gradient of

242 mm Hg from suprasternal notch
CARDIAC AND EXTRACARDIAC MASS

Cardiac tumours both benign and malignant are rare at
all ages and are extremely rare in infants and children.
Never the less an accurate diagnosis during life is
important because many children can benefit from surgical
excision of tumour. The prevalence of primary cardiac
tumours is 0.08 per cent. Echocardiography has contributed
significantly to the evaluation of cardiac tumours in
children, 2-D and Doppler echo are capable of direct
detection and imaging of cardiac mass. One can predict
the severity of the obstruction of the valve by using Doppler
technique and estimating the gradient. Echo is also useful
for the follow up after surgical removal of the tumour and
also to know the recurrence. Echo also gives information
about the macroscopic appearance, pericardial effusion is
easily detected by M mode and 2-D echo especially in cases
with malignant and metastasis lesions. Most common
tumour in the newborn is rhabdomyoma.
INFILTRATIVE CARDIOMYOPATHY
See Figures 5.34 to 5.41.
Myxomas are the most common type of cardiac
tumours. Approximately 30 per cent of all cardiac tumours.
The majority of the myxomas arise in LA and about 25 per
cent occur in RA. The RV and LV myxomas are rare. LA
myxomas when obstructing LA flow can mimick mitral
stenosis.
FIGURE 5.34A: Parasternal long axis view shows marked

thickening of left ventricular posterior wall and septum
FIGURE 5.34B: Modified view shows marked thickening of left

ventricular posterior wall and reduction in the cavity of LV
FIGURE 5.35: Shows large pericardial effusion

in a case of metastasis
FIGURE 5.36: 2-D echo shows extracardiac mass

compressing the left ventricle
FIGURE 5.37: Parasternal long axis shows large left atrial

myxoma obstructing mitral orifice
FIGURE 5.38: Large vegetation on a congenital bicuspid valve

in a 10 months infant with septicaemia appears like a mass
FIGURE 5.39A: Extracardiac mass compressing the LV
FIGURE 5.39B: Extracardiac mass with pericardial effusion

FIGURE 5.39C: LV mass almost filling the LV extending upto

the aortic valve (histopathological report of specimen removed
showed thrombosis)
FIGURE 5.40A: Parasternal long axis shows hyperechogenic

mass in RV compressing the LV (rhabdomyoma in an infant)
FIGURE 5.40B: Apical four-chamber view shows hyperecho-

genic mass in RV compressing the LV (rhabdomyoma in an
infant)
FIGURE 5.41A: Apical four-chamber view shows obliteration of

the apex of both RV and LV. Dilatation of both RA and LA in
a 14 years old with restrictive cardiomyopathy
FIGURE 5.41B: Parasternal long axis shows reduced LV

cavity due to obliteration of LV apex. LA is bigger than LV
FIGURE 5.41C: Doppler echo showing mitral inflow velocity

shows increased early diastolic filling velocity, decreased atrial
filling velocity
Chapter 6
Cyanotic
Congenital Heart
Diseases
The diagnosis of cyanotic congenital heart disease (CCHD)

is complex. The Echo plays an important role in precise
diagnosis. The CCHDs can be grouped for convenience
into:
Five ‘T’s Three ‘A’s
1. TOF 1. Atresia of pulmonary artery
2. TGA 2. Atresia of aortic valve
3. TAPVC 3. AV-canal defect
4. Truncus arteriosus Two ‘E’s
5. Tricuspid atresia 1. Ebstein’s anomaly
2. Eisenmenger’s complex
Other anomalies are single ventricle, single atrium,
DORV, DOLV.
TETRALOGY OF FALLOT
Tetralogy of Fallot (TOF) is the most common cyanotic
congenital heart disease. 2-D ECHO permits visualization
of the essential anatomic features of TOF. The subcostal
and parasternal long axis views show the malaligned
infundibular septum, large sub-aortic VSD, aortic-mitral
continuity, overriding large biventricular aorta (Figure 6.1).
The short axis shows, infundibular, pulmonary valvar
stenosis, size of main pulmonary artery and its branches,
relative size of aorta and left atrium. In the same view sub-
arterial extension of VSD is seen beneath the right coronary
cusp and anomalous muscle bundles are seen below the
infundibulum. Nearly 10 per cent of patients with TOF
have anomalous coronary arteries. The coronary arteries
can be traced with some effort (Figure 6.2). With the colour
CYANOTIC CONGENITAL HEART DISEASES 95
FIGURE 6.1: A.Parasternal long axis show the malaligned

infundibulum septum, large VSD, aortic-mitral continuity, over-
riding biventricular large aorta, B. Modified view shows good
sized pulmonary artery
FIGURE 6.2: Short axis shows right coronary artery arising

from left coronary cusp
Doppler and CW Doppler of the collaterals should be

looked for. Right sided aortic arch can be detected in
suprasternal notch view. The TOF may be associated with
pulmonary atresia or rarely dysplastic/absent pulmonary
valve/absent left pulmonary artery/right pulmonary artery.
In case of pulmonary atresia, there are no demonstrable
pulmonic valve and 25 per cent of them are associated
right-sided aortic arch.
TOF with Absent Pulmonary Valve and Absent

Left Pulmonary Artery (LPA)
The incidence of absent left pulmonary artery is 2.8 per
cent and absent right pulmonary artery is 0.7 per cent. The
patients with TOF with unilateral absence of pulmonary
artery or valve are high risk group for whom there is no
consensus on the correct approach to management.
Usually in TOF there is RV hypertrophy, infundibular
and/or pulmonary valvar stenosis with small underfilled
and under developed pulmonary tree.In contrast in TOF
with absent pulmonary valve, the RV is dilated due to
pulmonary regurgitation and though the pulmonary
annulus is narrow causing stenosis, the pulmonary artery
is huge and aneurysmally dilated (Figures 6.3A to C).
TRUNCUS ARTERIOSUS
The Truncus arteriosis is characterized by a single great
artery that arises at the base of the heart and gives rise to
coronary, pulmonary and systemic arteries.The pulmonary
FIGURE 6.3A: Short axis shows echo-dense ridges projecting

into the lumen at the expected site of the pulmonic valve. Dilated
RVOT, main pulmonary artery and right pulmonary artery with
absent left pulmonary artery
FIGURE 6.3B: Colour Doppler in short axis shows pulmonary

stenosis and pulmonary regurgitation in a 13 years old boy
of TOF with absent pulmonary valve and Left pulmonary artery
FIGURE 6.3C: The pulsed Doppler shows antegrade systolic

flow due to stenosis and retrograde diastolic flow due to
pulmonary regurgitation
valve is absent. 2-D Echo in parasternal long axis view

reveals large single arterial trunk overriding a large defect
in the infundibular septum, with continuity between the
truncus and the anterior mitral leaflet (Figures 6.4A and
B). The short axis reveals blunt RVOT with no pulmonary
valve or pulmonary artery, but sometimes more than three
cusps in truncal valve (Figure 6.5) . Normally the ascending
aorta does not give rise to any branches, other than
coronaries. But in truncus arteriosus, direct visualization
of truncal origin of the main pulmonary artery from the
side of truncus (Type 1), or separate right and left branches
(Type 2) is the major requirement for echocardiographic
diagnosis.The truncal valve is usually tricuspid or
FIGURE 6.4A: Apex down image reveals large trunk

overriding VSD
FIGURE 6.4B: Parasternal long axis shows mitral to truncal

continuity, Large VSD with overriding large great artery
FIGURE 6.5: Short axis shows quadricuspid truncal valve

with truncal regurgitation
FIGURE 6.6A: Modified parasternal long axis shows main

pulmonary artery arising from the side of the truncus arteriosis
(Type-A)
FIGURE 6.6B: CW Doppler with cursor on PA
quadricuspid. Direct visualization of truncal origin of the

main pulmonary artery or its main branches is the major
requirement for the echocardiographic diagnosis of truncus
arteriosis and it is done through the long axis, sub-costal
and short axis views (Figures 6.6A and B). The short axis
view also permits the interrogation of the truncal valve to
determine the number of leaflets, generally 3 or 4.
TRICUSPID ATRESIA
The atretic tricuspid valve is represented by dense band
of echoes (Figure 6.7). The 2-D Echo is not only diagnostic
of tricuspid atresia, but it also establishes the position of
great arteries, the condition of the inter-ventricular septum
and the nature of ASD.The ventricular to great arterial
FIGURE 6.7: Apical four-chamber view shows dense band of

echoes at the site normally occupied by the tricuspid valve and
single atrium, as there is no IAS and single ventricle as there
is only a small rudimentary septal tissue at the apex
connections are best established in parasternal and

subcostal short axis views.These views along with CW
Doppler study assist in identification of the presence or
absence of pulmonic stenosis and collaterals (Figures 6.8A
and B). The comparison of the relative sizes of aortic root
and pulmonary trunk can also be done (Figure 6.9). In the
common variety of tricuspid atresia, the apical 4-chamber
view identifies a dense band of echoes at the site normally
occupied by the tricuspid valve. The right ventricular
cavity is small, and the left ventricular cavity is only
moderately enlarged. The right atrium is increased in size
and the atrial septum bulges towards the left if the inter-
atrial communication is restrictive. When tricuspid atresia
FIGURE 6.8A: CW Doppler shows associated

pulmonary stenosis
FIGURE 6.8B: CW Doppler shows continuous signals in

associated collaterals
FIGURE 6.9: Apex down image shows transposition of great

arteries in 6 years-old girl with tricuspid atresia, the great
arteries run parallel and pulmonary artery is smaller than aorta
due to pulmonary stenosis
occurs with transposition of the great arteries, a large

ventricular septal defect is imaged in the apical 4-chamber
view and the right ventricular cavity is well developed.
When pulmonary stenosis is present the echocardiogram
identifies the obstruction.
COMPLETE TRANSPOSITION OF GREAT ARTERIES (D-TGA)

In D-TGA there is ventriculoarterial discordance, but there
is atrioventricular concordance.The connection in TGA
results in a unique two independent parallel circulations
in contrast to one continuous circulation in series in
normal heart. That is in TGA the blood circulates from
FIGURE 6.10A: Modified view shows the arching vessel

aorta arising from dilated morphological right ventricle
FIGURE 6.10B: Apical two-chamber view shows, branching

vessel pulmonary artery arising from ellipsoid, smooth walled
LV
RA→RV→AO→ and back to RA and from LA → LV →

PA → and back to LA.The 2-D Echo establishes the spatial
relationships of great arteries, their ventricular origins
Ventriculoarterial discordance (Figures 6.10A and B),
presence or absence of ASD/VSD/PDA. As the survival
of the neonate with TGA depends on patency of PDA and
timely surgery, hence Echo plays most crucial role, not only
in early precise diagnosis, but also in management of the
infant with TGA. Infact complete diagnosis including
coronary artery anatomy, pulmonary stenosis or pul-
monary hypertension helps the surgeon to plan the
surgery.
FIGURE 6.11: The typical cup and sausage appearance in short

axis formed by anterior “sausage” by RVOT and main
pulmonary artery transected tangentially and a “posterior
circle”—the aorta
FIGURE 6.12: Short axis in TGA the great arteries typically

appear as “double circles,” with the aorta anterior and to the
right/by the side of main pulmonary artery which is to the left
and posterior
In the normal heart the spiral relationship of the main

pulmonary artery and aorta in the short axis appears
(Figure 6.11) in the Echo as an anterior “ sausage” (RVOT
and main pulmonary artery transected tangentially) and
a “posterior circle” (the aorta). In complete TGA, the great
arteries typically appear as double circles, with the aorta
anterior and to the right/by the side of main pulmonary
artery (Figure 6.12). In the normal heart, there is mitral to
aortic continuity, whereas in TGA there is mitral to aortic
discontinuity ( Figure 6.13).
FIGURE 6.13: Parasternal long axis shows mitral to aortic

discontinuity, as the aorta does not arise but the pulmonary
artery arises from left ventricle
TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION

(TAPVC)
TAPVC accounts for about 2 per cent of deaths from CHD
in the first year of life. Hence it is important to detect it in
time in the neonates to get surgical correction in time. On
2-D echo, RA and RV are dilated, absence of pulmonary
vein draining to small left atrium in the presence of bulging
of septum primum at foramen ovale indicate TAPVC. But
positive diagnosis is made by identifying pulmonary
venous connection to systemic veins (either SVC or IVC)
or coronary sinus rather than left atrium. Depending on
where the pulmonary veins are connected, there are 3 types
of TAPVCs: (i) Supra-cardiac TAPVC where all the
FIGURE 6.14A: Apex down image in subcostal view

shows dilated superior vena cava, the colour Doppler
shows horizontal vein draining into the junction of RA
and superior vena cava
FIGURE 6.14B: Suprasternal short axis shows the

upper part of figure of eight, formed by vertical vein,
horizontal vein and dilated SVC.The colour Doppler also
shows the associated PDA in 18 months-old boy
FIGURE 6.15: Colour Doppler shows turbulence in vertical

vein caused by haemodynamic vice
pulmonary veins drain into common chamber (CC) or

confluence and through the vertical vein, horizontal vein
drain into the junction of RA and superior vena cava
(Figures 6.14A and B). Sometimes the vertical vein can be
obstructed when it is compressed and caught between
dilated left pulmonary and left bronchus-called haemo-
dynamic vice (Figure 6.15). (ii) Intra-cardiac TAPVC—all
the pulmonary veins communicate directly with the right
atrium or coronary sinus. (iii) Infra-diaphragmatic—
consists of confluence of pulmonary veins that give rise to
a vascular channel that descends anterior to the oeso-
phagus and penetrates the diaphragm through the
oesophageal hiatus terminating in the portal vein or less
commonly in the inferior vena cava or ductus venosus.
Invariably obstruction of the descending venous channel

is almost always present. So when the connection is to the
portal vein, the blood has to traverse through the hepatic
capillary bed which constitutes an obligatory zone of
resistance. This is a more difficult one to detect on the echo
unless one has it in mind and looks for it.
2D Echo visualizes the venous confluence in the
parasternal long axis view. A supra-sternal view images
the venous confluence with the cross like shape or figure
of 8. In addition to the left vertical vein and the right
superior vena cava. The connection of the venous con-
fluence to the right atrium is identified in the sub-costal
right axis view. When the venous confluence is behind the
left atrium or when the anomalous venous connection is
to the coronary sinus, two chambers are imaged in that
location in place of the normally undivided left atrium
(Figures 6.16A to C). A dilated coronary sinus is also
imaged behind the aortic root and in the atrio-ventricular
groove running rightward towards the inter-atrial septum
(Figure 6.17).
Infra-diaphragmatic TAPVC is diagnosed in the sub-
costal view. The visualization of descending anomalous
venous channel anterior to the descending aorta is
diagnostic. The contrast echocardiography identifies this
channel as distinct from the inferior vena cava because the
anomalous vein remains echo free owing to filtration of the
injected contrast material in the pulmonary capillary bed.
Dopplar echo is an effective alternative in identifying the
anomalous venous channel, the descending aorta and the
FIGURE 6.16A: Apical four-chamber view reveals RA, RV dilated

and LV, LA small with no pulmonary veins draining into LA, but
venous confluence adjacent to a nondilated left atrium
FIGURE 6.16B: Parasternal long axis view reveals dilated RV,

compressed small LV with common chamber (CC) or venous
confuence below LA
FIGURE 6.16C: TAPVC-figure of eight appearance shown

in echo. Vertical vein (VV), horizontal vein (HV) draining into
SVC and RA junction
FIGURE 6.17: Subcostal view shows ASD, dilated RV, RA, small
LV, pulmonary veins not draining into LA and a dilated coronary
sinus is imaged in the atrio-ventricular groove running right-
ward towards the inter-atrial septum in a 10-month-old infant
inferior vena cava. The aorta is recognized by the pulsatile

high frequency signal. The anomalous venous channel and
the inferior vena cava are distinguished from each other
by the direction of the flow which is in opposite direction.
TAPVC without obstruction results in volume overload of
the right ventricle and paradoxical septal motion. The
converse is the case when the pulmonary veins are
obstructed.
AORTIC ATRESIA (HYPOPLASTIC LEFT HEART SYNDROME—

HLHS)
Aortic atresia typically occurs with intact ventricular
septum, small ventricular cavity and either hypoplastic or
atretic mitral valve. Aortic atresia is said to be the most
malignant among the CHDs, because the average life span
is 14 days. Hence the echocardiographer is least likely to
see this anomaly as the neonates die before reaching the
tertiary hospital. It is extremely a rare anomaly with 0.03
per cent incidence.
2-D echo provides important diagnostic information
about aortic atresia. The parasternal long axis and apical
four-chamber views reveal a small left ventricular cavity,
with bright echoes arising from areas of endocardial
fibroelastosis.The free wall and septum are thick but
virtually immobile (Figure 6.18A). In the same view an
imperforate aortic valve and hypoplastic ascending aorta
are seen (Figures 6.18B and C). The calibre of the aortic arch
is best determined through suprasternal notch or high
parasternal windows, but care must be taken to avoid
FIGURE 6.18A: Parasternal long axis shows small left

ventricular cavity, with bright echoes arising from areas of
endocardial fibroelastosis.The free wall and septum are
thick. Large pulmonary artery arising from dilated RV
FIGURE 6.18.B: Parasternal long axis shows the colour

Doppler flow through hypoplastic mitral valve and with no
flow across the imperforate aortic valve and hypoplastic
ascending aorta
FIGURE 6.18C: Modified parasternal long axis shows small

LV and dilated RV, pulmonary regurgitation in colour Doppler
in a 15 days neonate with HLHS
FIGURE 6.19: Subcostal view shows dilate RV, RA,

small LV, hypoplastic MV, ASD with R-L shunt
mistaking continuity between pulmonary trunk, ductus

and the descending aorta. A subcostal four-chamber view
images the atrial septum, which bulges to the right and
contains the patent foramen ovale with its moving flap or
ASD (Figure 6.19).
AV CANAL DEFECT (ENDOCARDIAL CUSHION DEFECTS)

The term endocardial cushion defects or AV canal defects
mean a defect in the atrioventricular septum.2D echo
permits assessment of the atrioventricular junction,
identifying the abnormalities of the valve orifices, leaflet
tissue and tension apparatus in addition to defining the
interatrial and interventricular communications, the
morphology of the left ventricular outflow tract and left
ventricular papillary muscles and the secondary physio-
logic changes in cardiac chambers and in the ventricular
septum. In the partial endocardial cushion defect (ostium
primum ASD) the subcostal 4-chamber view shows ostium
primum ASD, the attachments of separate tricuspid and
mitral valve leaflets at the same level (Figure 6.20). The short
axis near the apex reveals the characteristic cleft in the
anterior mitral leaflet. In the parasternal long axis view the
right and the left ventricular cavities with the paradoxical
motion of IVS and long narrow LVOT is noted (Gooseneck
appearance). Ostium primum defects involve areas of the
atrial septum adjacent to the atrioventricular valves. It is
important to recognize that primum defects are not the only
defects in the atrial septum. Rather, the defect also involves
the common atrioventricular septum, and the result is
FIGURE 6.20: Echo picture in subcostal view of AV canal defect,

in a four-year-old child with Down’s syndrome
deformity of the alignment of the atrioventricular valves

and may be associated with defects of the adjacent
interventricular septum. It is best visualized from the
subcostal view.
In complete AV canal defect the sub costal 4 chamber
view reveals the interatrial and interventricular communi-
cations with the bridging leaflets between them (Figure
6.21). The attachments of the bridging leaflets to the crest
of the ventricular septum is distinguished from the free
floating leaflets that appear as a bar across the AV canal
defect. The VSD is beneath the anterior bridging leaflet and
interatrial communication lies above. The echo also
identifies the number and positions of the left ventricular

papillary muscles and the relative sizes of the left
ventricular and right ventricular cavities.
FIGURE 6.21: Subcostal four-chamber view reveals the

interatrial and interventricular communications with bridging
common atrioventricular valve (AVV) leaflets between them
UNIVENTRICULAR HEART (SINGLE VENTRICLE—SV)

The term univentricular heart or single ventricle is applied
to the congenital malformation in which both the atria are
connected to one ventricle (Figure 6.22), that qualifies as
left or right or indeterminate ventricle purely on morpho-
logical grounds. 2D echo is diagnostic of not only the
univentricular heart, it categorizes the heart into left, right
or indeterminate ventricular type. In addition the echo can
locate an antero-superior outlet chamber in the apex down
position. It can also detect the presence or absence of
FIGURE 6.22: In apex down image both left atrium (with

pulmonary veins) and right atrium are connected to one
ventricle
pulmonary stenosis and also the size of the bulbo-

ventricular foramen. It can identify a postero-inferior
rudimentary left ventricle. 2D echo also identifies the type
of atrio-ventricular connection as 2 valves: an imperforate
right or left component (Tricupid atresia or mitral atresia)
a common AV valve and hypoplastic valve or a straddling
or overridding valve (Figures 6.23A and B).
A major criterion for the echocardiographic diagnosis
of single ventricle with main morphological LV chamber
is a rudimentary chamber that is anterior-superior and
either left sided (inverted) or right sided (non-inverted). The
morphological left ventricle gives rise to pulmonary artery
and the rudimentary chamber is an outlet that gives rise
to the aorta (Figures 6.24A and B).The bulbo-ventricular
FIGURE 6.23A: Parasternal long axis view shows single

ventricle, common AV valve
FIGURE 6.23B: Parasternal long axis view shows highly

trabeculated (morphologically RV type) single ventricle with
single AV valve
FIGURE 6.24A: Apex down image shows right sided outlet

chamber (OC) with non-restrictive bulbo-ventricular foramen
(BF) giving rise to aorta (coronary artery is seen arising from
the side) in a 3 years-old boy of single ventricle
FIGURE 6.24B: Rudimentary anterior-superior outlet chamber

(OC) with restrictive bulbo-ventricular foramen is seen from
main morphological LV
FIGURE 6.24C: Apex down image shows aorta arising from left
sided outlet chamber (OC) and pulmonary trunk (bifurcating)
arising from main morphological left ventricle
foramen may be restrictive or non-restrictive.A major

criterion for echocardigraphic diagnosis of single ventricle
with a morphologic right ventricular main chamber is a
rudimentary chamber that is inferio-posterior, a location
well seen in the short axis (Figure 6.24C). In single ventricle
of indeterminate type a rudimentary chamber is not
present.
SINGLE ATRIUM (COMMON ATRIUM)

Common atrium is a rare variety of intra-atrial com-
munication characterised by absence or virtual absence of
the atrial septum or vestigial remnants which may
occasionally remain. The right-sided portion of common
atrium has anatomic features of RA, (Crista terminalis,

pectinate muscle, right atrial appendage) and receives both
vena cavae and the coronary sinus. The left sided portion
has anatomic features of a left atrium (smooth, non-
trabeculated walls; left atrial appendage) and receives the
pulmonary veins (Figure 6.25A). The cleft-anterior mitral
leaflet is nearly always present. 2D echo in common atrium
shows absence of echoes from any part of the atrial septum
(Figure 6.25B) together with the cleft anterior mitral leaflet.
FIGURE 6.25A: Parasternal long axis shows absence of

echoes from any part of the atrial septum common atrium with
left sided portion of a left atrium (smooth, non-trabeculated
walls; left atrial appendage) receiving the pulmonary veins. And
the right-sided portion of common atrium with features of RA,
receiving suprahepatic IVC and SVC with hypoplastic mitral
valve HMV and normal TV
FIGURE 6.25B: Colour Doppler echo shows AV

valve regurgitation
EBSTEIN’S ANOMALY
The basic anatomic feature of Ebstein’s anomaly is a
remarkable displacement of septal-tricuspid leaflet leading
to 3 morphological components in the right side of the
heart. These components are: (i) right atrium proper, (ii)
the functional right ventricle, and (iii) an intervening zone
that is anatomically ventricular but functionally right
atrium (atrialized right ventricle). The displacement of
septal tricuspid leaflet is measured as the distance from
the anatomic annulus to the distal attachment of the septal
leaflet. For the diagnosis of Ebstein’s anomaly, the
displacement index to body surface area of 8 mm/m2 is
significant.
2D echo is uniquely equipped for studying and

establishing the diagnosis of Ebstein’s anomaly. M -mode
imaging reveals an increased excursion of the anterior
tricuspid leaflet and delayed closure of the tricuspid valve
(Figure 6.26). An exception to this useful sign is the closure
of tricuspid valve earlier than closure of mitral valve in
response to pre-excitation of the right ventricle (WPW
syndrome).
FIGURE 6.26: M-mode imaging reveals an increased excursion

of the anterior tricuspid leaflet and delayed closure of the
tricuspid valve
In apical 4-chamber view displacement of the septal

tricuspid leaflet is measured as the distance from the
anatomic annulus to the distal attachment of the septal
leaflet, the atrialized right ventricle is identified in the same
view as the distance between the anatomic tricuspid
FIGURE 6.27: Apical 4-chamber view shows septal tricuspid

leaflet adherent to IVS and the displacement of the septal
tricuspid leaflet, atrialised RV and small functional RV
annulus and the functional annulus, defined as the leading

edge of the displaced septal leaflet (Figure 6.27). The
functional right ventricle occupies the area distal to the
displaced leaflet and the large atrialized RV and the large
atrium is proximal to the anatomic annulus. The tethered
septal leaflet is in contrast to the “whip like” or “sail like”
motion and the diastolic doming of the large, elongated
anterior leaflet seen in real-time (Figure 6.28). The
parasternal long axis view and apical four-chamber view
shows septal tricuspid leaflet adherent to IVS .The echo in
same views also image the altered left ventricular geometry
and cavity size caused by diastolic displacement of the
atrialized ventricular septum toward the left ventricular
free wall (Figure 6.29).
FIGURE 6.28: Echo shows “whip like” or “sail like” large,

elongated anterior tricuspid leaflet
FIGURE 6.29: Parasternal long axis view shows altered left

ventricular geometry and cavity size caused by diastolic
displacement of the atrialised ventricular septum toward the
left ventricular free wall
DOUBLE OUTLET RIGHT VENTRICLE (DORV)

When both the great arteries arise from the right ventricle,
the left ventricular stroke volume is then ejected through
the VSD into both aorta and pulmonary artery, it is termed
DORV.The 2-D echo provides the diagnostic information
like (i) the origin and the spatial relationships of great
arteries, (ii) the presence and position of infundibular
septum, (iii) the relationship of the VSD to aortic and
pulmonic valves, (iv) mitral to aortic discontinuity, (v) the
absence or presence of pulmonic stenosis, and (vi) the
association of subaortic stenosis, COA, PDA, and subpul-
monic VSD.The aorta and pulmonary artery run parallel
and are separated by a prominent conus septum and are
related entirely or predominantly to the morphologic right
ventricle (Figures 6.30A and B). In the short axis at the
cardiac base, the great arteries appear as double circles,
with aorta to the right of the pulmonary trunk. The
pulmonary trunk is cofirmed as such by its bifurcation into
right and left branches, and the aorta by its brachiocephalic
branches.The VSD may be sub-pulmonic or doubly
committed. There could be 16 types of DORV, depending
on combination and permutation of four types of position
of great arteries and four type of commitment of VSD.The
DORV with subpulmonic VSD is called “Taussig-Bing
anomaly.”
DOUBLE OUTLET LEFT VENTRICLE (DOLV)

Double outlet left ventricle or the origin of both great
arteries from the morphologic left ventricle is among the
FIGURE 6.30A: Parasternal long axis view shows mitral to aortic

discontinuity, the aorta and pulmonary artery run parallel and
are separated by a prominent conus septum and are related
entirely or predominantly to the morphologic right ventricle
FIGURE 6.30B: Apex down image shows both aorta and pul-
monary artery arising from the morphologic right ventricle
rarest of abnormal ventriculoarterial alignments. In

simplest term the DOLV is the converse of DORV, i.e. both
great arteries arise entirely or predominantly from the
morphologic left ventricle. The VSD may be sub-aortic or
sub-pulmonic or absent and obstruction to ventricular
outflow may or may not be present.
Eisenmenger’s complex is irreversible pulmonary
hypertension as a result of long standing left to right shunts
through large ASDs, VSDs, PDAs where there is systemic
or suprasystemic pulmonary artery pressure, leads to right
to left shunts through the defects. On ECHO there is gross
dilatation of RA and RV, MPA and its branches. When
patient is in congestive cardiac failure, the pericardial
FIGURE 6.31: Apical four-chamber view shows gross

dilatation of RA and RV with pericardial effusion and
compressed LV and LA. IAS bulging from right to left in a
case of Eisenmenger’s complex
effusion and pleural effusion can be detected on echo. The

colour Doppler will show right to left shunt through the
defects. Many a time large PDAs can be missed. The
Eisenmenger’s complex is a contraindication for any
procedure or surgery as any attempt to operate upon can
lead to suicidal RV and mortality. Hence every left to right
shunt should be detected in time for the management and
echo plays an important role in detecting the defect, size
of defect, the shunt and pulmonary artery pressure (Figure
6.31).
Point to Remember
ECHO plays an important role not only in the diagnosis
of L to R shunt but also in monitoring, management and
preventing Eisenmenger’s complex.
Chapter 7
Rare Congenital
Heart Diseases
Echocardiography has emerged as an important, non-

invasive method for diagnosing all Congenital Heart
Diseases (CHD). There are some extremely rare conditions
which are not seen in daily practice but seen once in a
lifetime. Despite the experience in doing the echo, the eyes
will not see what the mind does not know. Hence there
is a likelihood of either missing the diagnosis or mis-
diagnosing the condition. Among such conditions are: (i)
non-compaction of left ventricle, (ii) aorticoventricular
tunnel, (iii) cor-triatriatum, (iv) anomalous left coronary
artery from pulmonary artery (ALCAPA), (v) sub-aortic
obstruction, (vi) double aortic arch, (vii) primary
endocardial fibroelastosis, (viii) mitral atresia (ix) isolated
double chambered right ventricle, and (x) cardiac mal-
positions.
Echocardiography not only plays an important role in
detection of these conditions but also in management
strategy. Echocardiographic features are so characteristic
that once understood it is very easy to diagnose these
conditions.
NON-COMPACTION OF LEFT VENTRICLE OR

“SPONGY MYOCARDIUM”
This is a very rare congenital cardiomyopathy causing
malignant arrhythmias and pump failure, leading to high
incidence of morbidity and mortality. Hence, early, precise
diagnosis is important. It is caused by arrest in endomyo-
cardial embryogenesis. The endomyocardial morphogene-
sis is characterized by numerous, excessively prominent
RARE CONGENITAL HEART DISEASES 135
ventricular trabeculations and deep intertrabecular recesses

of the myocardium, more prominent at the apex of the
ventricle (Figure 7.1).
FIGURE 7.1: Echo shows, numerous, excessively prominent

ventricular trabeculations and deep intertrabecular recesses
of the myocardium, more prominent at the apex of LV
To quantify the depth of penetration of the inter-

trabecular recesses with 2-D Echo, X to Y ratio is developed
(Figure 7.2). This ratio is the quotient of the distance
between the epicardial surface and trough of a trabecular
recess (represented by X) to the distance between epicardial
surface and peak of the trabeculae (represented by Y).
Spongy myocardium has to be differentiated from muscle
bundles. Discrete muscle bundles are more than 2 mm in
diameter that stand out against the background of the left
ventricular endocardium and have been reported in 68 per
cent of normal hearts and are virtually always 2 to 3 or
FIGURE 7.2: The diagrammatic description of quantifying

the depth of penetration of the intertrabecular recesses
less in number. In contrast, in non-compaction there are

numerous prominent trabeculations and conspicuous
intertrabecular recesses that penetrate deep into left
ventricular myocardium. The echocardiographic pattern is
characteristic and diagnostic. Spongy myocardium may be
either isolated or may be associated with other congenital
anomalies, including anomalous origin of the left coronary
artery from the pulmonary trunk and obstruction to right
or left ventricular outflow.
Points to Remember
Usually LV is smooth walled with fine trabeculations.
When the left ventricle is highly trabeculated one should
distinguish the morphological RV with the lower attach-
ment of tricuspid valve of CTGV from deep intertrabecular
recesses with normal mitral valve of spongy myocardium
AORTICO-LEFT VENTRICULAR TUNNEL (ALVT)

ALVT is a very rare congenital anomaly, the true incidence
is not known. ALVT is an abnormal paravalvular pathway
between the aortic root and left ventricular cavity by
passing the aortic valve and not penetrating the septal
musculature. It has an arterial like structure with walls rich
in elastic tissue. In contrast, more common rupture of sinus
of Valsalva aneurysm originates proximal from the
sinotubular junction within the right or non-coronary
sinuses, seldom from the left and ruptures into right atrium
or right ventricle or left ventricle or rarely into pericardium
and interventricular septum.
Echocardiography in parasternal long axis view shows
the extent of the tunnel between the aorta and left ventricle
(Figure 7.3A). Parasternal long axis view with colour
Doppler demonstrates the tunnel and the Doppler flow
forward into the aorta and regurgitate into the ventricle.
(Figure 7.3B). Echo helps in accurate diagnosis of ALVT
as it helps in differentiating from rupture of Sinus of
Valsalva into LV which originates proximal to sinotubular
junction and transoesophageal Echo also assists during
FIGURE 7.3A: Parasternal long axis view shows the extent

of the tunnel between the aorta and left ventricle
FIGURE 7.3B: Parasternal short axis view demonstrates the

tunnel and the Doppler flow forward into the aorta and regurgi-
tation into the ventricle
device closure. It also helps to detect residual flow if any

after the device closure (Figure 7.4) and degree of aortic
regurgitation which is invariably associated.
FIGURE 7.4: Echo with colour Doppler shows device

in situ, with no residual flow
Point to Remember
In parasternal long axis view when there is abnormal
colour flow and CW Doppler signals, then look carefully
whether the flow is below the aortic cusp (sub-aortic VSD
with aortic regurgitation) or above the aortic cusp but
below/proximal to sinotubular junction (Rupture of sinus
of Valsalva), above the sinotubular junction but into
pulmonary artery (Aorto-pulmonary window), above the
sinotubular junction and entering the left ventricle, then
it is aortoventricular tunnel.
COR-TRIATRIATUM SINISTRA
It is a rare cardiac malformation with the prevalence of
about 0.1 per cent of all CHDs. It is characterized by the
presence of a fibro muscular membrane that divides the
left atrium into a distal chamber (DC), which is the true
left atrium and is related to the left atrial appendage (LAA)
and the mitral valve and the proximal chamber (PC) or
accessory left atrium which is related to the pulmonary
veins (Figures 7.5A to C). When this membrane is
obstructive type the patient presents clinically like mitral
stenosis or Leutembacher’s syndrome. In its classic form,
the accessory chamber (PC) receives blood from pulmonary
veins and communication with left atrium is accomplished
FIGURE 7.5A: Diagrammatic representation of cor

triatriatum. The arrows indicate the membrane
FIGURE 7.5B: Apex down image shows a fibromuscular

membrane that divides the left atrium into a distal chamber
(DC), which is the true left atrium and is related to the left
atrial appendage (LAA) and the mitral valve (MV) and the
proximal chamber (PC) or accessory left atrium in which
the pulmonary veins (PV) drain
FIGURE 7.5C: Apical four-chamber view shows, ASD,

membrane, DC with MV and LAA and PC with PV and ASD
by way of one or more fenestrations in the membrane and

the colour flow Doppler demonstrates the flow through the
fenestration (Figures 7.6A and B).
FIGURE 7.6A: Apex down image shows the fenestration

(arrow) in the membrane in LA
FIGURE 7.6B: Apex down image with the colour Doppler blood
flowing through the fenestration in the membrane in LA
The malformation is usually isolated but in about one

in four patients is associated with other congenital defects
of a complex nature. The transthoracic echo (TTE) with
Doppler is an excellent modality to diagnose this condition.
Rarely transesophageal echo (TEE) can give better yield on
the number and location of openings in the membrane.
This condition should be differentiated from supravalvar
mitral ring which is characterized by the presence of a
membrane in left atrium immediately above the mitral
valve and does not divide the left atrium into two chambers
(Figure 7.7A ) in contrast to cor-triatriatum which divides
the LA into 2 chambers (Figure 7.7B). The mitral annulus
usually appears hypoplastic and the membrane often
FIGURE 7.7A: Apical four-chamber view shows ASD with

supravalvar mitral ring which is seen as a membrane
in left atrium immediately above the mitral valve
FIGURE 7.7B: Zoomed picture of LA shows a mem-

brane (arrow) that divides the left atrium into a distal
chamber (DC) and proximal chamber (PC), pulmonary
veins (PV) draining into PC
appears to be incorporated into the mitral leaflets and

hence often overlooked in echo. The echo with Doppler is
considered as an excellent means of describing all aspects
that point to diagnosis, pathophysiology and corrective
surgery.
Cor-triatriatum Dexter
It is an extremely rare anomaly (about 0.1% incidence)
characterized by a membrane that divides the right atrium
into two chambers. A distal chamber is related to the right
atrial appendage (RAA) and tricuspid valve and the
proximal chamber is related to superior and inferior vena
cavae and coronary sinus. Rarely membrane could be
stenotic. To differentiate it from Eustachian valve, the

coronary sinus is medial to membrane in cor triatriatum
dexter.
Point to Remember
One should not be content with identifying just ASD, but
look for other associated lesions. Other wise rare anomalies
like cor triatriatum and supramitral ring are missed. In a
case of ASD, if colour flow shows turbulence in LA look
carefully in subcostal view for the membrane and where
the pulmonary veins are draining, proximal or distal to the
membrane.
ANOMALOUS LEFT CORONARY ARTERY FROM

PULMONARY ARTERY (ALCAPA)
The left coronary artery arises anomalously from the
pulmonary trunk or either left or right pulmonary artery
branch. The incidence of ALCAPA is 0.4 per cent of all
CHDs. 2D echo with colour Doppler flow mapping has
replaced cardiac catheterization as a standard method of
diagnosis in the sick neonates with reduced LV function
in ALCAPA. 2-D echo not only identifies the origins of
coronary artery and also provides information on left
ventricular function, wall motion, thickness and mitral
regurgitation. The portion of left ventricle supplied by the
aberrant left coronary is thin, scarred, dilated (Figure 7.8)
and occasionally aneurysmal. The endocardium exhibits
fibroelastosis. The pulmonary origin of anomalous left
FIGURE 7.8: Apical four-chamber view in 30 days-old infant

shows dilated LV (reduced LV function-EF-25%) with the
glistening endocardium (fibroelastosis secondary to
ischaemia)
coronary artery is visualized from long and short axis

views with the beam directed toward the base. It is
important to note that the left coronary artery is easier to
image than the right and can be identified in most patients.
The short axis with appropriate rotation of the transducer
with colour flow mapping, it is feasible to trace the
anomalous left coronary artery to its pulmonary arterial
attachment and demonstrate the retrograde flow in the left
coronary system and an abnormal jet from the left coronary
system into the pulmonary trunk (Figures 7.9A and B).
Detection of antegrade diastolic flow in the left coronary
system virtually precludes the diagnosis. Absence of LAD
from its usual origin in the left sinus of Valsalva does not
distinguish the lesion from single coronary artery.
FIGURE 7.9A: The parasternal short axis view with the

beam directed toward the base, shows there is no left
coronary artery arising from left coronary cusp but
abnormal coronary towards PA
FIGURE 7.9B: The parasternal short axis view with the

appropriate transducer rotation and colour flow mapping
demonstrates retrograde flow in the left coronary system
and an abnormal jet from the left coronary system into
the pulmonary trunk
Point to Remember
In any neonate with dilated LV with reduced function
when other causes like COA, critical aortic stenosis are
ruled out look for ALCAPA in short axis with appropriate
transducer rotation and colour Doppler.
CONGENITAL SUBAORTIC OBSTRUCTION

Congenital subaortic obstruction is a challenge for the
clinician. Echocardiography can help in the precise
diagnosis so that appropriate treatment modality can
be decided. Subaortic stenosis is divided into three types:
(i) discrete membranous “diaphragmatic”, (ii) fibro-
muscular ridge, and (iii) tunnel like obstruction.
Discrete Subaortic Membrane

It is usually associated with other congenital anomalies
in 60 to 70 per cent of cases, e.g. VSD, (Figure 7.10) bicus-
pid AV (Figure 7.11), coarctation of aorta—?COA (Figure
7.12) atrioventricular septal defects (AV-canal). The
subaortic obstruction is almost always progressive and is
not static. Subaortic obstruction may have a “diaphragm”
beneath the aortic valve, which may be crescenteric or form
a complete circle. Often it may be attached to anterior mitral
leaflet and this crucial information should be reported so
that the surgeon can take precaution during excision of the
membrane and avoid damage to anterior mitral leaflet
(AML).
Because of the likelihood of progressive damage to the
aortic valve leading to thickening, regurgitation and
FIGURE 7.10: Modified parasternal long axis view shows,

large VSD with subaortic membrane narrowing the LVOT
FIGURE 7.11: Zoomed picture of short axis of aorta shows

bicuspid aortic valve in a case of subaortic membrane
FIGURE 7.12: Suprasternal view with the colour Doppler

shows COA in 2 months old infant with subaortic membrane
and bicuspid aortc valve
sometimes severe LV dysfunction, there is some consensus

that the membrane should be removed early to prevent
progressive aortic valve damage. There is some debate
about whether this lesion is indeed congenital, since it is
rarely observed in newborns. It is therefore often regarded
as an acquired lesion. It has been proposed that a left
ventricular outflow malformation characterized by a wider
mitral aortic separation, an exaggerated aortic override and
a steeper aortoseptal angle is present in children with VSD
or COA, who subsequently develop subaortic stenosis.
Clinically it is difficult to diagnose subaortic membrane.
Echocardiographically it may be missed because of its close
proximity to the aortic valve. One has to look carefully for
the fibroelastic membrane just below the aortic valve
(Figures 7.13A and B). Otherwise one ends up reporting
FIGURE 7.13A: The parasternal long axis view shows both

anterior and posterior insertion of a thin subaortic
membrane (arrow), demonstrated separately, simul-
taneously just below opened aortic valve
FIGURE 7.13B: Parasternal long axis view, with colour

comparison shows a thin subaortic membrane obscuring
and obstructing VSD
wrongly as just Left ventricular hypertrophy. Though on

echo it looks like small ridges, on necropsy they are much
more extensive. Therefore careful parasternal long axis
imaging as well as apical four chamber and five chamber
imaging may delineate the defect or sometimes trans-
oesophageal echocardiography may be necessary. Early
systolic closure of the aortic valve in M-mode gives a clue
to subaortic membrane. As treatment consists of complete
resection of the membrane along with a limited myomec-
tomy, it is important for an echocardiographer to evaluate
the extent of the diaphragm.
Fibromuscular Subaortic Fibromuscular Ridge

It is associated with fibrous ring associated with muscular
hypertrophy and located approximately 1 cm below the
aortic valve and extending downwards 1 to 2 cm causing
more diffuse area of left ventricular outflow obstruction
(Figures 7.14A and B) but also frequently encroaches on
the AML. Apical five chamber view may be useful adjunct,
because it places the membrane or ridge perpendicular to
the path of the scan plane, thereby enhancing the visuali-
zation. Doppler study to obtain the peak velocity is very
important and must be distinguished from the signal of
VSD and mitral regurgitation (MR).
Subvalvular Fibromuscular Collar or Tunnel

It produces a more extensive area of obstruction which is
characterized by an inward bowing of the echoes from the
anterior and posterior margins of the outflow tract
FIGURE 7.14A: Parasternal long axis view shows a thick

fibrous ring associated with muscular hypertrophy, located
approximately 1 cm below the aortic valve, part of the LVOT
above ring is seen. A small opening in the fibromuscular ring
is clearly seen indicate the stenosis
immediately beneath the aortic valve. There is more diffuse

obstruction extending well into the ventricle associated
with left ventricular hypertrophy. In this type there is a
dense fibroelastic endocardial tissue involving the entire
LVOT, annular hypoplasia, fibrous cusps is a major
surgical challenge. Therefore careful imaging in the
parasternal long axis and short axis is important to
delineate the true dimensions of the LVOT. The hypothesis
that discrete subaortic stenosis is a progressive disorder
and may develop into tunnel like subaortic stenosis is
documented by serial haemodynamic and angiographic
FIGURE 7.14B: Apical five-chamber view shows subaortic

fibromuscular collar/tunnel like subaortic stenosis caused by
dense fibromuscular ridge, seen as echo dense ridge
attached to ventricular septum and extends onto the anterior
mitral leaflet
investigations. One should not allow it to progress to type

II or III, as extensive myomectomy could lead to complete
heart block in type II or extensive resection, aortic valve
and/or root replacement is needed in type III.
Case Report
12 years old child presented with history of fever and
general body ache was diagnosed as a case of acute
rheumatic fever with carditis in a medical college, as the
child had a systolic murmur and tachycardia. The child
was put on steroids. When the condition deteriorated the

child was shifted to NIMHANS for history of convulsions
and was diagnosed to have tubercular meningitis and
treated appropriately. The cardiac evaluation with 2D Echo
showed subaortic membrane but no evidence of rheumatic
heart disease. The murmur caused by the subaortic
membrane was mistaken for Carey Coombs’ murmur of
rheumatic carditis! Hence echo is the best diagnostic tool
which can delineate the type of subaortic obstruction and
give information about gradient across left ventricular out
flow tract (LVOT), LV function, LV hypertrophy (LVH),
aortic regurgitation and associated anomalies. Echo not
only helps in accurate diagnosis but also assists in
management strategy. Hence it is very important for every
echocardiographer to know about this condition to prevent
mistakes like in the above case.
Point to Remember
Early detection of subaortic membrane is very important
to avoid LVH, LV dysfunction and extensive surgery.
Hence the onus lies on echocardiographer to detect the
subaortic membrane in time, when it can be treated with
simple excision by looking carefully just below the aortic
valve in both parasternal long axis and apical five-chamber
view.
PRIMARY ENDOMYOCARDIAL FIBROELASTOSIS (EMF)

Primary Endomyocardial Fibroelastosis is relatively a rare
condition with an estimated incidence of one case in 1000
to 6000 live births. The abnormalities characterized by
opaque whitish-grey thickening of the endocardium due

to proliferation of the collagenous and especially elastic
tissue. It is seen in infants without co-existing congenital
anomalies. 2D echo is a major tool in the diagnosis of
primary EMF. M mode echo may show increased bright
echoes in the endocardium from the dense collagen
deposition. 2D echo demonstrates the obliteration of the
apices of the ventricles (Figure 7.15A) and the presence of
thrombus, especially in the apical 4 chamber view; bright,
patchy echoes at the cavity surface suggest calcium and
there is evidence of involvement of the papillary muscles
and posterior AV valves, preserved ventricular contraction
and bilateral atrial dilatation are prominent. Doppler
studies show the abnormal haemodynamics and abrupt
premature cessation of ventricular filling and diastolic
FIGURE 7.15A: Apical 4-chamber view shows obliteration of the

apices of the ventricles, bright echoes of endocardium,
papillary muscle, posterior mitral leaflet and dilated large LA
and RA (both the atria are bigger than both the ventricles)
mitral and tricuspid regurgitation characteristic of the

reduced myocardial compliance with dilated IVC and its
tributaries (Figures 7.15B and C).
FIGURE 7.15B: Subcostal view shows grossly dilated

inferior vena cava and its tributaries in a case of EMF
FIGURE 7.15C: Pulse Doppler of IVC in 5 years old boy of EMF

ISOLATED DOUBLE CHAMBERED RIGHT VENTRICLE (DCRV)

DCRV is usually associated with VSD. Isolated DCRV is
very rare and is another variety of obstruction to right
ventricular outflow tract (RVOT). It is characterized by
aberrant hypertrophied muscular bands that divide the RV
cavity into a proximal high pressure chamber and distal
low pressure chamber located beyond the hypertrophied
muscle bands (Figure 7.16). These muscle bundles may be
formed by hypertrophied moderator band or markedly
hypertrophied trabacula septomarginalis. The fibro-
muscular diaphragm causes double chambered right
ventricle and the obstruction caused is sometimes
progressive and very severe. The echocardiography not
FIGURE 7.16: Subcostal apex down image shows anomalous

muscle bundle seen crossing the RVOT (indicated by an
arrow) beneath the pulmonary valve—a “napkin ring” type of
double chambered RV
only shows the dense echoes by the hypertrophied bands

but also the gradient across the chambers can be assessed,
focus on ventricular-morphology, ventriculo-arterial
connections and associated congenital malformations like
ASD, VSD, PAPVC, TAPVC, CTGV, PS, pulmonary atresia,
etc.
MITRAL ATRESIA AND CONGENITAL MITRAL STENOSIS

(CMS)
Mitral atresia is an extremely rare condition (less than 0.03%
incidence) characterized by a blind dimple in the floor of
the left atrium. On 2-D echo thick echogenic band (due to
fibromuscular tissue) is seen in the place where mitral valve
should have been present (Figures 7.17A and B). Congenital
FIGURE 7.17A: Apex down image shows large VSD

(almost like a single ventricle) with mitral valve atresia
FIGURE 7.17B: Modified view in a case of mitral atresia, VSD,

PS, normally related great arteries, aorta is seen arising from
LV and pulmonary artery identified by branching is ariging from
RV
FIGURE 7.18: Subcostal view shows dilated LA. IAS bulging

into RA. Single papillary muscle in LV in a 10-month infant with
COA, PDA and congenital MS
mitral stenosis (CMS) is a broad spectrum of abnormalities

of the mitral valve apparatus causing obstruction to the
blood flow from LA to LV. The incidence of CMS is 0.5 per
cent. The echo can define the anatomy of CMS accurately
(Figure 7.18). In parasternal long axis and apical four-
chamber view show LA enlargement, left to right bowing
of the interatrial septum, the chordae may be short and
hyper-echogenic, narrow or obliterated interpapillary space
or fusion into single papillary muscle. Pulse and colour
Doppler can estimate the severity of stenosis by trans mitral
gradient.
DOUBLE AORTIC ARCH (DAA)

Double aortic arch is a rare anomaly in which the
ascending aorta divides into right and left aortic arches
which join posteriorly to form the descending aorta. Each
arch provides separate origin to the ipsilateral common
carotid and subclavian arteries. In about 85 per cent of
cases the right arch is the larger (Figure 7.19A). Double
aortic arch is seen in less 1 per cent of all the CHDs, usually
associated with TOF, TGA, COA. Rarely associated with
ASD, VSD and LSVC. It constitutes a vascular ring around
the trachea and oesophagus and almost invariably
produces severe airway compression during infancy.
Severe feeding difficulties and respiratory symptoms are
due to DAA with PDA creating a tight vascular ring around
oesophagus and trachea. Surgical division of PDA gives
immediate and complete relief. It has to be distinguished
from truncus arteriosus (type I) in which pulmonary artery
FIGURE 7.19A: Modified parasternal long axis view shows the

aorta bifurcating into left aortic arch (LAOA) and bigger right
aortic arch (RAOA)in a 5-month-old infant
FIGURE 7.19B: High parasternal short axis view PDA in a

5-month-old infant who presented with stridor and hypoxia
was found to have DAA
arises from the side of truncus and normal pulmonary

valve and pulmonary artery are absent, whereas in DAA
apart from normal PV and PA, invariably there is a PDA
(Figure 7.19B) to complete the vascular ring.
CARDIAC MALPOSITIONS
Depending on visceroatrial situs and base to apex major
axis, there are three clinically important cardiac malposi-
tions in patients in whom the spleen is present and single:
i. Visceroatrial situs inversus with dextrocardia: It is
simply the mirror image of normal (Figures 7.20A and
B).
ii. Visceroatrial situs solitus (concordance) with
dextrocardia (isolated Dextrocardia): Though the
major axis of the heart (base to apex) points to the right
and the major cardiac shadow to the right of midline
(right hemidiaphragm is lower than or at the same
level as the left), the ascending aorta and aortic
knuckle occupy their normal border forming location
and the descending aorta runs its normal course
parallel to the left vertebral border. On echo in
subcostal view the morphological RV is seen to the
right and forms the apex (Figure 7.21A).
iii. Visceroatrial situs inversus with levocardia (Isolated
levocardia): Visceroatrial situs is inverted but the
cardiac mass is on the left (base to apex axis of the
heart points to the left). This is the rarest of the
malpositions of the heart. As the IVC, RA and RV are
on the left side and apex is formed by RV-mirror image
of isolated dextrocardia (Figure 7.21B).
FIGURE 7.20A: Schematic representation of situs solitus with

normal lie of the heart. The liver, IVC, RA, RV are to the right,
LA, LV, aortic knuckle, descending aorta, stomach and spleen
are to the left
Echocardiography helps in segmental analysis of

viscero-atrial situs, ventricular localization and
identification of the spatial relationships and ventricular
attachments of the great arteries. In subcostal short axis
abdominal view the atriovisceral situs is characterized by
the presence of the inferior vena cava and the major portion
of the liver to the right of the spine and the stomach,
FIGURE 7.20B: Schematic representation of situs inversus with

dextrocardia (mirror image of the heart).The liver, IVC, RA, RV
are to the left and LA, LV, aortic knuckle, descending aorta,
stomach and spleen are to the right
descending aorta to the left of the spine in situs solitus.

Identification of the aorta is confirmed by its systolic
pulsation and thick wall. A long axis scan visualizes
hepatic venous connection to the inferior vena cava and
identifies the course of the inferior vena cava to the right
sided morphologic right atrium. Visceroatrial situs
inversus is diagnosed when the major portion of the liver
FIGURE 7.21A: Schematic representation of situs solitus with

dextrocardia (Isolated dextrocardia) though the cardiac mass
is on the right side with base to apex major axis is to the right,
the suprahepatic IVC, RA, RV are to the right only. The LA, LV,
aortic knuckle and descending aorta are to the left, along with
stomach and spleen
and the inferior vena cava are visualized to the left of the
spine, and the stomach, descending aorta visualized to the
right of the spine, the long axis scan images hepatic venous
connections to the inferior vena cava and the course of the
inferior vena cava to the left side of the morphologic right
atrium.
FIGURE 7.21B: Schematic representation of situs inversus with

left thoracic heart, suprahepatic IVC, RA, RV are to the left. The
LA, LV, aortic knuckle and descending aorta are to the right,
along with stomach and spleen to the right
Once visceroatrial situs is established 2D echo imaging

should look for the other lesions that occur in dextrocardia
which are TGA, DORV, VSD, single ventricle, pulmonary
stenosis or atresia. But TOF in dextrocardia is extremely
rare. Dextrocardia with complete situs inversus occurs in
approximately 2 per 10, 000 live births but the associated
lesions is rare (3%).Whereas dextrocardia with situs solitus
FIGURE 7.22A: Subcostal view shows dextrocardia with

large VSD in a six years old girl
FIGURE 7.22B: Apex down image in a case of situs solitus with

dextrocardia with ASD shows RV to the right forming the apex
(Isolated dextrocardia) is considerably less common

(occurs 1 in 20, 000 live births), but incidence of associated
lesions is extremely high, almost 90 per cent of them have
other lesions (Figures 7.22A and B).
Points to Remember
Suprahepatic IVC, RA and RV are on the same side as the
liver. The LA, LV, aortic arch and descending aorta are on
the same side as the stomach.
Mesocardia
Mesocardia is sometimes considered a fourth malposition.
But in mesocardia (ventricular portion of the heart extends
equally from the midline) with situs solitus is practically
like a normal heart.Whereas mesocardia with situs
inversus is extremely rare and L-loop that stops in the
midline as it pivots to the right.
Points to Remember
The cross sectional echocardiography with segmental
approach makes the understanding and diagnosis of
complex cardiac lesions easy and accurate. The eyes will
not see what the mind does not know; hence knowledge
about rare anomalies is essential. As the echo report is like
a road map for the management, it is important that all
the lesions are identified and documented accurately.
Chapter 8
Foetal
Echocardiography
INTRODUCTION
Foetal echocardiography (FE) is a complete two dimen-
sional and Doppler ultrasound evaluation of the human
foetal cardiovascular system. It is completely noninvasive
and harmless for the foetus. Hence it has become the only
means that allows a complete evaluation of cardiac
structure and function. Foetal Echo (FE) also serves as the
electrocardiogram for the foetus and helps monitoring
during foetal life. Hence it is the most useful tool to detect,
diagnose and monitor arrhythmia in utero, which are now
manageable with transplacental therapy.
The earliest experience with 2D echo detection of
congenital heart disease (CHD) in foetus was reported in
1972 by Winsberg. Since then widespread use of general
foetal ultrasound examination among women receiving
prenatal care has resulted in increased referral for specific
cardiac analysis. Definition of foetal cardiac structure is
currently possible at 10 to 12 weeks of gestation with the
use of vaginal probes with high resolution transducers. By
16 to 18 weeks, accurate segmental analysis of cardiac
structure is possible with a conventional transabdominal
approach. The specificity is 99 per cent and the sensitivity
is over 80 per cent depending on the expertise of the
examiner.
The first level of foetal echocardiographic examination
is carried out by obstetrician or generic ultrasonographer
who screens routinely all normal and abnormal preg-
nancies. A second level foetal echocardiographic
FOETAL ECHOCARDIOGRAPHY 173
examination must be done for all expectant women who

present with any conditions listed below are indications
for foetal echocardiography:
INDICATIONS FOR FE
Class
1. Abnormal appearing heart on general
foetal ultrasound examination. I
2. Foetal tachycardia, bradycardia or
persistent irregular rhythm on clinical
or screening, ultrasound examination. I
3. Maternal/family risk factors for cardio-
vascular disease such as a parent,
sibling or first degree relative with
congenital heart disease. I
4. Maternal diabetes. I
5. Maternal systemic lupus eythematosus I
6. Teratogen exposure during vulnerable period. I
7. Other foetal system abnormalities
(including chromosomal) I
8. Performance of transplacental therapy or
presence of a history of significant but
intermittent arrhythmia. Reevaluation
examinations are required in these
conditions. I
9. Foetal distress or dysfunction of unclear
etiology. IIa
10. Previous history of multiple foetal losses. IIb
11. Multiple gestation. IIb

12. Low-risk pregnancies with normal
anatomic findings on ultrasound
examination. III
13. Occasional premature contractions
without sustained tachycardia or
signs of dysfunction or distress. III
14. Presence of a non-cardiovascular III
system abnormality when evaluation
of the cardiovascular system will not
alter either management decisions or
foetal outcome.
FEATURES OF FE
FEATURES
The FE has unique features like-
1. It serves as the ECG for the foetus. Since we cannot
obtain routine ECG for the foetus, we rely on ECHO
demonstration of atrial and ventricular wall motion
simultaneously in M-mode and use the temporal
relationship of the motions to determine rate and
rhythm, i.e. when atrial contraction precedes ventricular
contraction and the contractions are in one to one
relationship at a normal rate—the foetus is in sinus
rhythm (Figure 8.1).
2. The FE can be used to assess foetal well-being in a more
global sense. Umbilical and cerebral arterial and
umbilical venous flow patterns change with altered
foetal and placental vascular resistance and provide
important clues to foetal status, cerebral vessels dilate
FIGURE 8.1: Foetus in sinus rhythm
in the distressed foetus while peripheral and placental

vessels generally constrict (Figure 8.2). Serous cavities
can accumulate effusions (Figure 8.3) pericardial,
pleural effusion/ascites in a foetus with infection or
low cardiac out-put. Advanced level OB ultrasound is
capable of this type of assessment and is more
frequently used for this purpose. However foetal “well-
being” should be routinely assessed and commented
upon as a part of a complete FE.
HOW ACCURATE IS FE?

Most published reports describe a 10 to 15 per cent rate
of detecting structurally abnormal foetal hearts (when
using the high risk referral indications mentioned above).
In these published series false negative rates have been
FIGURE 8.2: Normal foetal circulation
FIGURE 8.3: Pericardial effusion and pleural effusion

very low (1-2%). False-positive occurs at similar rates.

Because of the small size of the structures being examined,
limitations exist. Pulmonary veins can be difficult to image.
Thus, anomalies of pulmonary venous drainage are one
major cardiac lesion not likely to be imaged reliably. Also,
because of the foetal flow patterns, coarctation of the aorta
can be difficult to diagnose reliably, although interruption
of the aortic arch can usually be distinguished. Also very
small VSD, any secundum ASD, mild forms of pulmonary
and aortic stenosis are difficult to detect. Therefore FE
reports tend to contain much greater percentage of complex
anomalies than we expect to see in the general newborn
population. As a result most common foetal diagnosis are
AV canal defects, TOF (Figure 8.4), single ventricle (Figure
8.5), Ebstein’s anomaly, transposition of great arteries,
FIGURE 8.4: Tetralogy of Fallot in 24 weeks fetus

FIGURE 8.5: Single ventricle
FIGURE 8.6: ASD with septal aneurysm

tricuspid atresia and hypoplastic left heart syndrome,

while the most common post-natal diagnosis tend to be
ASD, VSD, PS, PDA. The PDA is a normal finding
prenatally and cannot be diagnosed by FE. There is a
similar problem with ostium secundum ASD, since
foramen ovale is patent in utero (Figure 8.6).
EQUIPMENT
The requirements of FE are more stringent than for the
examination of an infant or child with congenital or
acquired heart disease. This is due to the increased
demands for both spatial and temporal resolution.
Anatomic surveys require axial resolution of 1 mm or less
and this is particularly important, given the small size of
critical foetal cardiac structures. Frames rates of 80 to 100
Hz are frequently needed to view important events
occurring at heart rates in excess of 140 beats per minute.
2-D, M-mode and all modalities of Doppler including
colour, pulse, high pulse repetition frequency, and
continuous wave should be available (Figure 8.7). Tissue
Doppler imaging has been recently applied in the assess-
ment of foetal arrhythmia. Compared to fundamental
imaging, harmonic imaging improves image quality and
visualization of cardiac structures in foetuses with
suboptimal echocardiographic windows (Figures 8.8A and
B). Harmonic imaging is useful when acoustic penetration
is difficult such as in the presence of maternal obesity, scar
tissue, polyhydramnios or other problems. Further phased
array transducers with fundamental frequencies between
4 and 12 MHz are generally used to improve the image.
FIGURE 8.7: Doppler study
FIGURES 8.8A and B: Comparison of (A) fundamental imaging

and (B) harmonic imaging in a 4-chamber view in a
suboptimal echocardiographic windows
EXAMINATION TECHNIQUES
Although the goal is to achieve visualization of each of the
essential components, not all will be visualized in every
foetus at every examination. Foetal position in the uterus
or increased activity may limit the ability to obtain
visualization of each of the components. The number of
vessels in the umbilical cord is counted and Doppler
sampling of the umbilical artery and umbilical vein is
performed. After establishing the position of the foetus and
the right/left and anterior/posterior orientation, an initial
survey of foetus is used to estimate the gestational age and
to establish abdominal situs and cardiac position. The
presence or absence of fluid in pericardial/pleural/
peritoneal space is noted. The position of inferior vena cava
and descending aorta at the level of the diaphragm are
noted. A systematic uniform method must be followed. All
studies begin with a wide-angle format to permit the
determination of foetal orientation heart within the thorax.
The diagrams demonstrate the anatomical correlates to the
tomographic imaging planes used for the views may be
utilized to image the various structures of the foetal heart
and accomplish a comprehensive foetal echocardiogram.
The four-chamber view is the single most useful view
of the foetal heart. Although it does not allow detection of
all defects, it allows determination of the more serious
defects. Before other areas of the heart can be examined,
a good four-chamber view is mandatory (Figures 8.9A and
B) as it permits examination of the atrioventricular valves,
atrial septum and inlet portion of the ventricular septum.
FIGURE 8.9A: Schematic representation of four-chamber view
FIGURE 8.9B: Apical four-chamber view

Most importantly, the septum primum is seen moving

within the left atrium because of flow across the foramen
ovale. Because the septum primum is always within the
left atrium, it serves as a marker for the left atrium, even
when there is atrioventricular discordance.
Next, the tricuspid and mitral valve can be distin-
guished because the mitral valve attaches to the crux of
the heart slightly more cephalad than the tricuspid valve.
Because the mitral valve is always within the morphologic
left ventricle, atrioventricular concordance or discordance
can be determined. Finally, abnormalities of the atrioventri-
cular junction resulting in atrioventricular septal defects
can be evaluated. Of particular importance is the diagnosis
of hypoplastic left heart syndrome in utero because of the
extremely poor prognosis. This is best diagnosed in the
four-chamber view and the view of the left ventricular
outflow tract where measurements of the aortic annulus
can be made and compared with normal. From the four-
chamber view, progressive angulation of the scan plane
in a ventral direction results in imaging the outlet portion
of the heart beginning with the left ventricular outflow
region. The aorta is seen to arise from the left ventricle
(Figures 8.10A and B).
Then the identity of the aorta is confirmed by following
its course and imaging the origin of the arch vessels
(Figures 8.11A and B). The ascending aorta and arch
vessels are easily seen by following the aortic arch as it
arcs cephalad to the heart. The transition between the
proximal arch and the descending aorta may be small,
because the flow through this region is low. It may be
FIGURE 8.10A: Schematic representation of five-chamber view
FIGURE 8.10B: Five-chamber view

FIGURE 8.11A: Schematic representation of ductal view
FIGURE 8.11B: Aortic arch with cephalic vessels
difficult to distinguish a normal small transverse arch and

a true coarctation. The systemic veins should be imaged
next. Entrance of the hepatic veins and the inferior vena
cava into the right atrium can be imaged (Figure 8.12). The
position of the inferior vena cava and descending aorta at
the level of the diaphragm are established.
FIGURE 8.12: Entrance of hepatic veins and IVC

into right atrium
Parasternal long-axis view with left ventricular outflow

tract (Figures 8.13A and B), short-axis sweep (cephalad
angling includes “3-vessel” view (Figures 8.14A and B).
Caval long-axis view, ductal arch view, aortic arch view,
Doppler examination of inferior and superior vena cava,
pulmonary veins, hepatic veins, ductus venosus, foramen
ovale, atrioventricular valves, semilunar valves, ductus
arteriosus, transverse aortic arch, umbilical artery,
umbilical vein, measurement of atrioventricular valve,
semilunar valve, diameter of main pulmonary artery
(MPA), ascending aorta (AO), branch pulmonary arteries
should be noted. Other lesions that have a very poor
prognosis in utero include atrioventricular septal defect
with complete heart block and pulmonary atresia with
FIGURE 8.13A: Schematic representation

of parasternal long axis view
FIGURE 8.13B: Parasternal long axis view

FIGURE 8.14A: Schematic representation of short axis view
FIGURE 8.14B: Short axis view
intact ventricular septum. Finally, lesions such as tetralogy

of Fallot and hypoplastic left heart syndrome, as well as
the cardiomyopathy associated with infants of diabetic
mothers, may progress in utero. Serial foetal echo
examinations should thus be performed when these
diseases are suspected.
FOETAL ARRHYTHMIAS
In recent years, echocardiography has become the most
useful means to detect, diagnose, and monitor foetal
arrhythmias as well as to direct the therapeutic mana-
gement of pregnancies complicated by foetal arrhythmias.
This application of echocardiography has been parti-
cularly successful for several reasons. Foetal ECGs are very
difficult to record and when they are recorded only the
ventricular depolarization is seen. Thus no information is
provided concerning the atrial rate or the sequence of
atrioventricular activation. The FE provides information on
the atrial rate, ventricular rate, and the atrial-ventricular
relationship and thus allows a precise determination of the
type of arrhythmia. A second reason for the success of
echocardiography is that it provides a technique to assess
the hemodynamic consequences of the arrhythmia.
Thirdly, the echo provides a method to detect associated
structural cardiac malformations. This information is
particularly useful since 15 per cent of all foetuses with
a persistent arrhythmia have a structural cardiac defect.
From such a recording, the atrial rate, the ventricular rate,
and the relationship between the atrial and ventricular
contractions can be determined in the same way an ECG
can be analysed.
BRADYARRHYTHMIAS
BRADYARRHYTHMIAS
Slow foetal heart rates are often the reason for referral for
an echo. The most common cause is complete heart block;
this must be distinguished from sinus bradycardia, which
has a much worse prognosis. Sinus bradycardias, defined

as rates below 100/min, are uncommon; when they occur,
they usually indicate foetal compromise. In the presence
of complete heart block or sinus bradycardia, careful
inspection of the anatomy is necessary as well as
evaluation for signs of in utero heart failure. Heart rate in
an older foetus is normally somewhat slower but should
remain above 100/min. Complete heart block, manifest as
a slow ventricular rate with a faster atrial rate can be noted
at any stage of gestation but is not necessarily present from
the time of conception. The heart block often becomes
manifest after 20 wk. The foetus must be observed for the
development of heart block when predisposing factors,
such as the presence of maternal collagen vascular disease,
are present. This does not have to take the form of overt
clinical disease in the mother but may be present only as
a positive serologic test. In addition, complete heart block
can be associated with certain structural diseases in the
foetus, such as L-transposition of the great arteries,
atrioventricular septal defects, and any anomaly of the
tricuspid valve. Foetuses with heart block should be
monitored closely with repeat echoes for development of
congestive heart failure. There is no proven in utero therapy,
and premature delivery must be considered when signs of
significant heart failure develop. The consequences of a
premature birth must be weighed against the possibility
of in utero foetal death. If multiple risk factors are present,
this possibility may be high.
TACHYARRHYTHMIAS
Arrhythmias with a rapid foetal heart rate in excess of 200/
min are the most common pathologic arrhythmias. The
tachyarrhythmias that have been recognized in utero
include supraventricular tachycardia, atrial flutter, and
ventricular tachycardia. Of these, supraventricular
tachycardia is the most common and can be recognized as
a foetal heart rate in excess of 200/min with 1:1 atrio-
ventricular conduction. Although supraventricular tachy-
cardia is usually not associated with structural disease, a
careful inspection of the heart is needed, because the
haemodynamic effects tend to be worse in those foetuses
with cardiac defects. Also, when anomalies of the tricuspid
valve are present, the presence of Wolff-Parkinson-White
syndrome is more likely and may have some influence
upon the form of therapy used. Finally, the examiner
should try to determine if the tachycardia is incessant or
intermittent. The intermittent form tends to have less serious
haemodynamic effects. Again, the foetus must be examined
for signs of heart failure, such as cardiac enlargement or
hydrops, and frequent serial examinations are indicated
to determine whether heart failure is present.
Atrial flutter can be recognized by a more rapid atrial
rate and usually by the presence of a slower ventricular
rate. In our experience, atrial rate is usually greater than
400/mm, which distinguishes atrial flutter from supra-
ventricular tachycardia with associated atrioventricular
block. Although atrial flutter is usually not associated with
structural disease, structural anomalies must be excluded.

The presence or absence of heart failure depends not only
upon the ventricular rate but also upon the degree of effect
of the arrhythmia on ventricular filling. More chaotic atrial
rhythms tend to have a more pronounced effect. It is
unknown if this is caused entirely by altered filling in a
less compliant ventricle or if other factors contribute.
Ventricular tachycardia can be difficult to recognize.
The diagnostic feature is atrioventricular dissociation with
the ventricular rate faster than the atrial rate In general,
the ventricular rate is 160 to 200/min, although it can be
quite variable. When retrograde conduction is present,
resulting in a 1:1 atrioventricular relationship, the
diagnosis of ventricular tachycardia can only be inferred.
One clue is the presence of variability in the foetal heart
rate. In the presence of ventricular tachycardia, the foetal
heart rate is quite stable with loss of the normal variability.
This is particularly true in late gestation and can be
detected as a lack of heart rate variability on a non-stress
test. Ventricular function must be assessed because of the
association of ventricular tachycardia and viral myo–
carditis. Effects of this arrhythmia upon cardiac haemo-
dynamics are quite variable. In our limited experience, the
presence of atrioventricular synchrony leads to less
haemodynamic compromise. Thus, even if the arrhythmia
cannot be completely stopped, slowing it enough to restore
atrioventricular synchrony can lead to significant
improvement.
EXTRACARDIAC ANOMALIES
The frequency of structural cardiac defects in the presence
of various other anomalies is variable, depending upon the
organ system involved. The incidence can range from as
low as 2 to 5 per cent when central nervous system
malformations are present and as high as 50 per cent when
there are anomalies of the renal system. However, even
when the lesser-associated organ systems are involved, the
incidence of cardiac defects is still increased significantly
enough over the incidence in the general population that
foetal echocardiography is warranted.
In addition, the examiner should be aware of certain
extracardiac anomalies that have a high association with
specific forms of congenital cardiac defects. The most
common association is that of omphalocele and either a
ventricular septal defect or tetralogy of Fallot. The
incidence can range from 10 per cent if the caudal fold is
involved to nearly 100 per cent if the cephalic fold is
involved. Abnormalities of splenic formation (asplenia or
polysplenia) are commonly associated with atrioventri-
cular septal defects. In the presence of asplenia, right atrial
isomerism may also be present, and abnormalities of
pulmonary flow and pulmonary venous drainage may
exist. In the presence of polysplenia, left atrial isomerism
may exist with an associated increase in systemic venous
anomalies. In addition, an increased incidence of arrhy-
thmias presumed to be caused by altered sinoatrial node
development may be present. Whenever a chromosomal
FIGURE 8.15: AV canal defect
anomaly is detected by amniocentesis, FE is indicated,

because the association with congenital heart disease is
high. Nearly 40 per cent of Down’s syndrome have AV
canal defect. Therefore whenever FE shows AV canal defect
(Figure 8.15), Karyotype study should be done
(Figure 8.16) to confirm the diagnosis, before genetic
counselling or contemplating on medical termination of
pregnancy. There is a dictum which states that if an
anomaly is detected, one must look for a second; if one finds
a second, one must search for a third. This is particularly
true for congenital heart defects, as they often occur in the
context of aneuploidy. Even in the absence of chromosomal
abnormalities or genetic syndrome complexes, certain extra
cardiac anomalies such as cleft lip/palate, spina bidifa,
tracheo-esophageal fistula, duodenal atresia, omphalo-
FIGURE 8.16: Karyotype – 47xy+21 in Down’s syndrome
coele, diaphragmatic hernia, nuchal edema or a single

umbilical artery should make one suspicious for a possible
associated cardiac defect.
WHAT TO DO WITH THE INFORMATION GAINED FROM FE?

If FE is normal, reassure high risk families that major
cardiac anomalies are absent and the baby is probably
normal from cardiovascular stand point. If FE detects major
defect, then counselling and preparation of the family for
expected congenital heart disease is done. In complicated
cases, this will usually involve a multidisciplinary team
(e.g. obstetrician, neonatologist, paediatric cardiologist and

genetician).
a. High risk lesions delivery in the center where definitive
care of the newborn can be provided is advised.
Because it is always safer to transport a healthy
pregnant mother than a sick neonate.
b. Low-risk lesions (i.e. not duct dependent)—delivery can
occur locally with foreknowledge of how the child is
likely to “behave” in the neonatal period.
c. High risk lesions with limited long-term prognosis—
usually multiple congenital anomalies (Trisomy 13,
18)—offer counselling, amniocentesis and explore all
therapeutic options like intervention, comfort care,
termination of pregnancy.
d. Can monitor foetal “well being”in selected situation
like, foetal heart block, diaphragmatic hernia, chronic
indomethacin tocolysis.
FUTURE IMPACT OF FE
1. May reduce the frequency of newborns with severe
CHD.
2. Increasing frequency of prenatal interventions.
3. Increased prenatal “patient” transfer to centre of
excellence.
4. Uncertain impact on the prevalence of complex
CHD.
CONCLUSION
For high-risk pregnancies, early foetal echocardiography
can effectively identify abnormal hearts. In such a highly
sensitive area of diagnosis, reassuring parents is of

paramount importance and the need for follow-up
echocardiography to confirm initial scan results. Foetal
cardiology represents one of the most exciting and rapidly
evolving areas in the field of pediatric cardiology. During
the next 10 to 20 years, we expect to see incredible advances
in the prenatal diagnosis and management of CHD.
Increased ability to intervene medically, percutaneously
and perhaps surgically on the fetus has enhanced the
importance of prenatal detection of CHD. Foetal echo has
opened a new horizon and is the best tool in this direction.
FUTURE
The technology of echocardiography has grown by leaps
and bonds and has become an essential part of diagnosis
and surgical/non-surgical management of congenital
heart diseases (CHD). So much so that it has almost
threatened the supremacy of catheterization and angio-
gram, which is considered as a gold standard for diagnosis
especially of complex congenital heart diseases. Today 2-
D echo along with colour doppler, transoesophageal echo,
fetal echo, intracardiac echo and 3-D echo have made the
work of an interventional cardiologist and cardiac surgeons
so much more easy. In many centers, cross-sectional
echocardiography has replaced cardiac catheterization
and angiography as the primary diagnostic tool, and
cardiac operations are planned and executed on the basis
of echocardiography. The transoesophageal approach has
now been extended to very small infants with smaller multi

plane probes used in infants of 2 to 3 kg and this
intraoperative approach has dramatically reduced the need
for immediate postoperative reoperation, morbidity
and mortality. Fetal echocardiography has provided a
new frontier for diagnosis, counseling and treatment of
CHD.
In the neonates and children, echo window is excellent
and gives every details of the heart, whereas catheteri-
zation and angiography under general anesthesia carry
high incidence of morbidity and rarely mortality in very
sick children. Hence, non-invasive Echo which is cost
effective, safe, reproducible has replaced invasive cardiac
catheterization in decision making in congenital heart
disease in many cases.
Index
Note: Letter f refer to figure
A parasternal long axis, sub-

aortic membrane
Acyanotic CHDs 46 obstructing VSD
American Society of 80 f
Echocardiography rupture of sinus of
(ASE) options 2 valsalva (RSOV)
apex down imaging 2 76
apex up imaging 2 short axis, aneurysm of
Aortic stenosis 82 sinus of valsalva
doppler, transaortic 78 f
gradient, from short axis, large AP-
suprasternal notch window with COA
84 f 77 f
M mode, eccentric aortic Atrial septal defect 46
orifice 84 f apex down image, apical
M mode, hypertrophied four-chamber,
septum 83 f ostium secundum
parasternal long axis view, ASD 47 f
gross hypertrophy apex down image, high
of LV 83 f sinus venosus type
Aortopulmonary window (AP- ASD 50 f
window) 76 apical four-chamber view,
colour doppler RA 48 f
comparison, RSOV colour comparison, high
to RV colour ASD with right
doppler in VSD pulmonary vein
79 f draining into right
colour doppler in parasternal atrium 50 f
short axis view, contrast echo,
aortic valve, large microbubbles enter
communication 77 f LA from patent
parasternal long axis view, foramen ovale
ROSV 79 f 55 f
intracardiac TAPVC 54 f branching pulmonary
M-mode, paradoxical artery 27 f
septal movement concordant 22
49 f concordant and discordant
parasternal long axis view, connections of
dilated coronary chambers 23 f
sinus 53 f connections 20
parasternal long axis view, discordant 23
dilated RV malposition 26
compressing LV over-riding 20
54 f parasternal long axis view,
septal aneurysm 51 f aortic root and
sinus venosus defects 48 pulmonary trunk
27 f
subcostal abdominal view,
parasternal long axis view,
amplatzer septal
large subaortic
occluder 52 f
VSD 21 f
subcostal view, whole
straddling 20
length of IAS and
subxyphoid echo images,
fossa ovalis 47 f
large subaortic
TEE, deficient aortic rim VSD 21 f
52 f transposition 26
types of straddling and
over riding 22 f
C univentricular heart 23
Colour flow mapping 12
Cardiac and extracardiac colour comparison in
mass 85 parasternal long
Cardiovascular segments 20 axis shows small
aorta, arising from VSD 13 f
trabeculated short axis view, small
morphological RV subpulmonic VSD
26 f 13
apex down image, double small VSD, short axis
inlet SV 24 f view, below the
apex down image, mitral pulmonary valve
valve atresia 25 f 12 f
apical four-chamber view, Congenital heart diseases
dense band of (CHD) 134
echoes 25 f anomalous left coronary
apical four-chamber view, artery from
LA with pulmonary pulmonary artery
vein 24 f (ALCAPA) 145
INDEX 201
aortico-left ventricular tunnel bicuspid aortic valve,
(ALVT) 137 subaortic membrane
apex down image, colour 149 f
doppler blood cardiac malpositions 163
flowing fenestration congenital subaortic
in the membrane in obstruction 148
LA 142 f cor-triatriatum sinistra 140
apex down image, discrete subaortic
fenestration in the membrane 148
membrane in LA double aortic arch (DAA)
142 f 161
apex down image, echo with colour doppler,
fibromuscular device in situ, with
membrane 141f no residual flow
apex down image, large 139 f
VSD 159 f echo, prominent ventricular
apex down image, situs trabeculations,
solitus with intertrabecular
dextrocardia with recesses of
ASD 168 f myocardium 135 f
apical 4-chamber view, fibromuscular subaortic
obliteration, apices
fibromuscular ridge
of the ventricles
152
156 f
high parasternal short axis
apical five-chamber view,
view, PDA with
subaortic
stridor and
fibromuscular
hypoxia 162 f
collar/tunnel like
isolated double chambered
subaortic stenosis
154 f right ventricle
apical four-chamber view, (DCRV) 158
ASD with LA, membrane that
supravalvar mitral divides the left
ring 143 f atrium into a
apical four-chamber view, distal chamber
ASD, membrane, 144 f
DC with MV and mesocardia 169
LAA and PC with mitral atresia and
PV and ASD 141 f congenital mitral
apical four-chamber view, stenosis
dilated LV 146 f (CMS) 159
modified parasternal long parasternal short axis
axis view, aorta view, with the
bifurcating into left beam directed
aortic arch 162 f toward the base,
modified parasternal long no left coronary
axis view, large artery from left
VSD with sub- coronary cusp,
aortic membrane abnormal coronary
narrowing LVOT towards PA 147 f
149 f primary endomyocardial
modified view, mitral fibroelastosis
atresia, VSD, PS (EMF) 155
160 f pulse doppler of IVC,
non-compaction of left EMF 157 f
ventricle or “spongy quantifying the depth of
myocardium” 134 penetration
parasternal long axis view, intertrabecular
anterior and recesses 136 f
posterior insertion, situs invertus, dextrocardia
thin subaortic 165 f
membrane 151 f situs invertus, left thoracic
parasternal long axis view, heart, IVC, RA, RV
extent of tunnel 167f
between the aorta situs solitus, dextrocardia
and LV 138 f 166 f
parasternal long axis view, situs solitus, normal heart
thick fibrous ring 164 f
153f subcostal apex down image,
parasternal long axis view, anomalous muscle
thin subaortic bundle 158 f
membrane subcostal view,
obscuring and dextrocardia 168 f
obstructing VSD subcostal view, dilated LA
151 f 160 f
parasternal short axis subcostal view, grossly
view, appropriate dilated inferior
transducer rotation, vena cava,
retrograde flow tributaries, EMF
147 f 157 f
parasternal short axis subvalvular fibromuscular
view, tunnel 138 f collar 152
INDEX 203
suprasternal view, colour apex down image, aorta
doppler, COA with and pulmonary
subaortic membrane artery arising from
and bicuspid aortic morphologic right
valve 150 f ventricle 130 f
Continuous wave (CW) apex down image, aorta
doppler 6 arising from left
Coronary arteriovenous sided outlet
fistulas 80 chamber and
doppler, continuous, pulmonary trunk
turbulent, systolic (bifurcating) 123 f
and diastolic flow apex down image, large
pattern 82 f trunk overriding
VSD 99 f
parasternal short axis
apex down image, right
view, dilated right
sided outlet
coronary artery,
chamber with non-
aneurysmally
restrictive bulbo-
dilated fistulous ventricular foramen
tract 81 f 122 f
Corrected transposition of apex down image,
great vessels subcostal view,
(CTGV) 67 dilated superior
apical four-chamber view, vena cava 109 f
LA with apex down image,
pulmonary veins, transposition of
septal aneurysm, great arteries 104 f
AV discordance apical 4-chamber view,
68 f septal tricuspid
Cor-triatriatum dexter 144 leaflet adherent to
CW doppler 8, 10 IVS 127 f
Cyanotic congenital heart disease apical four-chamber view,
(CCHD) 94 dense band of
echoes 102 f
aortic atresia (hypoplastic
apical four-chamber view,
left heart syndrome
gross dilatation of
—HLHS) 114 RA and RV with
apex down image both left pericardial effusion
atrium and right 131 f
atrium, connected apical four-chamber view,
to one ventricle RA, RV dilated
120 f and LV 112 f
apical two-chamber view, modified parasternal long
branching vessel axis, main
pulmonary artery pulmonary artery
105 f 100 f
AV canal defect (endocardial modified parasternal long
cushion defects) 117 axis, small LV,
color doppler echo, AV dilated RV,
valve regurgitation pulmonary
125 f regurgitation with
colour doppler in short HLHS 116 f
axis, pulmonary modified view, arching
stenosis and vessel aorta from
pulmonary dilated morpholo-
regurgitation 97 f gical right ventricle
colour doppler, turbulence 105 f
in vertical vein parasternal long axis view,
110 f altered left
complete transposition of ventricular
great arteries (D- geometry, cavity
TGA) 104 size 128 f
CW doppler, associated parasternal long axis view,
pulmonary stenosis dilated RV,
103 f compressed smal
CW doppler, continuous LV with common
signals in associated chamber 112 f
collaterals 103 f parasternal long axis view,
highly trabeculated
CW doppler, on PA 101 f
single ventricle
double outlet left ventricle
with single AV
(DOLV) 129
valve 121
double outlet right ventricle
(DORV) 129 mitral to aortic
Ebstein’s anomaly 125 discontinuity 130 f
echo, “whip like” or “sail parasternal long axis view,
like” large elongated single ventricle,
anterior tricuspid common AV valve
leaflet 128 f 121 f
M-mode imaging, increased parasternal long axis,
excursion of absence of echoes
anterior tricuspid from any part of
leaflet 126 f left atrium 124 f
INDEX 205
parasternal long axis, subcostal view, ASD,
colour doppler dilated RV, RA,
flow through small LV,
hypoplastic mitral pulmonary veins
valve 115 f not draining into
parasternal long axis, LA 113 f
echocardial subcostal view, dilate RV,
fibroelastosis 115 f RA, small LV,
parasternal long axis, mitral hypoplastic MV,
to aortic disconti- ASD with R-L
nuity 108 f shunt 116 f
parasternal long axis, suprasternal short axis,
mitral to truncal upper part of
continuity, large figure of eight,
VSD 99 f associated PDA
parasternal long axis, the 109 f
malaligned TAPVC-figure of eight
infundibulum appearance shown
septum, large VSD in echo 113 f
95 f tetralogy of fallot 94
pulsed doppler, antegrade total anomalous
systolic flow due pulmonary venous
to stenosis 98 f connection 108
rudimentary anterior- tricuspid atresia 101
superior outlet truncus arteriosus 96
chamber 122 f typical cup and sausage
short axis in TGA 107 f appearance in
short axis, echo-dense short axis, RVOT
ridges projecting 106 f
into lumen 97 f univentricular heart (single
short axis, quadricuspid ventricle—SV) 119
truncal valve with
truncal regurgitation D
100 f
short axis, right coronary Doppler effect 7
artery from left Doppler ultrasound 6
coronary cusp 95 Double outlet right ventricle
single atrium (common (DORV) 20, 28
atrium) 123
subcostal four-chamber E
view, interatrial
and interventricular 2-D ECHO 3
communications in apical four-chamber
119 f view 4 f
in high parasternal short pericardial effusion and
axis view 5 f pleural effusion 176
in parasternal long axis short axis view 188 f
view 3 f single ventricle 178 f
in parasternal short axis tachyarrhythmias 191
view 5 f tetralogy of fallot, 24
in subcostal cardiac view weeks fetus 177 f
4 f
I
F
Infiltrative cardiomyopathy
Foetal echocardiography (FE) 85
172 2-D echo, extracardiac
aortic arch with cephalic mass compressing
vessels 185 f the left ventricle
apical four-chamber view 87 f
182 f apical four-chamber view,
ASD with septal aneurysm hyperechogenic
178 f mass RV compres-
AV canal defect 194 f sing the LV 91 f
bradyarrhythmias 189 apical four-chamber view,
doppler study 180 f
obliteration apex of
ductal view 185 f
both RV and LV
entrance of hepatic veins
91 f
and IVC into right
doppler echo, mitral inflow
atrium 186 f
velocity 92 f
equipments 179
extracardiac mass
extracardiac anomalies 193
features of 174 compressing the
five-chamber view 184 f LV 89 f
foetal arrhythmias 189 extracardiac mass with
foetus in sinus rhythm 175 pericardial effusion
how accurate is FE 175 89 f
indications for 173 large pericardial effusion,
karyotype, Down’s metastasis 87 f
syndrome 195 f large vegetation, congenital
normal foetal circulation bicuspid valve 88 f
176 f LV mass filling the LV
parasternal long axis view extending upto
187 f aortic valve 90 f
INDEX 207
modified view, marked parasternal short axis, long
thickening left fingerlike LA
ventricular appendage 17 f
posterior wall and
reduction, cavity of
LV 86 f P
marked thickening Patent ductus arteriosus
of left ventricular (PDA) 69
posterior wall and colour doppler, amplatzer
septum 86 f device not causing
parasternal long axis, obstruction to left
hyperechogenic pulmonary artery
mass in RV 75 f
compressing the CW doppler, continuous
LV 90 f signals 71 f
parasternal long axis, large high ductal view with
left atrial myxoma colour doppler,
obstructing mitral flow from ampulla
orifice 88 f 72 f
parasternal long axis, parasternal short axis
reduced LV cavity view, aortic valve
92 f shows fuzzy
rhabdomyoma, infant 90, echoes in main
91 pulmonary artery
75 f
parasternal short axis,
M aorta 69 f
M-mode with Ebstein’s large tubular PDA
anomaly 6, 7 72 f
PDA 70 f
N short axis, the coil used to
close the small,
Normal heart 16 large PDA 74 f
apical four chamber view, suprasternal notch view,
interventri-cular COA 73
septum 16 f suprasternal notch view,
parasternal long axis view, with PW doppler,
mitral to aortic gradient across
continuity 17 f COA 74 f
Pulse doppler 9 parasternal long axis view,
Pulse wave doppler 10 mitral to aortic
PW doppler, descending aorta discontinuity in
in CoA 11 f TGA 40 f
PW doppler, inferior vena situs or sidedness 31
cava 11 f situs solitus—aorta 31 f
subcostal abdominal view,
suprahepatic
S
portion IVC 37 f
TGA, the great arteries
Segmental approach CHD 30
apex down image, and aorta 41 f
ventriculoarterial ventriculoarterial
concordance 39 f discordance 42 f
apical four-chamber view, visceral situs 32 f
RV hypertrophy Suprasternal CW doppler 9
42 f
atrial morphology 36
atrial segment 36
V
atrial situs or sidedness 33
atrial-ventricular Ventricular septal defect
connection 38 (VSD) 56
atrioventricular, apex down image, large
ventriculoarterial single mid-muscular
discordance 38 f VSD 57 f
cardiac orientation 35 apex down image, large
diagnosis, rare combination subaortic VSD 56 f
of complex apex down image,
congenital multiple small
anomalies 43 swiss cheese
IAS profiled subcostal
defects 58 f
views, thin valve of
apical four-chamber view,
the oval fossa 34 f
internal cardiac crux in amplatzer septal
concordant and occluder in situ 66 f
discordant AV apical four-chamber view,
connection 39 colour doppler,
normal, mirror-image, right device in situ 66 f
isomerism, left apical four-chamber view,
isomerism of atria colour doppler,
33 f small VSD 61 f
INDEX 209
apical four-chamber view, echo with colour doppler,
large peri- residual shunt with
membranous VSD echogenic surgical
with septal patch 67 f
aneurysm 60 f parasternal long axis view,
apical four-chamber view, ‘T’ artifact 59 f
large VSD and large
ASD 64 f
colour doppler,
apical four-chamber, colour
severe aortic
doppler, large VSD
with bidirectional regurgitation 63 f
shunt 64 f parasternal long axis view,
colour compare in short prolapse of the
axis, 2 mm VSD aortic cusp 62 f
59 f parasternal long axis,
CW doppler, trans-VSD bacterial
gradient 61 f endocarditis 65 f

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Step by Step

Step by Step Echocardiography in Congenital Heart Diseases

First Edition: 2006

Typeset at JPBMP typesetting unit

It gives me great pleasure to write this

A precise and complete diagnosis of

entitled Step by Step Echocardiography in Congenital Heart

There are many systematic text-

apex down imaging is not familiar to many Echocardio-

1. Basics and Technical Background ........................... 1

Historically, the emergence of echocardiography (ECHO)

The ECHO gives complete information about the anatomy

FIGURE 1.1A: ECHO picture in parasternal long axis view

FIGURE 1.1B: Diagrammatic representation and echo

FIGURE 1.1C: ECHO picture in subcostal cardiac view

FIGURE 1.1D: Echo picture in parasternal

FIGURE 1.1E: ECHO picture in high

children, lack of attenuation from the rib cage permits

CONTINUOUS WAVE (CW) DOPPLER

FIGURE 1.2: M-mode in 12 years old girl with Ebstein’s anomaly

FIGURE 1.3: Diagrammatic representation of Doppler effect

FIGURE 1.4: Diagrammatic representation of velocity display,

FIGURE 1.5A: Diagrammatic representation of CW Doppler

FIGURE 1.5B: Suprasternal CW Doppler, 14 years old boy with

The shape of the wave form and the density of the

FIGURE 1.6: CW Doppler in a case of TOF with absent pul-

FIGURE 1.7A: Diagrammatic representation of

FIGURE 1.7B: PW Doppler of inferior vena cava

FIGURE 1.7C: PW Doppler in descending aorta in CoA

COLOUR FLOW MAPPING

FIGURE 1.8A: Small VSD seen in short axis view,

FIGURE 1.8B: Colour comparison in short axis view

FIGURE 1.8C: Colour comparison in parasternal

It is very pertinent to know the normal heart to detect the

FIGURE 2.1: Apical four chamber view shows interventricular

FIGURE 2.2: Parasternal short axis shows long fingerlike

FIGURE 2.3: Parasternal long axis view shows mitral to

is attached slightly lower than the mitral valve (Figure

It is very essential to know the terms used to describe the

Figure 3.1: Parasternal long axis view shows large

Figure 3.2: Subxyphoid echo images, Large subaortic VSD,

FIGURE 3.3: Diagrammatic representation of types of

Point to remember: An atrioventricular valve can both

FIGURE 3.4: Diagrammatic representation of concordant

FIGURE 3.5: Apical four-chamber view shows LA with pulmonary

FIGURE 3.6B: Apical four-chamber view shows a dense band

FIGURE 3.6C: Apex down image shows mitral valve atresia,

FIGURE 3.7A: Aorta is seen arising from

FIGURE 3.7B: Branching pulmonary artery is seen arising

are always malpositioned in double outlet right ventricle

Two-dimensional echocardiography is ideally suited

FIGURE 4.1: Situs solitus—aorta(red) to the left and

FIGURE 4.2A: Diagrammatic representation of visceral situs

FIGURE 4.2B Diagrammatic representation of normal,

ATRIAL SITUS OR SIDEDNESS

connection abnormalities. Sometimes this term is used to

FIGURE 4.3: The IAS profiled subcostal views shows thin

The atrium to which the coronary sinus and hepatic

Levocardia—base to apex axis is “pointed” from upper

Levoposition—most of the cardiac mass is to the left of

Dextroposition—most of the cardiac mass is the right of

Mesocardia—base to apex axis is “pointed” nearly directly

Venous segment—includes both the systemic, pulmonary

• Systemic—SVC, IVC and hepatic veins.