- Dr.

Akif Baig
Epidemiology
 Ventricular septal defects occur either as an isolated defect or as a
component of a more complex lesion

 It occurs in 50 percent of all children with CHD and in 20 to 30 percent as

an isolated lesion

 Most common congenital cardiac anomaly in children

 Second most common congenital abnormality in adults, second only to

bicuspid aortic valves

 They are more common in premature infants and those born with low
weight

 VSDs are slightly more common in females (56%)

 Most common congenital cardiac anomaly in children

 Second most common congenital abnormality in adults,

second only to bicuspid aortic valves
 They are more common in premature infants and those
born with low weight

 VSDs are slightly more common in females (56%)

Anatomy
 The IVS is a complex
curvilinear, non-planar
intracardiac partition

 The normal ventricular

septum is mostly
muscular with a small
fibrous portion, the
membranous septum
 The four regions are the inlet septum, trabecular septum,
outlet or infundibular septum (together making up the
muscular septum), and

 The Membranous Septum

 The inlet septum is smooth walled and extends from the
septal attachment of the tricuspid valve (TV) to the
distal attachments of the tricuspid tensor apparatus

 The inlet septum separates the septal cusps of the mitral

and tricuspid valves
The trabecular portion separates
the body and apices of the two ventricles

It extends from the

attachments of the tricuspid leaflets outward to the apex and
upwards to the crista supraventricularis
The smooth-walled outlet or infundibular septum, extends from the
crista to the pulmonary valve

The outlet septum separates the outlets

of the ventricles.
 The membranous septum is further divided by the septal
leaflet of the TV into atrioventricular and interventricular
components
 ANATOMICAL
CLASSIFICATION
 Many classifications of VSDs have been proposed

 Soto et al classified VSDs depending on their location in

the IVS as seen from the right ventricular side

 They are divided into four types of defects:

 Perimembranous
 Muscular
 Outlet
 Inlet
Perimembranous defects
 Infracristal, subaortic, membranous

 They are the most common and

account for 80 percent of all VSDs

 These defects involve the

membranous septum with extension
into the adjacent inlet, outlet or
muscular septum

 They lie in the outflow tract of the

left ventricle (LV), immediately
beneath the aortic valve
 There is fibrous continuity between the aortic and
tricuspid valves

 The conduction bundle is always found in the

posteroinferior margin of the defect

 In perimembranous VSD, rarely LV to right atrium (RA)

shunt (Gerbode defect) may be seen
Classification of Gerbode defect
 Gerbode defects can be classified according to location :

 Supravalvular (direct): defect is superior to the septal

leaflet of the tricuspid valve

 Infravalvular (indirect): defect is inferior to the septal

leaflet of the tricuspid valve

 Intermediate: both inferior and superior defects are

present in relation to the septal leaflet of the tricuspid valve
Muscular defects (trabecular)
 They account for 5 to 20 percent of all the VSDs

 They are entirely bounded by the muscular septum and are

often multiple, when viewed from the right side

 Kirklin et al further subclassified them depending on their

location in the muscular septum as:
 Anterior
 Midmuscular,
 Apical
 Posterior.
 When multiple muscular defects are seen, it is often referred
to as ‘Swiss cheese’ type of VSDs

 Swiss cheese defects cannot close spontaneously

 Subarterial or Outlet defects
 Supracristal, conal, infundibular,
subpulmonary, doubly committed
subarterial, doubly committed juxta-
arterial

 5 to 7 percent of VSDs

 Situated just beneath the pulmonary

valve and communicate with the right
ventricular outflow tract above the
supraventricular crest
 The incidence is as high as 30 percent in Asian populations

 This defect frequently leads to prolapse of the right coronary

cusp or less likely the noncoronary cusp of the aortic valve
causing aortic regurgitation (AR)
Inlet defects
 Canal type, endocardial
cushion type, atrioventricular
septum type, juxtatricuspid)

 These VSDs account for about

8 percent of all the VSDs

 They are located posteriorly

and inferior to the
membranous septum
VSD Classification based
on Hemodynamics
 Small or restrictive Moderate or Large or Non
Moderately restrictive VSD
restrictive defects

Size Less than one-third of 1/3–2/3 of the size >2/3

the size of the aortic of the aortic root
root

Pulmonary/Aortic <0.3 < 0.66 >0.66

systolic pressure
ratio

Qp/Qs < 1.4:1 > 1.4–2.2:1 >2.2:1

LV volume overload Minimal Present Present
Tendency to Nil Minimal Markedly
develop increased
PVR

Chances of Yes Yes Yes

Infective
Endocarditis
 Small or restrictive Defects
 The small VSD is less than one-third of the size of the aortic root or the
orifical area is <0.5 cm²/m²

 It is also called restrictive as the size of the defect limits the left to right shunt
and there is a significant pressure gradient between the LV and RV

 The pulmonary/aortic systolic pressure ratio is <0.3 with a small shunt

(Qp/Qs < 1.4:1)

 The degree of LV volume overload is minimal

 There is no tendency to develop increased PVR

 But they can develop AR or bacterial endocarditis

 Moderate or Moderately Restrictive
Defects
 The VSD size is said to be moderately restrictive, when it is 1/3–
2/3 of the size of the aortic root or the orifical area is > 0.5
to 1 cm2/m2

 They are large enough to permit a moderate shunt, yet small

enough to offer some resistance to flow

 The pulmonary/aortic systolic pressure ratio is < 0.66

with a moderate shunt (Qp/Qs > 1.4–2.2:1)

 The peak systolic pressure difference is ≥ 20 mm Hg

between the two ventricles
 They develop moderate left to right shunt leading to
volume overload of the left sided cardiac chambers
causing LV and left atrium (LA) dilatation and hypertrophy

 The RV is not dilated and RV and pulmonary artery pressures

may remain low or be moderately elevated

 The PVR is low, but variable and rarely progresses to PH

Large or Nonrestrictive VSD
 The VSD is large, when it measures more than 2/3 of the size of the
aortic root or the orifical area is ≥1 cm2/m2

 It is also called nonrestrictive VSD as there is no resistance to flow

across the defect

 The pressures in the ventricles are equal and they function as a common
pumping chamber with two outlets

 The degree of left to right shunt is dependent on the relationship

between the pulmonary and systemic vascular resistance

 The pulmonary/aortic systolic pressure ratio is >0.66 with a

large shunt (Qp/Qs > 2.2:1)
 In infants with moderate or large VSDs the decline in the
PVR may be delayed for several months

 At about 4 to 6 weeks of life, the large left to right shunt

causes increased PBF and subsequently increases pulmonary
venous return into the LA and ultimately into the LV

 This leads to dilatation of LA and LV and increased LV end-

diastolic pressure
 The pulmonary overcirculation leads to increase in the pulmonary
interstitial fluid and in severe forms may manifest as pulmonary
edema

 The RV pressure increases and this causes RV dilatation and

hypertrophy

 The increased pulmonary blood flow raises the pulmonary

capillary pressure and there is elevated, but subsystemic PVR,
which is variable

 Therefore, in a large VSD both pulmonary arterial and venous

pressures are elevated
 During the second year of life, the manifestations of congestive heart
failure (CHF) decreases as the pulmonary artery pressure
increases

 As PVR increases and exceeds SVR, right to left shunt occurs

 Initially it is mainly during exercise, due to the fall in SVR

 Later, the right to left shunt occurs at rest with persistent cyanosis

 There is marked fall in PBF with persistent hypoxemia

 RV failure finally supervenes

Eisenmenger VSD
 A large VSD, if left untreated, can result in irreversible
damage to the pulmonary arterial tree with
development of pulmonary vascular obstructive disease
(PVOD) and Eisenmenger’s syndrome

 The systolic pressure ratio is 1 and Qp/Qs is less than 1 :

1, with a net right to left shunt

 There is identical RV and LV systolic pressures and

suprasystemic PVR
 Small or Moderate or Large or Non Eisenmenger
restrictive Moderately restrictive VSD
restrictive
defects
Size Less than one- 1/3–2/3 of the >2/3
third of the size of size of the
the aortic root aortic root

Pulmonary/Ao <0.3 < 0.66 >0.66 1.0

rtic systolic
pressure ratio

Qp/Qs < 1.4:1 > 1.4–2.2:1 >2.2:1 1:1

LV volume Minimal Present Present
overload
Tendency to Nil Minimal Markedly
develop
increased PVR

Chances of Yes Yes Yes

Infective
Endocarditis
Natural History
Spontaneous Closure
 The incidence in perimembranous and muscular VSDs
is high

 Low in outlet defects and inlet defects do not close

 Swiss cheese muscular defects do not close

spontaneously

 Studies have documented that the spontaneous closure within

the first year is significantly higher for muscular than
for perimembranous defects
 In patients with restrictive VSDs followed up from birth,
there is a higher incidence of spontaneous closure (50-75
percent)

 The incidence of spontaneous closure in moderate and large

VSDs is only 5 to 10 percent
 Most defects which close do so in the first year of life
and approximately 60 percent close before 3 years and
90 percent by 8 years of age
 Therefore, blanket advice by the pediatrician that VSD will
close should be avoided, unless the size and site of VSD is
assessed properly
Small Peri-membranous VSD can close
by various methods:
 The adherence of the septal leaflet of TV to the IVS causing an aneurysm-like
pouch

 This can partially or completely close the defect, but this is at the cost of
causing tricuspid regurgitation (TR)
 The ingrowth of fibrous tissue with endocardial proliferation
causing septal aneurysm
 Prolapse of the aortic cusp especially the noncoronary or
the right coronary cusp, through the defect can close the VSD
at the cost of causing AR
 Growth and hypertrophy of the muscular portion of the
septum around the defect
 The vegetation caused by bacterial endocarditis on the RV
side of the VSD, but this is at the cost of infection
Right Ventricular Outflow Obstruction
 Gasul’s Effect

 3 to 7 percent of cases

 Large VSD can over a variable period develop hypertrophy of the crista
supraventricularis leading to significant infundibular obstruction

 This is seen particularly with perimembranous trabecular defects

 The left to right shunt may decrease with increasing stenosis and in severe
stenosis may become right to left

 Cyanosis is initially seen with exercise and is intermittent and later becomes
persistent
Aortic Regurgitation
 The incidence of aortic cuspal prolapse in outlet VSDs has
been shown to be as high as 73%

 They can progress to AR in 52–78% of the patients

 In perimembranous VSDs, aortic cuspal prolapse has been

shown to be 14% with progression to AR in 6%
 In early systole, blood is ejected from the LV and is also
shunted through the VSD

 The anatomically unsupported coronary cusp and aortic sinus

are driven into the RV due to the Venturi effect
 In diastole the intra-aortic pressure forces the aortic valve
leaflet to close, but the unsupported cusp (right or
noncoronary) is pushed down into the left ventricular
outflow tract away from the opposed coronary cusp,
resulting in AR
Infective Endocarditis
 Occurs in <1 to 3 percent of patients with VSD

 A small perimembranous VSD that does not close

spontaneously is generally associated with a good
prognosis, but is at risk for development of IE

 The vegetation is usually located on the septal tricuspid

leaflet at the site of impact of the jet
 In muscular VSDs the incidence of IE is low, as the jet is
dispersed in the RV cavity

 The site of the vegetation can occasionally be on the

aneurysm of the ventricular septum

 Rarely, an acquired left ventricular to right atrial shunt,

Gerbode defect, can occur due to the perforation of the
septal tricuspid leaflet secondary to endocarditis
Pulmonary Vascular Obstructive
Disease
 Pulmonary vascular obstructive disease may develop in 10
percent of the large VSDs

 In patients with pulmonary artery and RV systolic

pressure < 50 percent of the systemic arterial systolic
pressure there is moderate left to right shunt with possible
CHF

 The PVR does not increase after the initial postnatal fall, but
there is a small risk of increase, usually beyond 20 years of
age
 In patients with pulmonary artery systolic pressure
>50 percent of the systemic arterial systolic pressure, there
is significant risk for the development of pulmonary vascular
changes

 Measured PVR falls to high normal levels in infancy

and gradually rises in the ensuing years if the defect does not
become smaller

 The risk of development of permanent pulmonary vascular

disease is very rare before the first year of life
 Hence, prompt diagnosis and closure of these defects at
least prior to 18 months of age is likely to reduce the
incidence of development of pulmonary vascular disease

 If untreated these large or non-restrictive VSDs will have a

progressive rise in pulmonary artery pressure and a fall in
left to right shunting

 In turn, eventually this leads to higher PVR and to

Eisenmenger syndrome
Clinical features
 The clinical manifestations of isolated VSDs have a wide
spectrum

 Depends upon the size of the defect and the magnitude of the
shunt

 It may range from being asymptomatic to severe heart failure

 The signs and symptoms begin to develop, when the fetal PH

starts declining sufficiently to permit left to right shunting
Large VSD
 The infants with large VSDs present with symptoms due to
CHF by 4 to 6 weeks, as the PVR decreases

 The symptoms are

 Increased respiratory rate (tachypnea)
 Chest retractions
 Feeding difficulties with suck-rest-suck cycle
 Excessive sweating of forehead
 Repeated respiratory infections and failure to thrive
Moderate VSD
 Parents may observe pulsations over the precordium or feel
a thrill

 Child may have mild tachypnea, cough during feeding and

fatigue

 Sweating especially during feeding is frequent in infants below 6

months

 They may also present with lack of adequate growth and with one
or more episodes of pneumonia

 Older children may present with effort intolerance and fatigue

Small VSDs
 The children with small VSDs are asymptomatic

 Murmur on a routine health checkup.

 Older asymptomatic children may be detected during

routine school health check-up.
Physical Examination
 The infants with large shunts with CHF are malnourished
with poor growth and development

 These infants are tachypneic with chest retractions and there

is precordial bulge with bilateral Harrison sulcus
 If CHF is severe or if there is added pneumonia, there may be
retractions and grunting

 Infants with nonrestrictive VSDs with balanced shunts may

become cyanotic on crying or exercise

 Cyanosis and clubbing are seen in adolescent and adults with

large VSD with high PVR/Eisenmenger syndrome

 Peripheral edema is unusual in infants

Arterial Pulse
 Pulse is normal in small VSDs

 In moderate VSDs, the pulse is brisk due to the vigorous LV

ejection

 In nonrestrictive defects with large left to right shunts and

CHF there may be low volume pulse

 The pulse is normal in Eisenmenger syndrome, as the

systemic stroke volume is maintained
Precordial Movement and Palpation
 In moderate to large VSDs, precordial pulsations are visible
due to LV volume overload

 The apical impulse is LV type, hyperdynamic, displaced

downward and outwards

 In small and moderate VSDs a precordial thrill is best felt

in the third and fourth intercostal space (ICS) at the left
sternal border (LSB)
 In cases with subarterial VSD

 Thrill may be palpated in the second or first ICS and may

radiate upwards to the left into the suprasternal notch and into
left side of neck
 Large VSDs with high PVR

 Left parasternal lift may be present

 In patients with severe PH, there is a left parasternal heave

 Palpable P2 in the left second ICS

Auscultation
 The first heart sound is normal

 The second heart sound (S2) is normal with normal split

 Sometimes A2 may be obscured by the long murmur in small

VSDs

 Pulmonary component is normal or mildly increased in

moderate VSDs
 The murmur in small VSDs is grade 4/6, harsh long
systolic, crescendo-decrescendo best heard along the lower
LSB

 The murmur in moderately large defects are long, low

pitched decrescendo murmur best heard in LSB

 The systolic murmurs in outlet defects are heard in the

second ICS and may radiate upwards to the left into the
suprasternal notch and into the left side of neck
 When the shunt is large (Qp/Qs > 2:1), a short mid-
diastolic murmur is heard at the apex due to the increased
flow across the mitral valve

 The soft blowing early diastolic decrescendo murmur in the

left second and third ICS could be due to associated AR and
peripheral signs are present if the AR is significant
ECG in VSD
 ECG findings in ventricular septal defect (VSD) depend on
the size of defect, magnitude of the left to right shunt and
severity of pulmonary hypertension

 ECG is normal in small ventricular septal defects with small

left to right shunts
 Left atrial enlargement may be noted in moderately
restrictive VSDs and in those with large left to right shunts

 Left axis deviation is common with inlet VSDs and AV

septal defects. Left axis deviation may also be seen in about
5% of moderately restrictive VSDs

 Ventricular septal aneurysms and multiple VSDs (Swiss

cheese ventricular septum) can be associated with left axis
deviation
 The Katz-Wachtel phenomenon / sign is tall diphasic
RS complexes at least 50 mm in height in lead V2, V3 or V4 –
mid precordial leads

 The sign has been described in ventricular septal defect

with biventricular hypertrophy in children

 It can be seen with isolated ventricular septal defect as well as

complex ventricular septal defect
 In Eisenmenger complex, the ‘P’ waves are peaked with
right sided axis

 There is a tall monophasic ‘R’ preceded by small ‘q’ or

followed by small ‘s’ wave in V1
 In Gerbode defects there is both biatrial and biventricular
enlargement

 The tall peaked right atrial P wave in Lead II may be present from
infancy

 There is rSr in V1, and prominent left precordial q waves, tall R

waves, upright T waves indicating biventricular volume overload

 The hallmark in the ECG is the combination of right atrial P waves

with left ventricular hypertrophy
Chest Xray
 Chest X-ray is practically normal in small VSDs

 Moderate VSDs show cardiac enlargement of varying severity

and increased pulmonary vascular markings (PVM) or
plethora

Cardiomegaly with pulmonary

plethora in moderate sized ventricular
septal defect (VSD)
 The downward and leftward displacement of the cardiac
silhouette is due to LV enlargement
 In large VSDs, there is generalized cardiac enlargement with
increased PVM

 There is prominence of the MPA with RV enlargement

 LV apex is displaced posteriorly due to RVH

 In large VSDs with PH, the heart size is normal

 There is RV enlargement with the cardiac apex rotated slightly upward and to
left and posteriorly

 There is marked prominence of the MPA and its adjacent vessels with decreased
pulmonary vascularity in the outer third of the lung fields or peripheral pruning

C. shows peripheral pruning with no

vascularity seen in lateral 1/3 of the lung
fields (multiple arrows) in a case of large
VSD with severe PH, with dilated right
atrium and no cardiomegaly
 The radiological finding in Gerbode defect is the
disproprionate RA enlargement

 This huge RA enlargement on the right with RV

infundibulum and LV enlargement on the left side, gives a
ball shaped appearance to the cardiac silhouette
 ECHO in VSD

- 2020 Journal of the Indian Academy of Echocardiography & Cardiovascular Imaging | Published by
Wolters Kluwer
 A systematic echocardiographic assessment of VSD includes
a detailed:
 Anatomic and
 Hemodynamic description
Anatomic description
 Exact location of the defect

 Number of defects

 Relationship to valve and valve attachments,

 Description of anatomic size of the defect, and

 Associated lesions (if any)

 Detailed assessment of ventricular septum requires sweeping
the entire ventricular septum in both 2D and color Doppler
imaging from apex to base and from left to right

 Because of the curved nature of the ventricular septum,

optimal imaging of a VSD needs to be done from subcostal,
parasternal, apical, and right parasternal windows

 The best acoustic window is determined by the fact that

shows the septum perpendicular to the ultrasound beam and
the flow across the defect parallel to the beam
 Perimembranous Ventricular Septal
Defect
 Perimembranous defects are the most common type of VSDs
and involve the membranous ventricular septum adjacent to
aortic and tricuspid valves
 Located adjacent to tricuspid valve, perimembranous VSD
can be associated with tricuspid septal leaflet
distortion with tricuspid regurgitation

 Accessory tissue or part of septal leaflet of tricuspid valve can

partially or completely close the defect, referred to as
“ventricular septal aneurysm”
 Occasionally blood from VSD can traverse through
aneurysmal tissue across tricuspid valve into the right atrium
leading to LV to right atrial shunt

 Misinterpretation of this high-velocity flow as tricuspid

regurgitation can lead to false overestimation of RV pressure
 About 10% of perimembranous defects are associated with
AV prolapse due to close proximity with AV

 It can be identified by right or noncoronary cusp protruding

into the VSD best seen in parasternal long-.and short-axis
views

 Due to importance in the development of aortic

regurgitation, the presence of aortic cusp prolapse should
be carefully reported
INLET VSDs
 Inlet VSDs are located posteriorly adjacent to both
atrioventricular valves

 These defects are commonly associated with atrioventricular

septal defects (AVSDs) but can be isolated

 These are best imaged from apical four-chamber or

parasternal short-axis views
 AV valve involvement is common with inlet VSDs

 Tricuspid valve may be intrinsically abnormal with associated

tricuspid regurgitation

 VSD associated with partial AVSD may have cleft in the

anterior leaflet of mitral valve resulting in mitral
regurgitation
 Inlet VSD may
rarely be associated
with malalignment
of atrial and
ventricular septa,
resulting in some
degree of AV valve
override, and
rarely with
straddling of AV
valve chordal
attachments
Subarterial Ventricular Septal Defect
 Subarterial or infundibular VSDs result from deficiency in
conal or outlet septum beneath both semilunar valves

 These defects are best assessed from parasternal long-.and

short-axis views
 About 60% of subarterial defects are associated with
prolapse and distortion of the right coronary cusp of AV with
half of these patients developing aortic regurgitation

 Prolapse can be demonstrated as diastolic bulging of the right

coronary cusp into RV

 It can be mild to severe

 AV prolapse can lead to a reduction in size of VSD with

associated risk of aortic regurgitation
Muscular Ventricular Septal Defect
 Muscular VSDs are defects appearing in trabecular or
muscular septum entirely surrounded by muscular rim

 They can be further subdivided into anterior, mid-muscular,

posterior, or apical defects according to their location

 The presence of multiple muscular defects especially in the

mid-muscular or apical segment is referred to as
“swiss-cheese” septum
Assessment of the Size of the
Ventricular Septal Defect
 Determination of the size of VSD is made on hemodynamic
basis like

 Degree of left-to-right shunt

 Presence of volume overload, and

 Pulmonary artery pressure

 Historically, the size of VSD has been related to the size of
aortic root

 VSD measuring <1/3 of aortic root diameter is classified as

small

 1/3–2/3 of aortic root diameter as moderate, and

 Lesion close to aortic root size is considered large

 Hemodynamic classification uses pressure difference across
LV to RV as a guide to size the defects

 When there is equalization of pressures in two ventricles in

the presence of isolated VSD in the absence of pulmonary
stenosis, it is called as a large or nonrestrictive defect
 A restrictive defect has a pressure gradient across LV and RV
as determined by Doppler technique , with pressure gradient
of more than 60 mmHg as restrictive defect and pressure
difference of 25–60 mmHg as moderately restrictive
Hemodynamic description
 Chamber size

 Estimation of right ventricular pressure and pulmonary

artery pressure, and

 Estimation of overall shunt size

 Hemodynamic assessment of VSD using echocardiography
usually includes evaluation of right heart pressure and
quantification of amount of shunt flow

 Velocity of blood flow across VSD as measured by Doppler is

used to measure right ventricular systolic pressure using
modified Bernoulli equation

 Right ventricular pressure = Systolic blood pressure − VSD

jet peak gradient
 Proper alignment of Doppler beam with VSD jet is necessary for accurate
determination of right ventricular pressure that is equal to systolic PA pressure
in the absence of right ventricular outflow tract obstruction

 In addition, tricuspid regurgitation peak velocity can be obtained to estimate

RV pressure as follows:

 RV pressure = 4 × (TR jet velocity) 2 + RA pressure.

 While determining TR jet velocity, proper alignment of Doppler beam to TR jet

and complete TR signal must be obtained

 Any LV to RA shunt must be ruled out in the presence of perimembranous VSD

to avoid false interpretation
 VSD shunt volume is determined by size of VSD and PVR

 Left heart chamber dilatation is associated with

pulmonary-to-systemic flow ratio (Qp/Qs) of > 1.5:1

 Doppler-based methods for shunt quantification are not

widely accepted due to variable results
Transesophageal and 3D
Echocardiography
 Transesophageal echocardiography (TEE) is occasionally used

 In the pediatric age group, it is used most often

intraoperatively to assess the completeness of the repair

 Three-dimensional echocardiography has proved

accurate for quantifying shunt and can provide accurate
visualization of defects that otherwise are difficult to evaluate
by TTE
Cardiac Catheterization and
Cineangiography
 Indications are :

 If there is uncertainty regarding either defect number, size, location

and hemodynamic burden or additional lesions . The anatomy of
multiple apical VSDs is delineated on angiogram even those defects
which MRI and echocardiography sometimes cannot identify

 Interventional device closure of one or more defects

 To assess PVR and to study reactivity of the elevated PVR to different

pulmonary vasodilators (100% O and inhaled nitric oxide), especially in
2

older patients
Management
MEDICAL MANAGEMENT
 The children with small VSDs are asymptomatic and have
excellent long-term prognosis

 The parents need to be given reassurance, advise on subacute

bacterial endocarditis prophylaxis and periodic clinical
follow-up

 Medical therapy is required for patients with moderate to

large VSDs till any intervention is done to close the defect
 The drugs are usually a combination of diuretics (i.e.
furosemide), afterload-reducing agents angiotensin-
converting enzyme (ACE) inhibitors and digoxin
Approaches To Ventricular
Septal Defect Closure
 Surgical closure.

 Transcatheter techniques.

 Hybrid approach.
Surgery for Ventricular Septal Defect
 The indications for surgical closure of VSD in general are:

 Refractory heart failure and/or failure to thrive.

 Large defects that are unlikely to close, with or without symptoms

 Development of AR or aortic cusp prolapse especially in subpulmonic

or outlet VSDs

 Asymptomatic older children with QP/QS greater than 2:1

 The inlet and outlet VSDs which do not close spontaneously

Devices For Percutaneous
Closure Of Ventricular
Septal Defect
 The transcatheter device closure of muscular VSDs have
been in vogue for the past 15 years

 Although relatively common, perimembranous VSDs can be

difficult to close percutaneously

 Previous devices (e.g. Rashkind or button devices) have been

unsuccessful in attempts to close these VSDs, because of the
proximity of the defects to the aortic valve and the potential
for aortic valve damage
 Now many varieties of new devices like muscular septal
occluder for muscular VSDs, asymmetrical and symmetrical
perimembranous septal occluder and Amplatzer duct
occluder II (ADO II) are available to close perimembranous
VSDs and rarely Gerbode shunts
Midmuscular ventricular septal
occluder
B. Asymmetric perimembranous VSD occluder with only 0.5 mm retention disc
towards the aortic valve

C. Symmetric perimembranous VSD occluder

D and E. Amplatzer® membranous VSD occluder 2, has a dual
layer waist to minimize radial pressure against the rims of the defect, and the
waist length is increased to 3 mm to decrease the clamping effect on the
ventricular septum
Recommendations For
Device Closure
Of Muscular Vsds
Class IIa
Infants who weigh ≥ 5 kg
Children and adolescents with hemodynamically significant (left
ventricular or left atrial volume overload or pulmonary to
systemic blood flow ratio ≥ 2:1) muscular ventricular septal
defect (MVSD) to undergo percutaneous VSD device closure
Class IIb
Neonates, infants who weigh < 5 kg
Children with hemodynamically significant (left ventricular or left atrial
volume overload or pulmonary to systemic blood flow ratio
> 2 : 1) MVSD and associated cardiac defects requiring
cardiopulmonary bypass may be considered for performance of
hybrid perventricular closure of the VSD off bypass, followed
by surgical repair of the remaining defects or device placement
during cardiopulmonary bypass
Class III
Neonates, infants and children with hemodynamically significant
(left ventricular or left atrial volume overload or pulmonary to
systemic blood flow ratio >2 : 1) inlet MVSDs with inadequate
space between the defect and the atrioventricular or semilunar
valves should not undergo device closure (hybrid or percutaneous)

Neonates, infants and children with a small to moderate sized

MVSD (without symptoms or evidence of pulmonary
hypertension) in whom there is a reasonable expectation that the
defect will become smaller over time should be followed up
expectantly and do not need closure of the VSD
Exclusion criteria
 Weight less than 3.0 kg (unless the hybrid perventricular
approach is used)

 Distance of less than 4 mm between the VSD and the

aortic, pulmonic, mitral or tricuspid valves

 PVR greater than 7 indexed Wood units;

 Sepsis and patients with conditions that would be expected to

be exacerbated by the use of aspirin unless other antiplatelet
agents could be used for 6 months
Guidelines
- AHA Guidelines 2018
- ESC 2020