Classification of congenital heart disease:

— left vs right
Murmur is caused either by passage of normal amount of
— cyanotic vs a-cyanotic blood through abnormal valve
— Obstructive vs regurgitate Or passage of large amount of blood through normal
— Parallel vs Mixed valve

— Increased pulmonary blood flow vs decreased

➖ In chronic left to right shunt lesion the large volume of
— PDA dependent blood in the lung will cause vascular remodelling of the
pulmonary circulation and an increase in pulmonary
Diagnosis depend on: resistance.
1- clinical features: ➖ If the resistance of the pulmonary circulation increases
— palpation beyond that of the systemic circulation the shunt with reverse
direction as pressures on the right side of the heart increase
— auscultation
(Eisenmenger Syndrome).
2- CXR: ➖ The pt will develop clubbing & marked cyanosis, which
— Chamber enlargement may be more apparent in the feet and toes than in the upper
— Plethoric lung part of the body: differential cyanosis.
➖ Also cause polycythemia as compensation to hypoxia
The dilation effect of extra volume entering the ventricles depend in which ➖ This is more common with large ventricular septal defects
or persistent ductus arteriosus than with atrial septal defects.
phase of cardiac cycle it entered:
Blood entering the ventricle during diastole will remain longer time in the ➖ Patients with Eisenmenger’s syndrome are at particular risk
from abrupt changes in afterload that exacerbate right-to-left
chamber leading to chamber dilatation
shunting, such as vasodilatation, anaesthesia and pregnancy.
While blood entering the chamber during systole will be ejected rapidly
thus doesn’t cause dilation ➖ These conditions will decrease the afterload leading to
decrease LV pressure and exacerbating the shunt
(Examples later)

Chamber hypertrophy occur when there is outflow stenosis

In shunt lesion there is: Obstructive lesion (AS,PS,AC)

➖ volume overload ➖ Pressure overload ➡ Ventricular Hypertrophy
➖ chamber dilation ➖ No volume overload
➖ risk of pulmonary hypertension ➖ No pulmonary hypertension
⭕ Ventricular septal defect (VSD):
➖ The left ventricular pressure is higher than the right ventricle ➡ Left to right shunt (no cyanosis)
➡ there is large amount of blood entering the RV during the systole (when the ventricle contract) ➡
thus the blood will not remain in the RV and rapidly ejected to the lung thus doesn’t cause RV dilation
➖ There is large amount of blood entering the lung ➡ increase pulmonary blood flow ➡ causing
lung plethora on CXR ➡ the blood will return to the LA ➡ in order for the blood to enter the LV the
mitral valve should be opened ( the mitral valve open in diastole) ➡ the blood will enter the LV
during the diastole ➡ leading to LV and LA dilatation
DDx:
VSD Pansystolic murmur, harsh, diffuse, not radiate to axilla, thrill (vsd, mr, tr)
PDA continuous murmur
⭕ Atrial septal defect (ASD):
➖ The Left ➡ Right shunt that occur in ASD is not caused by high LA pressure (because atrial contraction is weak not
like the ventricle) but it is caused by the high compliance of the RV increasing its relaxation after systole and thus
sucking of blood from the left atrium to the right atrium.
➖ after entering the RA the blood needs the tricuspid valve to be opened in order to enter the RV (as the tricuspid
open in diastole) ➡ Thus the blood will enter the RV during diastole ➡ causing RV dilation
➖ Also the is increase in pulmonary blood flow (large amount of blood in the RV and there is no pulmonary stenosis)
➡ leading to plethoric lung on CXR
⭕ Pulmonary stenosis (PS):
➖ obstructive lesion cause increase pressure over the RV ➡ leading to hypertrophy of RV, the degree of hypertrophy
is proportional to the severity of stenosis, the RV hypertrophy appear the same of RV dilation on CXR (enlargement of
RV) to differentiate look at the lung ➡ in pulmonary stenosis the pulmonary blood flow is decreased or normal (if
compensated by hypertrophy) thus unlike ASD there is no lung plethora, in-fact it’s oligemic
🌀 on auscultation both ASD & PS have Ejection systolic murmur in the #pulmonary area, but:
In ASD:
➖ The murmur is not caused by flow of blood through the shunt like in the VSD because as we said the arterial
contraction is weak not sufficient to produce audible sound during shunting of blood from L to R but the sound is caused
by the passage of large volume blood through normal pulmonary valve.
➖ The murmur is soft, superficial & maybe radiated to back
➖ there is wide fixed spliting of S2 (Normally there is physiological splitting of S2 in inspiration as the venous return to
the heart increase ➡ increasing blood in RV ➡ large amount of blood should be ejected from RV through the
pulmonary valve ➡ the valve will remain open for longer time than the aortic valve ➡ the aortic valve will close
earlier than pulmonary valve (closure of both valves produce S2 sound) ➡ splitting of S2, but this splitting occur only in
inspiration, while in ASD there is continuous large volume of blood in RV (during inspiration & expiration) thus the delay
in pulmonary valve closure is fixed ➡ fixed & wide S2 splitting
In PS:
➖ The murmur is caused by turbulent flow of blood through the stenosed pulmonary valve
➖ The murmur is long, harsh, radiated to back & associated with thrill
➖ The pulmonary component of S2 is diminished (S2 decreased) because the movement of the valve is decreased
*ASD
Anatomy
atrial septal defect (ASD) is a communication or
opening between the atria. There are 4 anatomic
types:
Ostium primum low in atrial septum, may involve a
cleft mitral valve.
Ostium secundum center of the atrial septum.
Most common type
Sinus venosus high in the atrial septum. Associated
with P-TAPVR. Total Anomalous Pulmonary Venous Return
Coronary sinus large opening between the coronary
sinus and LA
Physiology
Because of higher pressure in the left atrium, blood
is usually shunted from the left atrium across the
ASD and into the right atrium. The magnitude of the
shunt depends on the size of the defect and

Clinical Signs & Symptoms

Isolated ASD’s rarely cause symptoms during infancy.
A small percentage of infants and children present
Dyspnoea, chest infections, cardiac failure and arrhythmias, especially atrial fibrillation, are other
with CHF. possible manifestations.

Hyperactive precordium, RV heave, fixed widely

With thrill

split S2, II-III/VI systolic ejection murmur at LSB and

Mid-diastolic murmur over LLSB.
Lower Left Sternal Border

Paradoxical embolism
Mode of diagnosis: Physical examination, ECG,
CXR, TTE
CXR: Enlarged heart and PA, increased lung
because right ventricular d larisation is delayed as a result
vascularity. of ventricular dilatation (with a ‘primum’ defect there is
also left axis deviation).

ECG: Rt axis deviation ,RBBB and RVH

The dilation appears at ECG
as hypertrophy?..

ECHO: Diagnostic
Spontaneous closure: Rare if defect >8 mm at Atrial septal defects in which pulmonary flow is

birth. Rare after age of 2 years. increased 50% above systemic flow (i.e. flow ratio of
1.5:1) are often large enough to be clinically
recognisable and should be closed surgically.

Method of closure: Transcatheter device

closure ,Surgical closure ➖ The long-term prognosis t after is excellent
unless pulmonary hypertension has developed

Ideal age of closure: ➖ Sever pulmonary hypertension and shunt

reversal are both contraindications to surgery.

i. In asymptomatic child: 2-4 years

ii. Symptomatic ASD in infancy
iii. If presenting beyond ideal age: Elective closure
 Ventricular septal defects are the most common congenital
Anatomy cardiac defect, occurring once in 500 live births.

Ventricular septal defect (VSD) is a communication

between the right and left ventricles. Accounts for
25% of CHD, There are 4 anatomic types:
Perimembranous Upper portion of septum (most
common,80%).
Subpulmonary Below pulmonary valve.
Muscular In the muscular portion of ventricular
septum. Multiple defects may be referred to as ‘swiss
cheese’ defects.
Atrioventricular canal located beneath the tricuspid
valve. Also called an inlet VSD.
Physiology
Because of higher pressure in the left ventricle, blood
is usually shunted from the left ventricle, across the
VSD into the right ventricle, and into the pulmonary
circulation. The magnitude of the shunt depends on
the size of the defect and the amount of pulmonary
vascular resistance.
Clinical features:
Symptoms:
Small VSD: Patient usually asymptomatic maladie de Roger -> mean small
asymptomatic defect

Moderate and large VSD: Symptoms may be started

from birth. Dyspnea, Growth failure,recurrent
LRTI,Congestive heart failure, Cyanosis
Signs:
Small VSD: Harsh pansystolic murmur and systolic
thrill at 3rd and 4th intercostal spaces on LSB At erbs area
Moderate and Large VSD: Same as small VSD
with load P2 and middiastolic murmur at the apical
area.
Mode of diagnosis: Physical examination, ECG,
CXR, TTE Trans-thoracic echo
CXR: Cardiomegaly, Enlarged LA and LV, Increase
vascularity
ECG: LAD, LAE and left or Biventricular hypertrophy.
ECHO: Diagnostic
Spontaneous closure:
Spontaneous closure is uncommon in large VSDs.
30%-40% of moderate or small defects close
spontaneously by 3-5 years of age.
Indications for Closure:
Large VSD with medically uncontrolled symptoms
and FTT. Failure To Thrive
Ages 6-12 mo with large VSD and Pulm. HTN
Age > 24 mo with Qp:Qs ratio > 2:1.
Subpulmonic VSD of any size, secondary to risk of
developing AV insufficiency.
- Cardiac failure in infancy is initially treated medically with digoxin and diuretics Persisting failure is an
indication for surgical repair of the defect.

- Eisenmenger’s syndrome is avoided by monitoring for signs of rising pulmonary resistance (serial ECG and
echocardiography) and carrying out surgical repair, when appropriate. Surgical closure is contraindicated in
fully developed Eisenmenger’s syndrome when heart–lung transplantation may be the only effective
treatment.