Presentation Precordial findings ECG findings CXR findings Prognosis Management of Coarctation of aorta Pulmonary valve stenosis Aortic Stenosis

1. Acyanotic Congenital Heart Diseases
Coarctation of aorta
Aorta narrowed at isthmus
between L. subclavian artery
& arterial duct
Rarely in lower thoracic /
abdominal aorta
Present in > 15% of girls with
Turner’s syndrome
Commonly associated with
bicuspid aortic valve, VSD,
mitral valve anomalies.
Neonates
o Circulatory collapse
(with closure of duct)
Infants
o Heart failure
Older children / adults
o Asymptomatic
murmur
o weak femoral pulses
(In routine
examination)
o Systemic hypertension
o With Turner’s
syndrome
Present
ation
Precord
ial
finding
s
•
•
Absent / low volume femoral
pulse
Blood pressure
– R. arm > 10 mmHg
Pulmonary valve stenosis
Aortic Stenosis
May occur at valvuar, subvalvular or
supravalvular level
Leads to post-stenotic dilatation of pul
artery
Occurs in congenital rubella syndrome
Often occurs in bicuspid aortic valves
Usually associated with mitral stenosis
and coarctation of aorta
Commonly present in William’s syndrome
Family history (AD)
25 % sporadic
Mild stenosis
- Asymptomatic murmur
Mild – moderate
Asymptomatic murmur
Severe stenosis
-Cyanosis in neonates / infants
-R. heart failure in older children
Severe
Short ejection systolic murmur
– Maximal at 2nd L. intercostal space
Pulmonary ejection click
Pulse – low volume & slow rising
BP – reduced pulse pressure in severe stenosis
Systolic thrill at suprasternal notch
Second heart sound
Neonates – heart failure, circulatory
collapse
Older children – reduced exercise
tolerance, angina on exertion, syncope
(Sudden death in < 2 %)

2. ECG
CXR
higher than in legs
– BP in left arm depends
on site of coarctation
• Prominent left ventricular
impulse
• Short ejection systolic murmur
– Maximal at 2nd L.
intercostal space &
below left scapula
posteriorly
• Continuous murmur heard in
older children due to
development of collateral
arteries
L. ventricular hypertrophy and strain
pattern in older children
“3” sign- due to abnormal
aortic contour
Rib notching- due to collateral
arteries
Ba swallow may show
narrowing of the oesophagus “E” sign
Echocardiography & Doppler studies
In severe stenosis
• Jugular venous pulse
– Prominent “a” wave
• parasternal heave
• Thrill at 2nd L. intercostal space
• Soft, delayed P2
Soft aortic component
Single P2 or paradoxical splitting
Ejection systolic murmur
Radiates to neck & down the L.sternal border
Apical ejection click
R. ventricular hypertrophy
p pulmonale
L. ventricular hypertrophy +/- strain pattern
Post-stenotic dilatation of pul. Artery
R. ventricular hypertrophy
Echocardiography
Post stenotic dilatation of ascending aorta
L. ventricular hypertrophy may be present

3. (Demonstrates the site of coarctation )
MRI
Cardiac catheterization & angiography
Progno
sis
Manag
ement
Surgical correction
1. Excision of narrowed segment
with end-to-end anastomosis
of aorta (Rarely a patch / tube
graft is necessary)
2. L. subclavian flap procedure
Surgical mortality < 2 %
< 4 % incidence of restenosis
Risk of recurrent laryngeal / phrenic
nerve damage, spinal cord ischaemia
Balloon dialatation
(Is used to prevent / treat restenosis )
Thickened pulmonary valve
Pulmonary artery dilatation
R. ventricular hypertrophy
Tricuspid regurgitation
R. atrial enlargement
• Often decreases spontaneously
•
•
In 27 % progressive obstruction
Pressure gradient > 50 mmHg
→Surgery
– Balloon dilatation is the treatment
of choice
– Long term results excellent
– Risk of pul. regurgitation,
restenosis & rarely IE
•
•
Progresses with time
Regular clinical & echocardiographic
assessment of disease progress is
necessary
• Mild stenosis – no restriction of activities
• Surgery
If clinically severe stenosis or pressure gradient
across valve is > 50 mmHg
– Transcatheter balloon dilatation
– surgical valvotomy
– Valve replacement (if
unrelievable obstruction or AR)