Acyanotic Heart Disease

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OBSTRUCTIVE

LESIONS
Shishir Shrestha
It is one of the most common congenital defects.
Types: based on the site of obstruction
- valvular (at the valve level)
- supravalvular (above the valve)
- subvalvular (below the valve)
Valvar obstruction is overcome by raising the systolic
pressure of the left ventricle .
The emptying of the left ventricle is complete but the
duration of the systole is prolonged that causes delayed
closure of the aortic valve resulting in delayed A2.
The flow across the obstruction results in the aortic
ejection systolic murmur(diamond shaped, starting after S1
and ending before A2 with mid systolic peak.
Concentric hypertrophy of the left ventricle results in
decreased distensibility of the left ventricle in diastole.
With severe AS accompanied with marked left
ventricular hyper trophy, a forceful left atrial
contraction( due to increased resistance) results in
palpable as well as audible forth heart sound(S4).
When left ventricle starts failing in AS , besides
hypertrophy, dilatation also occurs and causes increase
in heart size.
With left ventricular failure, a third heart sound(S3)
becomes audible.
Symptoms :
- Mild to Moderate stenosis:
- usually asymptomatic
- Severe stenosis:
- Exertional dyspnoea
- Angina on effort
- Syncope
(Presence of any of the above symptoms suggest
severe AS)
- Narrow pulse pressure.( Width of pulse pressure is
inversely related to the intensity of AS)
- S1 normal.
- A2 component of S2 delayed but not diminished in
intensity. Delay results in normally split(mild), closely
split(moderate), single or paradoxically split(severe AS) S2.
- In severe AS, S4 audible and palpable.
- In left ventricular failure S3 is present.
- Ejection systolic murmur starting after ejection click
reaches a peak in mid systole, peak shifts towards the end
with increasing severity.
Chest x-ray :may be normal ; sometimes enlarged LV
and dilated ascending aorta.
ECG : left ventricular hypertrophy( large QRS
complex), ST segment and T wave changes occurs in
severe forms.
ECHO: concentric left ventricular hypertrophy , Site
of stenosis can be identified.
Doppler : Can quantitate the gradient againt
obstruction fairly nicely.
(6-12 monthly ECG must be taken, symptoms must be carefully
evaluated,Doppler ECHO used to quantitate gradient across the
obstruction)
Patients should be discouraged from outdoor
games,athletics,competitive sports and sternous exercise.
Balloon aortic valvuloplasty is the procedure of choice for valvar
AS.(indicated if gradient aove 75 mm Hg)
Supravalvar and subvalvar AS does not respond to balloon
dilation .
Procedure avoided in patient with significant aortic
regurgitation.
Surgical options include valve repair and replacement with a
prosthetic valve .patients need to be administered anticoagulants
if they have a prosthetic valve replacement .
Coarctation of the aorta is located at the junction of
the arch with descending aorta.
It may be distal or proximal to the ductus or
ligamentum arteriosus and also the left subclavian
artery . 40 to 80 percent have bicuspid aortic valve.
In fetal life , the right ventricular output passes down
the descending aorta through a wide ductus arteriosus
.
The left ventricular output empties into the
innominate , left carotid and left subclavian arteries
and little output reaches the descending aorta .
Following closure of the ductus arteriosus ,the
descending aorta must receive its total supply from the
left ventricle via the ascending aorta.
Neonates with severe coarctation therefore becomes
symptomatic immediately as the duct starts to close .
Infants with coarctation present with LV dysfunction and
heart failure.
In uncomplicated coarctation symptoms can be
intermittent claudication, pain, weakness of legs and
dyspnea on running
Systolic arterial pressure is higher in the arms than in the
legs , but the diastolic pressures are usually similar.
Systolic thrill may be palpable in the suprasternal notch
and left ventricular enlargement may be present.
S1 loud, S2 normal splitting accentuated A2, S3 with
LV failure, S4 with severe hypertension.
Continuous murmur may be heard over the interscapular
or infrascapular areas, indicating blood flow through
collateral channels .
Normal sized heart with prominent ascending aorta
and aortic knuckle.
Reversed E sign (d/t pre- and post- sternotic aortic
dilatation and dilatation of the subclavian artery)

ECG: shows left ventricular hypertrophy


Medical treatment consists of control of hypertension .
Surgial tratment is more preferred in newborns and
infants.
Surgical treatment consists of resection of coarctation
and reanastomosis or by aortoplasty. Prostaglandin E1
is used to maintain ductal patency prior tosurgery in
first few weeks of life.
In older children, adolescents and adults balloon
dilatation is often undertaken( palliative treatment)
It is usually valvar or subvalvar .
Uncommonly pulmonic stenosis may be in the
pulmonary artery above the valve or in the main right
or left branches or the peripheral branches.
Flow across the narrow pulmonary valve results in a
pulmonary ejection systolic murmur. To keep the flow
normal the right ventricle increases its systolic
pressure & develops concentric right ventricular
hypertrophy .
Obstruction causes prolongation of the right
ventricular systole reslting in delayed closure of the
pulmonic component (P2) of second sound.
Patients with mild to moderate pulmonic stenosis are
asymptomatic ;with severe stenosis,dyspnea on efforts
appears.
If foramen ovale is patent , a right to left shunt at the
atrial level may occur in severe PS & results in cyanosis
.but cyanosis is absent in mild and moderate PS.
A raised JVP with prominent a wave (in severe PS)
S1 normal,S2 - is widely split, P2 soft and delayed,S3-
with RV failure, S4 with severe stenosis.
Loud ejection systolic murmur heard over pulmonary
area.
Balloon pulmonary valvuloplasty is the treatment of
choice for isolated valvar PS.
Surgical treatment is indicated only if balloon
valvotomy is unsuccesful,as in patients with dysplastic
valves or small pulmonary valve annulus.
Infundiular PS requires surgical resection.

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