Congenital Heart Disease

characteristics VSD

blood shunts left→right thru vsd, dilation left heart, shunts into pul vasc

Eisenmenger syndrome

inbig non-restrictive defects of VSD, r ventricular p becomes suprasystemic (higher than left), blood shunts from right to left → cyanosis

clinical features restrictive vs big (non restrictive) VSDs

restrictive (maladie roger) - asymptomatic, loud pansystolic murmur

big - hf in childhood → pul htn, eisenmenger, cyanosis, clubbing

investigations VSD

echo

treatment vsd

surgical repair, device closure if shunt is causing left heart enlargement + NO pul htn

atrial septal defect types

secundum defects - fossa ovalis

sinus venosus defects - sup part septum near sup VC or inf septum near inf VC

ostium primum defects - lower atrial septum + av valves

effects ASD

left right shunt, right v overload + right heart dilation → arrhythmia

raised pul p

clinical features asd

split s2, murmur across pul valve

xray in asd

prominent pul arteries and cardiomegaly (r heart dilation)

ecg asd

right bbb, r axis deviation → atrial arrhythmia

what confirms the dg in asd

echo

when is closure of asd indicated

significant left→right shunt that resulted in right atrial and ventricular enlargement

patent ductus arteriosus

communication bw proximal l pul a and descending aorta = continuous left-right shunt

what is given in patent ductus arteriosus

indometacin

clinical features grades patent ductus arteriosus

silent - tiny pda

small - audible systolci or continuous murmur

moderate - bounding pulses + continuous murmur radiating to back, displaced apex beat

large - pul htn, eisenmenger → clubbing

chest xray in patent ductus arteriosus

enlarged pul a and increased cardiothoracic ratio

patent ductus arteriosus in ecg

left atrial abnormality, high voltage qrs complexes

indications for intervention in patent ductus arteriosus

left ventricular dilation w/ shunt and no pul disease

how to treat small defects in patent ductus arteriosus

device closure (risk endarteritis)

aorta coarctation

narrow aorta at it or distal to insert ductus arteriosus → intercostal arteries collateral circ

clinical features aorta coarctation

htn, headaches, nosebleeds, claudication

htn upper limb, weak pulses in legs

chest xray in aorta coarctation

dilated aorta at site coarctation, 3 sign, rib notching

ecg patent ductus arteriosus

left ventricular hypertrophy

when is intervention indicated in coractation

peak-peak gradient across coarctation >20mmHg cardiac catheter lab OR >50% narrowing + htn/lvh

treatment coarctation

neonates - surgical repair

older kids - balloon dilation, stent

ToF

• vsd

• overriding aorta

• r ventricular outflow tract obstruction

• r ventricular hypertrophy

symptoms ToF depend on

degree pul stenosis

fallots spells

episodes severe cyanosis in kids → squatting

treatment ToF

complete repair, pul valve replacement later

transposition great arteries

right atrium connects to right ventricles, which gives rise to aorta

left atrium connects to left ventricle, which gives rise to pul artery = 2 parallel blood flow circuits

babies are cyanosed and rely on what in transposition great arteries

asd, vsd or pda (for a mixed shunt)

treatment transposition great arteries without an adequate shunt

atrial septostomy - rashkind’s balloon

Marfan syndrome

connective tissue disorder AD by mutation FBN1 gene

dg marfan syndrome

modified ghent criteria - aortic root aneurysm, lens dislocation or family history

primary cause death marfan

cardiovasc

monitoring marfan

serial cardiovasc imaging, aortic dimensions

preconceptual counselling

medical therapy marfan

b blockers, arbs