Clinical Pearls in Atrial septal defect

Downs , Edwards , Pataus

Holt Oram syndrome hypoplastic thumb accessory phalanx,

TAR syndrome

Ellis van crevald syndrome

Eisenmenger – cyanosis and clubbing

Axenfeld rieger anomaly (AD- ocular anomalies)

20-30 %  more than 40 years have AF

Normally a=v wave

a>v wave small shunts , LV failure , PAH , LV dysfunction

v>a wave large left to right shunts

X descent well preserved

Prominent left IJV > right s/o PAPVC into right atrium by LSVC/coronary sinus

Normal apex in ASD is RV s/o increased amplitude and normal duration hyperdynamic and retraction during late systole

Large shunts s/o sustained RV lift/heave even in absence of PAH(very large RV chamber displaying and occupying apex

Palpable S3/S4

LV impulse   minimal/absent

RVOT/PA systolic thrill due to palpable PA

RVOT pulsations

Increased intensity of T1 , audible splitting S1 and delayed closure of T1 due to prolonged RV ejection

Second heart sound wide and fixed splitting(except in infants and young children where its narrow as little or no shunt in either direction), Loud P2(dilated PA and brisk elastic recoil)

Wideness due to prolonged RV ejection and increased hang out interval

Fixed as RV filling remains constant in both phases of respiration

Normal splitting in sinus venosus ASD

Other d/d of WFS are RV dysfunction , Acute PTE and RBBB

RVS3/RVS4 and Opening snap suggest Qp/Qs > 2:1

Pulmonary ejection sound in half of pts with ASD(soft and not as high frequency as PS)

Pulmonary ejecrion murmur creasendo decrescendo after S1 and ends well before S2

If ASD murmur is grade 4-6- suspect associated pulmonary stenosis

Other findings of associated PS in ASD– Longer and louder murmur , systolic thrill, loud click, P2 normal/prominent

Diastolic murmur(TV flow murmur s/o Qp/Qs > 2:1 and starts in early diastole , increase on deep inspiration and leg elevation, hemodynamic significant left to right shunt

Diastolic murmur across ASD - continuous murmur beginning in late systole and extends into diastole s/o lutembachers with restrictive ASD(associated MS)

ASD with PAH and PR may have early diastolic murmur(grahams steel)

Diatolic murmur of low pressure PR uncommon(aneurysmal dilatation of pulmonary trunk)

Continuous murmurs through restrictive ASDs are rare

Sinus node dysfunction as early as 2-3 years

Accelerated atrial rhythm in 1/3 children with ASD

AF , Atrial flutter and SVT by 4^th decade

Sinus arrhythmias does not occur

Atrioventricular conduction defects(prolonged PR)

Advanced first degree familial > non familial ASD

Holt Oram first degree HB , sinus Brady and ectopic atria; rhythm

Peaked right atrial P and prolonged P wave duration enlarged right atrium

 Normal p wave axis in OS ASD compared to SV ASD where it is inverted in inferior leads and upright in AvL( ectopic atrial rhythm);QRS axis vertical and clockwise depolarization q in inferior leads

RBBB; rad(symptomatic PAH or pulmonary vascular ds);LAD exceptional represents LAHB in older pts

Crochetage sign(OS and SV ASD) sometimes shunt severity; also seen in PFA

Increased vascularity to peripheral lung fields

Pulmonary trunk and proximal branches dilated

Asc aorta is seldom border forming

Jug handle appearance

SV ASD accompanied by localized ampullary dilatation of SVC proximal to attachment of RA

Aneursymal dilatation of MPA in extreme PAH

RA enlargement; LA enlargement in pts with AF and older pts

RV enlargement