RSR#$2014$

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CONGENITAL!HEART!DISEASE!(COMMON!CONGENITAL!HEART!LESIONS)! !
CHD$are$generally$well$tolerated$before$birth,$due$to$the$present$of$ductus$arteriosus$and$foramen$ovale.$CHD$can$be$categorized$as:$
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Acyanotic!Lesions!
No.$ Name$ Definition$ Pathophysiology$ Symptoms$ Physical$ Diagnostic$Studies$ Treatment$
Examination$
1.$ Atrial$Septal$ Persistent$ L$to$R$shunt$!O2$blood$ Mostly$asymptomatic$ P:$prominent$in$ #Chest$X#ray:$ Surgery$(to$
Defect$(ASD)$ opening$in$the$ from$LA$!$RA$(not$vice$ $ lower#left$sternal$ cardiomegaly$(RA$ prevent$heart$
interatrial$ versa);$depends$on$size$ If$symptoms$do$occur:$ border$ and$RV)$and$↑$ failure):$direct$
septum$after$ and$ventricular$ dyspnea$on$exertion,$ S2:$widened#$fixed$ pulmonary$vascular$ suture$closure$
birth$that$ compliance$!$volume$ fatigue,$and$recurrent$ splitting$pattern$ markings.$ or$use$cath.$
allows$ overload$and$ lower$respiratory$tract$ No$murmur:$no$ #ECG$
communication$ enlargement$of$RA$and$ infection,$decreased$ significant$ #Echocardiography$
between$LA$and$ RV$$ stamina,$and$ pressure$gradient$ #Cath.:$unnecessary$$
RA$ *can$lead$to$ES$ palpitation$ between$the$two$
$ atria$
2.$ Ventricular$Septal$ Abnormal$ L$to$R$shunt$!$ Mostly$asymptomatic$ Murmur:$Harsh$ #CXR:$cardiomegaly$ <2$y.$o.$small$
Defect$(VSD)$ opening$in$the$ hemodynamic$changes;$ $ holosystolic$ and$prominent$ or$moderate$
interventricular$ depends$on$size$and$ 10%$infant:$congestive$ murmur$at$the$left$ pulmonary$vascular$ openings$
septum.$Most$ pulmonary$and$ heart$failure,$ sternal$border$ markings$(in$case$of$ undergo$
often$located$in$ systemic$resistance$!$ tachypnea,$poor$ (smaller=$louder)$ pulmonary$vasc.$ spontaneous$
the$ RV,$LV,$LA,$and$ feeding,$failure$to$ $ Disease$!$enlarged$ closure$
membranous$ pulmonary$circulation$ thrive,$and$frequent$ Mid$diastolic$ pulmonary$arteries$ $
(70%)$and$ overload$!$↑LV$stroke$ LRTI,$(bacterial$ rumble$near$the$ with$peripheral$ surgery$for$
muscular$(20%)$ volume$!$chamber$ endocarditis)$ apex$ tapering)$ patient$with$
$ portions$of$ dilatation,$systolic$ #ECG:$LA,$LV,$RV$ HF$and$PD$
septum$ dysfunction,$and$ hypertrophy$ $
symptoms$of$heart$ #Echocardiography$
failure$
 *can$lead$to$ES$ #$Catheterization:$L$ Therapeutic$
to$R$shunt$ catheterizatio
ns$$
3.$ Patent$Ductus$ Vessel$that$ At$birth,$Pulmonary$ Mostly$asymptomatic$ Murmur:$ #CXR:$LA$and$LV$ Even$a$small$
Arteriosus$(PDA) connects$LPA$ vascular$resistance$ $ continuous,$ enlargement$with$ asymptomatic$
and$desc.$Aorta$ (PVR)$↓!$blood$from$ Severe:$congestive$ machine$like;$ prominent$ PDA$is$
(Ductus$ aorta$drifted$to$the$left$
heart$failure$(CHF)$ heard$best$at$left$ pulmonary$vascular$ commonly$
arteriosus)$fails$ pulmonary$artery$!$ with$tachycardia,$poor$ subclavicular$ markings$(in$adults,$ referred$for$
to$close$after$ volume$overload$in$LA$ feeding,$slow$growth,$ region$ calcification$of$ closure.$
birth.$ and$LV$!$chamber$ and$recurrent$LRTI.$ (because$pressure$ ductus$may$be$ $
$ dilatation$!$left#sided$ $ gradient$between$ visualized)$ Neonates$and$
RF:$1st$$ heart$failure$ Moderate:$fatigue,$ aorta$and$PA$ #ECG:$LA$and$LV$ premature$
trisemester$ $ dyspnea,$palpitations$ present$during$ hypertrophy$ infants:$
rubella$ *can$lead$to$ES$ $ both$systole$and$ #Echocardiography$ prostaglandin$
$ infection,$ Turbulent$blood$flow$ diastole)$ #diagnostic$ synthesis$
prematurity,$ across$ductus!$ Catheterization$not$ inhibitor$
birth$at$high$ endocarditis/$ necessary$$ (indomethaci
altitude$ endarteritis) n)$or$surgery$$
4.$ Congenital$Aortic$ Most$often$ Narrowed$valvular$ <10%$infants:$ Murmur:$harsh$ #CXR:$enlarged$LV$ Transcatheter$
Stenosis$(CAS) caused$by$ orifice$!$LV$pressure$↑$ experience$symptoms$ crescendo#$ and$dilated$asc.$ balloon$
abnormal$ to$pump$blood$across$ of$heart$failure$before$ decrescendo$ Aorta$ valvuloplasty$
structural$ the$aortic$valve$!$LV$ age$1$(tachycardia,$ systolic,$loudest$at$ #ECG:$LV$
development$of$ hypertrophies$ tachypnea,$failure$to$ the$base$of$the$ hypertrophy$
the$valve$ $ thrive,$and$poor$ heart$with$ #Echocardiography:$
leaflets$ High#velocity$jet$blood$ feeding)$ radiation$to$neck$ abn.$Structure$of$
(eccentric$ passes$through$ $ the$aortic$valve$
$ stenotic$ stenotic$valve$!$ Most$older$children:$ #Doppler$
opening$of$2$ proximal$aorta$ asymptomatic$or$ assessment:$
aortic$valve;$ dilatation$ fatigue,$exertional$ pressure$gradient$at$
normally$3)$!$ dyspnea,$angina$ aortic$valve$
pectoris,$syncope$
 worsen$after$ #Cardiac$
time$ catheterization$
5.$ Pulmonic$Stenosis$ Obstruction$to$ Stenosis$!Impaired$RV$ Asymptomatic$ Prominent$jugular$ #CXR:$enlarged$RV$ Mild:$doesn’t$
(PS)$ the$right$ outflow$!↑$RV$ $ venous$a$wave$(RV$ and$RA$with$post# required$
ventricular$ pressure$and$chamber$ Severe:$dyspnea$with$ hypertrophy)$ stenotic$pulmonary$ treatment$
outflow$at$the$ hypertrophy$!$long# exertion,$exercise$ $ artery$dilatation$ $
level$of$the$ term$severe$can$lead$to$ intolerance,$and$with$ RV$heave$ #ECG:$RV$ Transcatheter$
pulmonic$valve$ right#sided$heart$ decompensation$ $ hypertrophy$(+$right$ balloon$
within$the$body$ failure$ $ Murmur:$Loud,$ axis$deviation)$ valvuloplasty$
of$RV$or$PA.$ RS#$Heart$failure:$ late#peaking,$ #Echocardiography$
abdominal$fullness$and$ crescendo# with$Doppler$
$ pedal$edema$ decrescendo$ imaging;$assess$PV$
systolic$ejection$ morphology,$RV$
(upper$left$sternal$ hypertrophy,$and$
border)$ measure$pressure$
+$palpable$thrill$ gradient$across$
obstruction$
6.$ Coarctation$of$the$ Discrete$ Aortic$narrowing$!$LV$ Symptoms$of$heart$ Femoral$pulses$are$ #CXR:$aortic$site$of$ Neonates$
Aorta$ narrowing$of$ experience$increase$ failure$shortly$after$ weak$and$delayed$ coarctation$ with$severe$
the$aortic$ afterload!$blood$flow$ birth$ $ #ECG:$LV$ obstruction:$
lumen$(with$ to$systemic$area$ $ Elevated$BP$in$the$ hypertrophy$ (before$
most$common$ disturbed$!$if$not$ Differential$cyanosis$ upper$body$$ #Doppler$ surgery)$
cardiac$abn.$:$ corrected:$$ when$present$also$ $ echocardiography:$ prostaglandin$
bicuspid$aortic$ 1)$LV$hypertrophy$ with$PDA$ Actually$depends$ assess$pressure$ infusion$
$ valve)$ 2)$Dilatation$of$ $ on$the$site$of$ gradient$ $
$ collateral$BV$from$the$ Different$pressure$at$ coarctation$ #MRI:$detail$length$ Surgery$or$
Often$occur$in$ intercostal$arteries$that$ upper$and$lower$ and$severity$of$ trans$catheter$
patient$with$ bypass$coarctation$and$ extremities$ coarctation$ intervention$
Turner$ provide$blood$to$the$
Syndrome$(45,$ more$distal$des.$aorta$
XO)$
$
!
Cyanotic!Lesions!
No.$ Name$ Definition$ Pathophysiology$ Symptoms$ Physical$ Diagnostic$Studies$ Treatment$
Examination$
1.$ Tetralogy$of$Fallot Results$from$a$ As$a$consequence:$ Dyspnea$on$exertion$ Mild$cyanosis$ #CXR:$“boot# Surgical$
single$ 1)$VSD$caused$by$ $ most$notably$on$ shaped”$heart$ intervention$
developmental$ anterior$malalignment$ “spells”$while$feeding,$ the$lips,$mucous$ because$of$ (6$to$12$
defect:$an$ of$the$interventricular$ crying,$following$ membrane,$and$ prominence$of$RV$ months$of$
abnormal$ septum$ exertion$(systemic$ digits$ and$↓size$of$main$ age)$
anterior$and$ 2)$subvalvular$ vasodilatation$!$↑$R$ $ pulmonary$segment$
chepalad$ pulmonic$stenosis$ to$L$shunt)$ Chronic$ #ECG:$RV$
displacement$of$ because$of$obstruction$ #$cyanosis$ hypoxemia!$ hypertrophy$with$
$ the$infundibular$ from$the$displaced$inf.$ #$hyperventilation$ clubbing$fingers$ right$axis$deviation$
(outflow)$ septum$ #$occasionally,$syncope$ and$toes$ #Echocardiography:$
portion$of$the$ 3)$Overriding$aorta$ or$convulsions$ $ RV$outflow$
interventricular$ that$receives$blood$ $ Palpable$heaves$ anatomy,$
septum$ from$both$ventricles$ Squatting:$press$ along$the$left$ malaligned$VSD,$RV$
$ 4)$RV$hypertrophy$ femoral$arteries$!↑$ sternal$border$ hypertrophy,$etc.$
Microdeletion$ owing$to$the$large$ systemic$vascular$ $ #Cardiac$
in$chromosome$ pressure$placed$on$the$ resistance!$↓R$to$L$ Murmur:$Systolic$ catheterization$$
22$(22q11)$ RV$because$of$the$ shunt!$more$blood$ ejection$(best$
pulmonary$stenosis$ from$RV$to$lungs$$ heard$at$upper$left$
$ $ sternal$$border)$
retruning$blood$from$
VCS/VCI$!$because$of$
pulmonic$stenosis$!$R$
to$L$shunt$(VSD)!$
cyanosis$
 2.$ Transposition$of$ Each$great$ Desaturated$blood$ Baby$appears$blue$ #CXR:$Narrowing$ (emergency)$
the$Great$Arteries vessel$ from$the$systemic$ $ base$of$heart$owing$ $
inappropriately$ circulation$are$forced$ P:$RV$impulse$at$lower$sternal$border$as$the$ to$mor$antero# Prostaglandin$
arises$from$the$ back$to$the$systemic$ RV$faces$systemic$impulse$ posterior$ infusion$to$
opposite$ circulation$through$the$ $ orientation$of$the$ maintain$
ventricle;$aorta$ RV$to$the$aorta$ S2:$accentuated$!$reflects$closure$of$the$ aorta$and$ ductus$
from$RV$and$ without$undergoing$ anteriorly$placed$aortic$valve$just$under$the$ pulmonary$artery$ arteriosus$
Pulmonary$ the$normal$ chest$wall$ #ECG:$RV$ $
artery$from$LV$ oxygenation$in$the$ $ hypertrophy$ Interatrial$
$
*most$common$ lungs;$oxygenated# Prominent$murmur=$additional$effects$ #Echocardiography:$ communicati
cause$of$ pulmonary$venous$ definitive$diagnosis$ on$using$
cyanosis$in$the$ return$passes$through$ of$TGA$ balloon$
neonatal$period$ the$LV$to$the$PA$ catheter$
without$imparting$the$ (Rashkind$
oxygen$to$the$systemic$ procedure)$
circulation$!$LETHAL$ $
(better$with$PFO$and$ (later$on)$
PDA)$ Surgical:$
Jatene$
procedure$!$
arterial$switch$
!
*Eisenmenger!syndrome:!condition$of$severe$pulmonary$vascular$obstruction$that$results$from$chronic$left$to$right$shunting$through$a$
congenital$heart$defects.$The$elevated$pulmonary$vascular$resistance$causes$reversal$of$the$original$shunt$(to$the$right$to$left$direction)$and$
systemic$cyanosis$
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References:$
Lilly,$Leonard$S..$Pathophysiology)of)Heart)Disease.$5th$ed.$Lippincott$Williams$&$Wilkins:$Philadelphia,$2011.$