congenital heart defects Flashcards
L→R shunts
VSD (most common CHD)
ASD
PDA
symptoms of L→R shunts
late cyanosis “blue kids”
less dangerous in short term, less severe, more common than R→L shunt
asymptomatic until exercise: SOB, cyanosis
R→L shunts
5 T's Truncus arteriosus (1 vessel) Transposition of great vessels (2 vessels switched) Tricuspid atresia (3 = Tri) Tetrology of Fallot (4 defects = Tetra) (most common cause of early childhood cyanosis) Total anomalous pulmonary venous return (TAPVR) (5 letters)
symptoms of R→L shunts
early cyanosis “blue babies”: deoxy blood into systemic circulation, bypass lungs
diagnosed prenatally or immediately after birth
require urgent surgical correction or PDA maintenance
harsh holosystolic murmur
VSD
when ventricles contract: LV → RV
loud S1
wide, fixed S2
ASD
continuous, machine-like murmur in LUSB
PDA
VSD presentation
asymptomatic at birth
most resolve (esp first 6 mo)
may manifest weeks after birth or remain asymptomatic in life
larger lesion require surgery: LV overload → heart failure
harsh holosytolic mumur
cause of VSD
interventricular septum defect
cause of ASD
septum are missing tissue (not unfused septum primum + septum secundum causing PFA) foramen ovale (of septum secundum) and foramen secundum (of septum primum) overlap
ASD presentation
asymptomatic
larger lesion: RVH → heart failure
loud S1, wide, fixed split S2
PDA presentation
late cyanosis in lower extremities
continous machine-like murmur
cause of PDA
higher pressure in aorta → lower pressure in pulmonary trunk (↓ lung resistance with first breath)
progressive RVH and/or LVH → heart failure
name this condition:
longstanding, uncorrected L→R shunt (VSD, ASD, PDA) → ↑pulmonary blood flow → ↑pulmonary circulation pressure → progressive pulmonary HTN→ RVH →now R→L shunt → late cyanosis, clubbing, SOB
Eisenmenger syndrome
↑ blood pressure in upper extremities, ↓ blood pressure + weak, delayed pulses in lower extremities
other associations:
notching of ribs: intercostal arteries dilated along inferior edge = collateral circulation
aortic regurgitation: abnormal valve or ↑afterload damages valve→ regurgitation → heart failure
coarctation of aorta
conditions that require maintenance of ductus arteriosus with PGE2
infantile coarctation of aorta (narrowed aorta proximal to DA)
transposition of great vessels (need PGE2 to allow oxygenated left sided blood into aorta → systemic circulation until surgery, otherwise die in first few months)
cause of persistent truncus arteriosus
failure of neural crest cells to cause aorticopulmonary septum to divide the ascending aorta and pulmonary trunk
usually have VSD too - mixes blood
presentation of truncus arteriosus
infant with cyanosis, respiratory distress, heart failure
VSD: RV blood mixes with LV blood → leaves for systemic circulation
cause of transposition of great vessels
RV →aorta and LV → pulmonary trunk
aorticopulmonary septum doesn’t spiral
separation of systemic + pulmonary circulation
need shunt to allow mixing of blood (otherwise not compatible with life): VSD, ASD, PDA, patent foramen ovale
cause of tricuspid atresia
absence of tricuspid valve (no RA to RV opening) → hypolastic RV need ASD (deoxy RA blood → LA) AND VSD (LA → LV → RV → oxygenate blood) to be compatible with life
cause of total anomalous pulmonary venous return (TAPVR)
pulmonary veins drain into right-heart circulation (SVC or coronary sinus) → RA
closed loop → no deoxygenated blood going into LA → aorta
need R→L shunt to maintain CO (ASD, PDA)
cause of tetralogy of fallot
displacement of infundibular septum
IHOP
1) Interventricular septal defect = VSD
2) Hypertrophy - RVH (push against stenoic valve)
3) Overriding aorta (sits over VSD)
4) Pulmonic valve stenosis (RV outflow obstruction)
boot shaped heart on xray, think
kid: RVH due to tetralogy of fallot
adult: RVH due to COPD or primary pulmonary HTN
effect of squatting in tetralogy of fallot
↑ systemic vascular resistance → ↑afterload → R→L shunt becomes L→R shunt → VSD allows deoxy blood to be forced to R side into lungs for oxygenation→ improves cyanosis
presentation of tetralogy of fallot
most time: L→R shunt across VSD (acyanotic, RV pushes harder to get across pulmonic stenosis) some times (crying, feeding, ↑ activity): R → L shunt across VSD (cyanotic)
CHD associated with preexisting maternal diabetes
transposition of great vessels
CHD associated with maternal lithium use
Ebstein anomaly
CHD associated with alcohol use
TOF, VSD, ASD, PDA
CHD associated with DiGeorge syndrome (22q11 syndrome)
truncus ateriosus
TOF
CHD associated with Down Syndrome
endocardial cushion defect: ASD or VSD
CHD associated with congenital rubella
pulmonary artery stenosis
PDA
CHD associated with Turner Syndrome
bicuspid aortic valve
infantile coarctation of aorta
CHD associated with Marfan Syndrome
thoracic aortic aneurysm and dissection
aortic regurgitation
what is name for this condition:
tricuspid leaflets displaced into RV → hypoplastic RV
tricuspid regurgitation or stenosis
ebstein anomaly
complication of ebstein anomaly
dilated RA → risk for SVT or WPW
widely split S2 + tricuspid regurgitation
ebstein anomaly
cause of patent foramen ovale
unfused septum primum + septum secundum after birth
can lead to paradoxical emboli (venous thromboembolus enters arterial circulation)
complication of patent foramen ovale or ASD
can lead to paradoxical emboli (venous thromboembolus enters arterial circulation)
what type of CHD would ↑afterload (squat or knees to chest in infant) be beneficial
R→L shunt: TOF transposition of great vessels truncus arteriosus eisenmenger syndrome with VSD, ASD, or PDA
