congenital heart defects

Question 1

L→R shunts

Answer 1

VSD (most common CHD)
ASD
PDA

Question 2

symptoms of L→R shunts

Answer 2

late cyanosis “blue kids”
less dangerous in short term, less severe, more common than R→L shunt
asymptomatic until exercise: SOB, cyanosis

Question 3

R→L shunts

Answer 3

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)

Question 4

symptoms of R→L shunts

Answer 4

early cyanosis “blue babies”: deoxy blood into systemic circulation, bypass lungs
diagnosed prenatally or immediately after birth
require urgent surgical correction or PDA maintenance

Question 5

harsh holosystolic murmur

Answer 5

VSD

when ventricles contract: LV → RV

Question 6

loud S1

wide, fixed S2

Answer 6

ASD

Question 7

continuous, machine-like murmur in LUSB

Answer 7

PDA

Question 8

VSD presentation

Answer 8

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

Question 9

cause of VSD

Answer 9

interventricular septum defect

Question 10

cause of ASD

Answer 10

septum are missing tissue (not unfused septum primum + septum secundum causing PFA)
foramen ovale (of septum secundum) and foramen secundum (of septum primum) overlap

Question 11

ASD presentation

Answer 11

asymptomatic
larger lesion: RVH → heart failure
loud S1, wide, fixed split S2

Question 12

PDA presentation

Answer 12

late cyanosis in lower extremities

continous machine-like murmur

Question 13

cause of PDA

Answer 13

higher pressure in aorta → lower pressure in pulmonary trunk (↓ lung resistance with first breath)
progressive RVH and/or LVH → heart failure

Question 14

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

Answer 14

Eisenmenger syndrome

Question 15

↑ 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

Answer 15

coarctation of aorta

Question 16

conditions that require maintenance of ductus arteriosus with PGE2

Answer 16

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)

Question 17

cause of persistent truncus arteriosus

Answer 17

failure of neural crest cells to cause aorticopulmonary septum to divide the ascending aorta and pulmonary trunk
usually have VSD too - mixes blood

Question 18

presentation of truncus arteriosus

Answer 18

infant with cyanosis, respiratory distress, heart failure

VSD: RV blood mixes with LV blood → leaves for systemic circulation

Question 19

cause of transposition of great vessels

Answer 19

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

Question 20

cause of tricuspid atresia

Answer 20

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

Question 21

cause of total anomalous pulmonary venous return (TAPVR)

Answer 21

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)

Question 22

cause of tetralogy of fallot

Answer 22

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)

Question 23

boot shaped heart on xray, think

Answer 23

kid: RVH due to tetralogy of fallot
adult: RVH due to COPD or primary pulmonary HTN

Question 24

effect of squatting in tetralogy of fallot

Answer 24

↑ 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

Question 25

presentation of tetralogy of fallot

Answer 25

``` 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) ```

Question 26

CHD associated with preexisting maternal diabetes

Answer 26

transposition of great vessels

Question 27

CHD associated with maternal lithium use

Answer 27

Ebstein anomaly

Question 28

CHD associated with alcohol use

Answer 28

TOF, VSD, ASD, PDA

Question 29

CHD associated with DiGeorge syndrome (22q11 syndrome)

Answer 29

truncus ateriosus | TOF

Question 30

CHD associated with Down Syndrome

Answer 30

endocardial cushion defect: ASD or VSD

Question 31

CHD associated with congenital rubella

Answer 31

pulmonary artery stenosis | PDA

Question 32

CHD associated with Turner Syndrome

Answer 32

bicuspid aortic valve | infantile coarctation of aorta

Question 33

CHD associated with Marfan Syndrome

Answer 33

thoracic aortic aneurysm and dissection | aortic regurgitation

Question 34

what is name for this condition: tricuspid leaflets displaced into RV → hypoplastic RV tricuspid regurgitation or stenosis

Answer 34

ebstein anomaly

Question 35

complication of ebstein anomaly

Answer 35

dilated RA → risk for SVT or WPW

Question 36

widely split S2 + tricuspid regurgitation

Answer 36

ebstein anomaly

Question 37

cause of patent foramen ovale

Answer 37

unfused septum primum + septum secundum after birth | can lead to paradoxical emboli (venous thromboembolus enters arterial circulation)

Question 38

complication of patent foramen ovale or ASD

Answer 38

can lead to paradoxical emboli (venous thromboembolus enters arterial circulation)

Question 39

what type of CHD would ↑afterload (squat or knees to chest in infant) be beneficial

Answer 39

``` R→L shunt: TOF transposition of great vessels truncus arteriosus eisenmenger syndrome with VSD, ASD, or PDA ```