Congenital Heart Disease

Question 0

Truncus Arteriosus

Answer 0

Cyanotic (1)
Common great vessel with varying valve
Most have simultaneous VSD
22q11 associated

Question 1

Pulmonary atresia

Answer 1

Cyanotic heart lesion (0)

Question 2

Transposition of Great Vessels

Answer 2

Cyanotic (2)
Requires some combination of PDA, VSD, and ASD
Require PGs to keep DA patent
Associated with maternal diabetes

Question 2

Tricuspid Atresia

Answer 2

Cyanotic (3)
Atretic tricuspid valve
Hypo plastic right ventricle
Requires both ASD and VSD for survival

Question 4

Tetralogy of Fallot

Answer 4

Cyanotic (4)
Pulmonic stenosis, overriding aorta, RVH, VSD
Degree of cyanosis depends on stenosis and subsequent right to left shunt
22q11 associated

Question 5

Total Anomalous Pulmonary Venous Return (TAPVR)

Answer 5

Cyanotic (5)
Pulmonary veins don’t return to left atrium; aberrant connection causes return to right side circulation
Obstructive TAPVR has connections to small veins fluid backs up into lungs
Associated with PDA or ASD

Question 5

PDA

Answer 5

Normal DA flow in infant from PA to Aorta (ie right to left)
In persistently PDA, shunt reverses to aorta to PA (ie left to right)
–later in life demonstrate differential lower extremity cyanosis
Machine like murmur
Associated with congenital rubella

Question 7

Coarctation of the Aorta

Answer 7

Two types:
-infantile: stenosis proximal to DA, check femoral pulses, Turner associated

-adult: post ductal stenosis, upper limb HTN, rib notching, poor lower limb pulses

Question 7

VSD

Answer 7

Pathologic
Acyanotic
Harsh blowing holosystolic murmur @ LLSB
Many resolve spontaneously, but failure to close can lead to Eisenmenger syndrome

Question 8

ASD

Answer 8

Acyanotic
Diastolic flow murmur with wide/fixed split S2
LUSB
Left or right shunting
Can lead to Eisenmenger if untreated and severe overflow/shunting

Question 9

Tricuspid Regurgitation

Answer 9

Transient systolic murmur @LLSB
Innocent but must be monitored for persistence
Result of elevated PVR at birth

Question 11

Peripheral Pulmonary Stenosis

Answer 11

Innocent murmur
Early systolic murmur @ LUSB radiating to axilla and back
Turbulence at pulmonary artery branch point

Question 12

Venous Hum

Answer 12

Innocent murmur
Continuous soft humming @ neck/right upper chest that stops when supine
Turbulent flow in the jugular and caval systems

Question 13

Hypoplastic Left heart

Answer 13

Hypoplasia of left vent, stenosis/atresia of mitral aortic valve and ascending aorta
Pulmonary venous blood returns to right heart through ASD and is pumped to the lungs and systemically via PDA
Present with closure of DA with cardiogenic shock