Congenital

Question 1

Ventricular Septal Defect (epidemiology and association)

Answer 1

Most common congenital heart defect and FETAL ALCOHOL SYNDROME

Question 2

Eisenmenger Syndrome

Answer 2

Increased pHTN eventually gets so big that you get right to left shunt and cyanosis/RV hypertrophy/polycythemia/clubbing

Question 3

Atrial Septal Defect (ostium primum and secundum epidemiology, clinical finding, important complication)

Answer 3

Usually secundum (90%), but primum is associated with Down's Syndrome
Split S2 bc increase in blood in R heart delays pulmonary valve closure
Paradoxical emboli

Question 4

Patent Ductus Arteriosus (disease association, 2 clinical findings, treatment)

Answer 4

Associated w/ congenital rubella
Holosystolic murmur. Later in life can lead to lower extremity cyanosis from Eisenmenger’s
Treated with Indomethacin, which decreases PGE (because PGE maintains patency)

Question 5

Tetralogy of Fallot (4 components, which determines severity, clinical finding, radiographic finding)

Answer 5

1. Stenosis of RV outflow tract (determines degree of shunt)
2. RV hypertrophy
3. Ventricular Septa Defect
4. Aorta that overrides the VSD
   Patients learn to squat in cyanotic spell to increase arterial resistance
   “Boot shaped” heart on XR

Question 6

Transposition of Great Vessels (disease association and treatment)

Answer 6

Maternal diabetes

Administer PGE to maintain PDA until surgical repair

Question 7

Tricuspid Atresia

Answer 7

Tricuspid fails to develop so get RV atresia, usually associated with ASD and get RL shunt

Question 8

Infantile Coarctation of the Aorta (what it is, presentation, disease association)

Answer 8

Narrowing of aorta usually with PDA, after the AoA but before the PDA so get a RL shunt because of the decreased pressure in aorta after the narrowing
Lower extremity cyanosis in infants
Associated with TURNER syndrome

Question 9

Adult Coarctation of the Aorta (what it is, presentation, radiographic finding, association)

Answer 9

Narrowing not associated with PDA, still after AoA
Presents as hypertension in UEs and hypotension/weak pulses in LEs classically discovered in adulthood
Collateral circulation develops across intercostals, causing “notching” of the ribs
Associated with bicuspid aortic valve