Congenital Disorders

Question 1

Late cyanosis, clubbing, and polycythemia

Answer 1

Eisenmenger’s

ASD, VSD, and PDA

VSD > ASD > PDA - all start out with left-to-right shunting

Question 2

Fixed splitting

Answer 2

ASD (increased right preload)

Question 3

Pulmonary stenosis, RVH, Overriding aorta, and VSD

Answer 3

TOF

Most important determinant of shunting = stenosis

Question 4

Squatting increases afterload and decreases cyanotic right to left shunt in _______

Answer 4

TOF

Question 5

Boot-shaped heart

Answer 5

TOF

Question 6

Separation of systemic and pulmonary circulations - requires a patent VSD, PDA, or foramen ovale

Answer 6

Transposition of great vessels

Question 7

Give PGE to maintain PDA in this condition

Answer 7

Transposition of great vessels

Question 8

Aortic stenosis proximal to PDA, distal to aortic arch

Answer 8

Aortic coarctation, infantile form

Presents with cyanosis

Question 9

Check femoral pulses on exam

Answer 9

Aortic coarctation

Question 10

Stenosis is distal to ligamentum arteriosum (no PDA). Associated with notching of the ribs. HTN in upper extremities.

Answer 10

Adult coarctation

Question 11

Associated with bicuspid aortic valve

Answer 11

Adult coarctation

Question 12

Associated with Turner syndrome

Answer 12

Aortic coarctation, infantile form

Question 13

Associated with 22q11 syndromes

Answer 13

Truncus arteriosus, TOF

Question 14

Associated with Down’s

Answer 14

Primum Ostium Secundum (ASD) + VSD + AV septal defect (endocardial cushion defect)

Question 15

Assiciated with congenital rubells

Answer 15

Septal defects, PDA, pulmonary artery stenosis

Question 16

Associated with maternal diabetes

Answer 16

Transposition of great vessels

Question 17

Lower extremity cyanosis late in life

Answer 17

PDA, Eisenmenger (arises distal to aortic arch)

Question 18

Paradoxical emboli are an important complication

Answer 18

ASD, patent foramen ovale