Congenital

Question 1

What is Eisenmenger syndrome?

Answer 1

Large ASD - left to right shunt, right sided pressures increase causing pulmonary HTN and reversal of shunt direction - leads to cyanosis

Question 2

List 2 syndromes associated with pulmonary artery stenosis

Answer 2

Williams - (classically supravalvular stenosis),
Noonan - multiple PAS

Other associations: rubella, Ehlers danlos
Adults - iatrogenic, vasculitis, chronic PE, etc.

Question 3

Most common congential cardiac malformation

Answer 3

Bicuspid aortic valve

Question 4

Complications of bicuspid aortic valve

Answer 4

AS, AR, endocarditis, aortic dilation, aneurysm, dissection

Question 5

Associations with BAV

Answer 5

Coarctation
Interruption of aorta
VSD
Marfans/Turner’s - generalized loss of elastic tissue

Question 6

Most common vascular ring

Answer 6

Double aortic arch

Question 7

Syndrome associated with double aortic arch

Answer 7

DiGeorge (22q11 deletion), abnormalities of palate, larynx and trachea

Question 8

Which heterotaxy syndrome has a higher association with CHD

Answer 8

Right-sided (asplenia) - things just aren’t right

Left sided have complex congenital anomalies - AV canal

Question 9

What is the Shone complex?

Answer 9

Complex of obstructive lesions - mitral valve stenosis or parachute deformity, bicuspid aortic valve, aortic coarctation