Hypoplastic Left Heart Syndrome

Question 1

Hypoplasia of left ventricle is associated with:

Answer 1

Mitral stenosis/atresia

Aortic stenosis/atresia

Hypoplastic ascending aorta and coarctation

Question 2

What is the best diagnostic clue on imaging for HLHS

Answer 2

Abnormal 4 chamber view with small, nonapex-forming left ventricle

Question 3

Non cardiac anomalies are include the following:

Answer 3

Diaphragmatic hernias

Major central nervous system anomalies, such as holoprosencephaly (significant adverse impact on prognosis)

Question 4

Differential diagnosis of HLHS

Answer 4

Severe aortic stenosis
Coarctation of aorta
Shone syndrome

Question 5

What is shone syndrome

Answer 5

Supravalvular mitral membrane
Parachute mitral valve,
Sub aortic stenosis and aortic coarctation

Definition is often expanded to include mitral and aortic valve stenosis as well as supravalvular aortic stenosis