FE Cardiomyopathies & Tumors Flashcards

Fetal cardiomegaly is defined by a cardiothoracic ratio (CTR) greater than:

Answer: 0.50

A cardiothoracic ratio greater than 0.50 (heart area to chest area) defines cardiomegaly in the fetus.

Which metabolic storage disorder is associated with fetal hypertrophic cardiomyopathy and respiratory failure in the newborn?

Answer: Pompe disease (glycogen storage disease type II)

Pompe disease (GSD II) causes accumulation of glycogen in the myocardium, leading to severe hypertrophic cardiomyopathy and respiratory muscle involvement.

Which finding distinguishes hypertrophic cardiomyopathy due to diabetic fetopathy from genetic hypertrophic cardiomyopathy?

Answer: Resolution after birth with improved glucose control vs. persistence

Diabetic fetopathy-related hypertrophic cardiomyopathy typically resolves within months after birth as hyperinsulinism resolves, while genetic HCM persists.

A fetus with a large, hyperechoic mass in the right atrium with a stalk is most consistent with:

Answer: Cardiac myxoma

A pedunculated mass attached by a stalk in the atrium is characteristic of a cardiac myxoma, though rare in fetuses.

Endocardial fibroelastosis (EFE) in the fetus appears on echocardiography as:

Answer: Bright hyperechoic thickening of the endocardium with left ventricular dilation

EFE manifests as hyperechoic (bright) thickening of the endocardium with left ventricular dilation and systolic dysfunction.

Which condition is most commonly associated with secondary endocardial fibroelastosis in the fetus?

Answer: Critical aortic stenosis with evolving hypoplastic left heart

Critical aortic stenosis causing left heart obstruction leads to secondary EFE due to chronic left ventricular hypertension and reduced coronary perfusion.