Cardiac > Congenital > Fibroelastosis

Endocardial Fibroelastosis:

Clinical:

A marked endocardial thickening and myocardial fibrosis (may see focal areas of necrosis) which involves the left side of the heart. The disorder may be due to an ischemic etiology. Patients present within the first 6months of life and generally die by age 2 years.

X-ray:

On CXR there is cardiomegally with vascular congestion. Left lower lobe atelectasis may be seen due to compression of the bronchus by an enlarged LA/LV.