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.