Endocardial fibroelastosis in children

Endocardial fibroelastosis (EFE), also known as endocardial sclerosis, is a common cardiomyopathy of unknown etiology in infants and young children. The disease often presents with a sudden decline in ventricular diastolic and systolic function, ultimately leading to congestive heart failure, which progressively worsens. If left untreated, death often occurs within days or weeks. The acute phase can manifest as cardiogenic shock (heart failure) or sudden death. Pathological examination reveals significant endocardial thickening, with proliferation of elastic and collagen fibers, resulting in diffuse thickening of the left ventricular endocardium.