We reviewed medical records, echocardiograms, explanted hearts, and microscopic slides from 52 pediatric heart transplant cases at our institution with a diagnosis of dilated cardiomyopathy (DCM).
Fourteen hearts showed both gross and microscopic findings of primary EFE, with no apparent cause of the diffuse endocardial thickening. Patients with EFE were significantly younger than patients with DCM (median age: 10.1 vs. 142.0 months). No case of EFE was diagnosed clinically. LV wall and endocardial thickness were significantly greater in EFE, with the mitral valve and papillary muscles showing characteristic findings.
Clinically and pathologically, EFE is different from DCM. EFE is not rare and found in 25 % of pediatric cases transplanted for ¡°DCM.¡± EFE should be recognized to promote understanding of the natural history and etiology of EFE.