Contraced endocardial fibroelastosis in childhood: about one case

ABSTRACT

Endocardial fibroelastosis is un uncommon diseases and it has a very bad prognosis since fatal evolution is usual before 2 years old. We report the case of a 20 years old woman who is affected with the contracted form of this disease associated with atrial septal defect [ASD] and mitral regurgitation. This disease was discovered by endocardial biopsy when she was 4 years old and underwent surgical resection of endocardial fibrosis, a path on the ASD and mitral valve replacement. She was rehospitalised 15 years later with heart failure although continuous digitoxin therapy