Contracted endocardial fibroelastosis in children: report of a case

Endocardial fibroelastosis is un uncommon disease 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 patch on the ASD and mitral valve replacement. She was rehospitalised 15 years later with heart failure although continuous digitoxin therapy.