Dtsch Med Wochenschr. 2001 Feb; 126(7): 168-71.

[Symptoms of primary endocardial fibroelastosis in a young adult]

Meyer T, Reiter H, Mirzaie M, Hasenfuss G, Unterberg C.

Abteilung Kardiologie und Pulmonologie, Universität GÃƒÂ¶ttingen.

HISTORY AND ADMISSION FINDINGS: A 21-year-old woman with known endocardial fibroelastosis diagnosed when aged 3 months was admitted because of progressive dyspnoea. The physical examination revealed symptoms of heart failure, with pulmonary rales, mild hepatomegaly, and tachyarrhythmia. INVESTIGATIONS: The electrocardiogram showed atrial fibrillation, complete right bundle branch block and right ventricular hypertrophy. Echocardiography indicated hypertrophy and dilatation of the right ventricle (61 mm) with tricuspid regurgitation and hypoplasia of the left ventricle. Heart catheterization confirmed pulmonary hypertension (60/46 mmHg) as well as dilatation and hypokinesia of the right ventricle. Right ventricular biopsy showed severe myocardial hypertrophy resulting from secondary pulmonary hypertension, while no evidence of myocarditis or idiopathic dilated cardiomyopathy was found. TREATMENT AND COURSE: Symptoms of heart failure improved under medical treatment with digitalis, angiotensin-converting enzyme inhibitor and diuretics. CONCLUSION: Primary endocardial fibroelastosis of the contracted type must be included in the differential diagnosis of heart failure occurring in young adults.