title: Cardiomyopathy in cystic fibrosis: lymphoedema of the heart with focal myocardial fibrosis.
abstract: Cardiomyopathy in cystic fibrosis (CF) is an unusual heart disease, mainly characterized by a multifocal fibrosis of the left ventricle. The disorder chiefly occurs in the age group of 1-2 years and leads to fatal cardiac failure. The causal pathogenesis of the disease has not been discovered up to now. In two cases of CF-associated cardiomyopathy we found an oedema (mainly lymphoedema) of the myo- and epicardium and a lymph stasis in lymph vessels and lymph nodes of the heart. Based on a comparative study using animal models we speculate that a) CF may be complicated by a disorder of cardiac lymph circulation, and b) chronic cardiac lymphoedema of the heart in CF can cause focal myocardial damage with fibrosis.
mesh_terms: D006332:Cardiomegaly / Q000209:etiology; D009202:Cardiomyopathies / Q000150:complications*; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications*; D004719:Endomyocardial Fibrosis / Q000150:complications*; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D007223:Infant; D008209:Lymphedema / Q000150:complications*; D008297:Male; D010996:Pleural Effusion / Q000209:etiology