Primary restrictive cardiomyopathy. Non-tropical endomyocardial fibrosis and hypereosinophilic heart disease

Abstract

Eleven patients with a restrictive cardiomyopathy are described. Seven of these had biventricular disease and in them the haemodynamic abnormality resembled that in constrictive pericarditis but the condition was distinguishable because of unequal involvement of the two ventricles, murmurs of atrioventricular valve regurgitation, or characteristic ventricular deformity on angiography. Two of these patients had eosinophilia with the clinical features of Löffer's endocarditis. In 4 patients the disease was apparently confined to the left ventricle; they were investigated on account of atypical chest pain, third heart sound, or abnormal electrocardiogram. Simultaneous measurements of left ventricular pressure and volume throughout diastole were made in 3 patients and showed rapid but abruptly curtailed left ventricular filling. Transvenous endomyocardial biopsy in 2 patients showed fibrous tissue with collagen and irregular elastic fibrils. Surgical biopsy in 3 patients excluded pericardial constriction but was diagnostically unhelpful because of failure to obtain endocardium. Necropsy in one patient showed that the heart had features indistinguishable from tropical endomyocardial fibrosis. It is suggested that the spectrum of ventricular abnormalities in these patients results from endomyocardial fibrosis of varying severity and probably of differing cause. This study has shown that "tropical" endomyocardial fibrosis may occur outside the tropics and suggests that eosinophilia may play a leading role or associated part in the genesis of some cases.