Right-sided endomyocardial fibrosis with recurrent pulmonary emboli leading to irreversible pulmonary hypertension

AUTOR(ES)

Ribeiro, P A

RESUMO

A 26 year old Saudi man with features of both Loeffer's endocarditis and endomyocardial fibrosis presented with mild symptoms and pulmonary emboli. Echocardiographic examination showed obliteration of the right ventricular apex by an attached mass. The results of haemodynamic studies were somewhat abnormal and medical treatment was started. Despite anticoagulation with warfarin the patient's condition deteriorated rapidly over a four month period after a further episode of pulmonary embolism and the development of pulmonary hypertension. Two haemodynamic studies performed four months apart were typical of pulmonary hypertension and later right ventricular failure; they showed none of the characteristics of restriction. Pulmonary embolectomy was attempted but there was no cleavage plane between the organised thrombi and the endothelium of the pulmonary artery. The patient died of severe pulmonary hypertension and right ventricular failure several days after operation.

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