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A 50 year old woman presented with axillary vein thrombosis and was diagnosed as having the antiphospholipid syndrome. The patient also had hypereosinophilia and developed features of severe right heart failure. A transthoracic echocardiogram revealed features characteristic of hypereosinophilic cardiomyopathy leading to endomyocardial fibrosis. Various cardiac manifestations associated with antiphospholipid syndrome have been reported, including dilated cardiomyopathy, mural thrombi, valvar heart disease, non-bacterial vegetations, and pericardial effusion. However, the association between endomyocardial fibrosis and antiphospholipid syndrome has not been reported before.
The apical four chamber transthoracic echocardiogram shows endomyocardial fibrosis. The infiltration has characteristically involved the apical segments of both right (RV) and left (LV) ventricles leading to virtual obliteration of the apices in systole (top, arrows). The right ventricle cavity is very small and the right atrium (RA) is very enlarged. The diastolic view (bottom) reveals tethering of the apical interventricular septum. The basal segment of the interventricular septum is bowing to the left ventricle because of the raised intracavity pressure in the right ventricle during diastole.