Recurrent left ventricular thrombus after the direct surgical thrombectomy in Fabry disease

A 60-year-old woman with a recent history of presumed cardiogenic cerebral infarction was referred for surgical removal of a left ventricular mass. She was diagnosed with Fabry disease eight years before. Transthoracic echocardiography showed a mobile echogenic mass in the left ventricular apex. Emergency surgery was carried out to prevent the recurrence of embolism. On cardiopulmonary bypass, the left ventricle was opened in its apical portion and a pedunculated mass was removed from the left ventricular wall. Direct suturing closure followed. The mass was histologically a thrombus composed mainly of erythrocytes and fibrin, without a tumor component. The patient was put on a standard “heparin taken over by warfarin” anticoagulation regimen. The postoperative course was clinically uneventful, but immediate transthoracic echocardiography showed an immobile thrombus on the left ventricular suture line. The patient was followed up with intensified anticoagulation and additional antiplatelet therapy. At 7 months after surgery, the patient had not experienced recurrence of embolism. Early thrombogenesis at the surgical site against anticoagulation may feature the highly thrombogenic status in Fabry disease. Therefore, early and strong warfarinization with antiplatelet therapy may be recommended for patients who undergo cardiotomy with this pathology.

Learning objective

Fabry disease accelerates thrombogenesis in blood vessels but does so uncertainly in the cardiac chambers. We describe a patient who developed cerebral infarction from left ventricular thrombosis. After a successful surgical removal of the thrombus from the left ventricle, a new thrombus recurred over the ventricular suture line against a diligent but standard anticoagulation regimen.