Pulmonary thromboendarterectomy in a case of hereditary stomatocytosis

We present a case of pulmonary thromboendarterectomy performed successfully in a patient with stomatocytosis. Stomatocytosis is a rare condition of abnormal erythrocyte morphology in which haemolysis and hyperkalaemia occur at cooler temperatures. A 35-yr-old male with stomatocytosis was referred for pulmonary thromboendarterectomy in the context of chronic thromboembolic pulmonary hypertension. He had undergone splenectomy as a child, which rendered him hypercoagulable as the spleen normally removes the haemolysed red cell fragments from blood. By constantly monitoring urine for macroscopic haematuria, arterial and mixed venous blood gas analysis perioperatively and by limiting the period of deep hypothermic circulatory arrest that is normally required for this operation, we were able to perform the operation successfully.