SUCCESSFUL PULMONARY THROMBOENDARTERECTOMY IN 2 PATIENTS WITH SICKLE-CELL DISEASE

Patients with sickle cell disease have been reported to have an increa sed risk of thromboembolism and pulmonary hypertension. Some of these patients may benefit from pulmonary thromboendarterectomy (PTE), a pro cedure that requires profound hypothermia, cardiopulmonary bypass, and periods of circulatory arrest, factors that may potentially increase the risk of sickling. Two patients with sickle cell disease (sickle-th alassemia [Hb S/beta+] and Hb SS) presented to the Pulmonary Vascular Center of UCSD Medical Center with significant shortness of breath and limitation of daily activities. Both of these patients were found to have surgically accessible chronic thromboembolic disease with pulmona ry hypertension. PTE was performed in both patients using exchange tra nsfusion, with avoidance of anemia, hypoxia, and acidosis. A successfu l outcome with resolution of pulmonary hypertension was achieved in bo th cases. To our knowledge this is the first report of patients with s ickle cell disease who successfully underwent PTE for chronic thromboe mbolic pulmonary hypertension.