Chronic Thromboembolic Pulmonary Hypertension

Abstract

Under most circumstances, chronic thromboembolic pulmonary hypertension represents a correctable form of pulmonary hypertension. Approximately 1500 thromboendarterectomy procedures have now been performed worldwide. Mortality rates reported by established programs with experience in the management of patients with this disease process have fallen to a range of 6 to 8%. This reduction in mortality has been contributed to by several factors: improved methods of preoperative evaluation and more selective surgical referral, increased surgical experience and refined techniques, and an increased understanding of the unique postoperative problems that occur following pulmonary thromboendarterectomy. Despite these advances, a great deal more needs to be accomplished. The early natural history and pathophysiologic mechanisms of the disease remain uncertain; improved diagnostic techniques are required; and the most feared complication of the procedure, reperfusion pulmonary edema, remains enigmatic in terms of its pathogenesis, prevention, and therapy.