Chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension has proven amenable to surgical correction. In experienced hands, perioperative mortality rates have declined to the range of 6 to 8 percent. Long-term follow-up in the majority of patients undergoing thromboendarterectomy has revealed a sustained improvement in hemodynamic and functional status. As experience with this disease has evolved, new challenges have emerged. The contribution of the distal pulmonary vascular bed to the progression of the disease has taken on new importance. Furthermore, reperfusion pulmonary edema, a major cause of morbidity and mortality following the procedure, remains enigmatic in terms of its pathophysiologic mechanisms and potential therapeutic interventions. This article reviews the advances that have occurred in the approach to the patient with the chronic thromboembolic pulmonary hypertension as well as the areas of uncertainty that persist.