Purpose of review : Chronic thromboembolic pulmonary hypertension (CTEPH) is an important cause of pulmonary hypertension. Although surgery is potentially curative, some patients present with inoperable disease. In these patients, medical therapies for pulmonary arterial hypertension are increasingly being used.

Recent findings : The pathobiology of CTEPH development remains incompletely understood; however, evidence supports both large and small vessel disorder in patients with the disease. Surgical thromboendarterectomy is an increasingly well tolerated and often curative procedure and is the management strategy of choice for most patients. Although excellent outcomes in surgical management have been noted, the role of medical management in selected patients with inoperable or recurrent or persistent disease after surgery is increasing. A recent large, randomized controlled clinical trial of riociguat in CTEPH demonstrated improvements in exercise capacity, functional class, and hemodynamics. A safe, effective angioplasty approach to CTEPH is being pursued in addition.

Summary : The approach to CTEPH management in the operable patient remains surgical, without clear benefit to preoperative pulmonary arterial hypertension-specific therapy at this time. Patients with inoperable disease or pulmonary hypertension following thromboendarterectomy, however, should be considered for medical management, with riociguat currently having the strongest evidence specific to CTEPH.