Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Chronic thromboembolic pulmonary hypertension (CTEPH) is a relatively rare consequence of recurrent acute pulmonary embolic events. Screening for CTEPH post-acute PE is recommended only in patients with persistent symptoms. The disease is characterized by a combination of organized fibrotic clots in the larger proximal pulmonary arteries and a small vessel vasculopathy analogous to that seen in idiopathic PAH. The clinical presentation of CTEPH is one of progressive dyspnea followed by development of right heart failure. Diagnosis requires right heart catheterization together with an abnormal ventilation-perfusion lung scintigraphy scan. Lifelong anticoagulation is indicated. Treatment of choice in appropriate cases is surgical pulmonary thromboendarterectomy (PEA). Balloon pulmonary angioplasty (BPA) as well as medical therapy improve hemodynamics and functional class in patients not suitable for surgical intervention. There are ongoing studies aimed at identifying which treatment option or combination of treatments, are best suited for which patients.