Update on the Diagnosis and Treatment of Chronic Thromboembolic Pulmonary Hypertension: Diagnostic Imaging and Treatment Pathways in a New Era

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, chronic, progressive, and life-limiting condition with a poor five-year survival in untreated patients with advanced disease. Multi-modality imaging techniques are used in making the diagnosis and determining treatment options in CTEPH. Pulmonary endarterectomy (PEA) surgery is the treatment of choice in selected patients, providing excellent long-term survival. Balloon pulmonary angioplasty (BPA) is an established intervention for selected patients who are not suitable for surgery. Riociguat, a soluble guanylate cyclase stimulator, is a vasodilator and indicated in patients with inoperable disease. In this state-of-the-art review, we provide an update in the diagnostic evaluation with a focus on imaging modalities, treatment strategies, and future directions in CTEPH.