Clinical Presentations and Multimodal Imaging Diagnosis in Chronic Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but life-threatening pulmonary vascular disease caused by the presence of a prolonged thrombus in the pulmonary artery. CTEPH is a distinct disease entity classified as group 4 pulmonary hypertension according to the World Symposium on Pulmonary Hypertension. It is the only potentially curable cause of pulmonary hypertension. However, timely diagnosis and treatment are often hampered by nonspecific symptoms and signs and a lack of physician awareness regarding the condition. Thus, it is important to be familiar with the clinical features of CTEPH and the associated diagnostic processes. Herein, we cover the diagnostic approach for CTEPH using multimodal imaging tools in a clinical setting.