An Update on the Management of Chronic Thromboembolic Pulmonary Hypertension Article

Full Text via DOI: 10.1016/j.cpcardiol.2016.11.001 PMID: 27989311 Web of Science: 000391348200002

Cited authors

  • Edward, Justin A.; Mandras, Stacy


  • Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but life-threatening form of pulmonary artery hypertension that is defined as a mean arterial pulmonary pressure greater than 25 mm Hg that persists for more than 6 months following anticoagulation therapy in the setting of pulmonary emboli. CTEPH is categorized by the World Health Organization as group IV pulmonary hypertension and is thought to be due to unresolved thromboemboli in the pulmonary artery circulation. Among the 5 classes of pulmonary hypertension, CTEPH is unique in that it is potentially curable with the use of pulmonary thromboendarterectomy surgery. Despite an increasing array of medical and surgical treatment options for patients with CTEPH over the past 2 decades, patients commonly present with advanced disease and carry a poor prognosis, thus, the need for early diagnosis and appropriate referral to an expert center. This review article first highlights the epidemiology, pathophysiology, and clinical presentation of CTEPH. The article then provides diagnostic and therapeutic algorithms for the management of the patient with suspected CTEPH.

Publication date

  • 2017

Published in

International Standard Serial Number (ISSN)

  • 0146-2806

Start page

  • 7

End page

  • 38


  • 42


  • 1