Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2016 Oct-Dec;12(4):195-198.
doi: 10.14797/mdcj-12-4-195.

Epidemiology and Pathophysiology of Chronic Thromboembolic Pulmonary Hypertension: Risk Factors and Mechanisms

Sarah Medrek  1 , Zeenat Safdar  2
Affiliations
Review

Epidemiology and Pathophysiology of Chronic Thromboembolic Pulmonary Hypertension: Risk Factors and Mechanisms

Sarah Medrek et al. Methodist Debakey Cardiovasc J. 2016 Oct-Dec.

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) occurs when thromboemboli travel to the pulmonary vasculature, fail to resolve, and cause elevated pulmonary arterial pressure. Untreated, this disease leads to progressive right heart failure and death. It develops in approximately 1% to 5% of patients who suffer an acute pulmonary embolism (PE) and has an overall incidence of 3 to 30 per million in the general population. While it is not entirely evident why most but not all people are able to clear this clot burden, there are known risk factors for the development of CTEPH. These include signs of right heart strain at the time of incident PE, inherited coagulopathies, inflammatory conditions, hypothyroidism, and a history of splenectomy. Since CTEPH can be treated both surgically and medically, it is critical to understand the pathophysiology of the disease so affected patients can be identified and diagnosed appropriately.

Keywords: chronic thromboembolic pulmonary hypertension; pulmonary arterial hypertension; pulmonary embolism; pulmonary hypertension.

PubMed Disclaimer

References

    1. Fedullo PF, Auger WR, Kerr KM, Rubin LJ.. Chronic thromboembolic pulmonary hypertension. N Engl J Med. 2001. November 15; 345( 20): 1465– 72. - PubMed
    1. Simonneau G, Gatzoulis MA, Adatia I, . et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013. December 24; 62( 25 Suppl): D34– 41. - PubMed
    1. Lang IM, Pesavento R, Bonderman D, Yuan JX.. Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: a current understanding. Eur Respir J. 2013. February; 41( 2): 462– 8. - PubMed
    1. Schweikert B, Pittrow D, Vizza CD, . et al. Demographics, clinical characteristics, health resource utilization and cost of chronic thromboembolic pulmonary hypertension patients: retrospective results from six European countries. BMC Health Serv Res. 2014. June 9; 14: 246. - PMC - PubMed
    1. Delcroix M, Lang I, Pepke-Zaba J, . et al. Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension: Results From an International Prospective Registry. Circulation. 2016. March 1; 133( 9): 859– 71. - PubMed