Cor Vasa 2009, 51(7-8):481-487 | DOI: 10.33678/cor.2009.122

Chronic thromboembolic pulmonary hypertension

Michael Aschermann^1,*, Pavel Jansa¹, David Ambrož¹, Pavel Poláček¹, Jaroslav Lindner², Aleš Linhart¹

¹ 2. interní klinika kardiologie a angiologie

² 2. chirurgická klinika kardiovaskulární chirurgie, Všeobecná fakultní nemocnice a 1. lékařská fakulta Univerzity Karlovy, Praha, Česká republika

Chronic thromboembolic pulmonary hypertension (CTEPH) develops during progressive obstruction of lung vessels through organization of thrombi in the arterial lumen and remodeling of lung arterioles in perfused regions. The result of these alterations is a progressive increase in pulmonary artery pressures, an increase in pulmonary vascular resistance and, in the long run, right-heart failure. While CTEPH was originally believed to develop in about 0.1-0.5% of survivors of acute pulmonary embolism, a number of papers have reported an incidence of up to ten times higher. While the primary goal of diagnosis of CTEPH is to rule out or confirm the presence of pulmonary hypertension as the cause of the patient's problems, this is followed by determining the severity of pulmonary hypertension and identifying its etiology. In those confirmed to have CTEPH, it is critical to determine the location and extent of pulmonary vascular bed involvement. The basic methods used to diagnose CTEPH include Doppler echocardiography, lung scintigraphy and right-heart catheterization. While lesions involving the individual pulmonary artery branches are located using contrast-enhanced computed tomography and magnetic resonance imaging, lung scintigraphy continues to be the gold standard. The optimal therapeutic modality in CTEPH is surgery, i.e., pulmonary artery endarterectomy in patients with surgically accessible obstruction of the individual branches of the pulmonary artery. In properly indicated cases, successful surgery may even result in normalization of pulmonary artery pressures. Alternative treatments in patients ineligible for surgery include drug therapy (prostacyclin, bosentan, sildenafil) or, more rarely, balloon angioplasty or lung transplantation; however, the body of experience is not large. A program of pulmonary artery endarterectomy in the Czech Republic was launched in the Heart Center of the Prague-based General University Hospital in 2004. A total of 118 endarterectomy procedures have been performed in the period from September 2004 through June 2009. A prerequisite for a successful outcome is close multidisciplinary cooperation and experience with comprehensive diagnosis and treatment of pulmonary hypertension.

Keywords: Chronic thromboembolic pulmonary hypertension; Diagnosis; Pulmonary artery endartectomy; Drug therapy; Lung transplantation

Published: July 1, 2009  Show citation