Interv Akut Kardiol. 2016;15(4):172-176 | DOI: 10.36290/kar.2016.034

Chronic thromboembolic pulmonary hypertension

Pavel Jansa¹, David Ambrož¹, Jan Kuchař¹, Michael Aschermann¹, Aleš Linhart¹, Jaroslav Lindner²

¹ II. interní klinika kardiologie a angiologie, Centrum pro plicní hypertenzi,

Všeobecná fakultní nemocnice a 1. lékařská fakulta UK, Praha

² II. chirurgická klinika kardiovaskulární chirurgie, Všeobecná fakultní nemocnice a 1. lékařská fakulta UK, Praha

Pulmonary hypertension is a syndrome characterized by a mean pulmonary artery pressure which exceeds 25 mm Hg. Chronic thromboembolic

pulmonary hypertension (CTEPH) represents its third leading cause. CTEPH is caused by both persistent thrombotic obstruction

of the pulmonary arteries and peripheral vascular remodelling. As a result, pulmonary vascular resistance increases, leading to right

ventricular overload and right-sided heart failure. Ventilation/perfusion lung scintigraphy is a crucial imaging modality for detecting

CTEPH. CT angiography and conventional angiography along with invasive haemodynamic assessment are used for the definitive

diagnosis of the disease.The majority of patients with CTEPH are treatable and often curable with surgical pulmonary endarterectomy

(PEA). Patients with inoperable CTEPH due to peripheral involvement and those with persistent pulmonary hypertension after PEA are

potential candidates for specific vasodilator therapy. Previous randomized clinical trials have failed to show a beneficial effect of iloprost,

sildenafil, and bosentan in these patients. Riociguat, a soluble guanylate cyclase stimulator, is the first drug the efficacy of which has

been demonstrated for this indication. In selected cases, treatment also involves balloon angioplasty and lung transplantation.

Keywords: chronic thromboembolic pulmonary hypertension, ventilation/perfusion lung scintigraphy, pulmonary endarterectomy,

Published: December 1, 2016  Show citation