Medical Therapy for Chronic Thromboembolic Pulmonary Hypertension

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Abstract

Prior to the development of pulmonary endarterectomy (PEA), a surgical removal of organized chronic thrombi, there had long been no efficacious treatment to improve the prognosis of patients with chronic thromboembolic pulmonary hypertension (CTEPH). Anticoagulants are initiated when CTEPH is diagnosed to prevent progression, and treatment of hypoxia and right heart failure are added when these complications occur. In the meantime, because small pulmonary arterioles in CTEPH have the same histological abnormality as those in pulmonary arterial hypertension (PAH), which has been called “small vessel disease,” the pulmonary vasodilators used in PAH have started to be administered to patients with inoperable CTEPH with peripheral lesions. Retrospective studies showed effectiveness of these vasodilators in improving the prognosis of CTEPH, and riociguat, a newly developed soluble guanylate cyclase (sGC) stimulator, demonstrated significant clinical benefit in 6-min walk distance (6MWD) and pulmonary hemodynamics. However, the effectiveness of vasodilators in patients with operable CTEPH is not known.

Keywords

Chronic thromboembolic pulmonary hypertension (CTEPH) Anticoagulant Small vessel disease Riociguat Pulmonary vasodilator 
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Copyright information

© Springer Science+Business Media Singapore 2017

Authors and Affiliations

  1. 1.Division of CardiologyKyorin University School of MedicineTokyoJapan

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