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Chronic thromboembolic pulmonary hypertension: Medical treatment

Author
Peter F Fedullo, MD
Section Editor
Jess Mandel, MD
Deputy Editor
Geraldine Finlay, MD

INTRODUCTION

Medical therapy for chronic thromboembolic pulmonary hypertension (CTEPH) refers to the use of pulmonary vasodilators and remodeling agents to lower the pulmonary vascular resistance and pulmonary artery pressure, thereby improving symptoms and signs such as exercise capacity and oxygenation. Medical therapy is not curative and its effects are relatively modest; therefore, it is indicated in only a few situations [1,2]. Pulmonary thromboendarterectomy remains the mainstay of therapy for CTEPH.

The indications, outcomes, and approach to treatment with medical therapy are reviewed here. The general approach to the treatment of CTEPH, including general measures such as anticoagulation, and surgical measures such as pulmonary thromboendarterectomy and pulmonary balloon angioplasty are described separately. (See "Overview of the treatment of chronic thromboembolic pulmonary hypertension" and "Chronic thromboembolic pulmonary hypertension: Surgical treatment".)  

INDICATIONS AND OUTCOMES

Pulmonary thromboendarterectomy is the only potentially curative therapy for chronic thromboembolic pulmonary hypertension (CTEPH); thus, there are only a few clinical situations in which medical therapy may be indicated: patients who are not operative candidates; patients who have a suboptimal hemodynamic and functional outcome following pulmonary thromboendarterectomy; and as a bridge to definitive surgical intervention in patients with severe pulmonary hypertension and right heart failure [1,2].

Inoperable and persistent CTEPH are the two most common indications for medical therapy that have stronger evidence to support its efficacy. The use of medical therapy as a bridge to surgery is based upon small observational series and clinical experience.

Medications used to treat CTEPH are those that are used to treat idiopathic pulmonary arterial hypertension. They include drugs in the following classes: prostanoids (epoprostenol, treprostinil, iloprost), soluble guanylate cyclase stimulants (riociguat), endothelin receptor antagonists (bosentan, ambrisentan, macitentan), and phosphodiesterase 5 inhibitors (sildenafil, tadalafil, vardenafil) (table 1). Evidence in support of their use for each indication is discussed in this section. (See "Treatment of pulmonary hypertension in adults", section on 'Advanced therapy'.)

       

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Literature review current through: Nov 2016. | This topic last updated: Tue Sep 13 00:00:00 GMT+00:00 2016.
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