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Overview of the treatment of chronic thromboembolic pulmonary hypertension

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


The natural history of acute pulmonary embolism is near-total resolution with minimal residual abnormalities; however, a minority of patients will develop chronic thromboembolic pulmonary hypertension (CTEPH) [1,2]. The exact incidence of CTEPH is unknown, but several series suggest that it occurs in 0.57 to 3.8 percent of survivors of acute pulmonary embolism and in over 10 percent of those with recurrent pulmonary embolism [3-7]. Patients with untreated CTEPH are likely to develop progressive disease and have a high risk of dying from right heart failure; therefore virtually all patients should receive treatment [8]. In one study, a mean pulmonary artery pressure >30 mmHg (3.99 kPa) appeared to be a threshold value portending a poor prognosis [9]. Surgery is the only definitive therapy for CTEPH. Medical therapy is not curative and its effects are relatively modest; therefore, it is indicated in only a few situations.

The approach to the treatment of CTEPH is reviewed here. The clinical presentation, diagnostic evaluation, and administration and outcomes of surgical and medical therapy are described separately. (See "Clinical manifestations and diagnosis of chronic thromboembolic pulmonary hypertension" and "Chronic thromboembolic pulmonary hypertension: Surgical treatment" and "Chronic thromboembolic pulmonary hypertension: Medical treatment".)


The first step in the management of CTEPH is the initiation of anticoagulant therapy. The purpose of anticoagulant therapy is to prevent both recurrent venous thromboembolism and in situ pulmonary artery thrombosis [2]. Anticoagulant therapy is initiated using intravenous unfractionated heparin or subcutaneous low molecular weight heparin. Once the patient is fully anticoagulated with one of these agents, they can be transitioned to an oral anticoagulant, the details of which are discussed separately. (See "Treatment of pulmonary hypertension in adults", section on 'Anticoagulation'.)

There are no studies comparing indefinite anticoagulant therapy to either no therapy or a shorter duration of therapy in patients with CTEPH. However, the usual approach to anticoagulant therapy in patients with CTEPH is based upon clinical experience, as well as the extrapolation of data from patients with a single or recurrent acute pulmonary embolism. In such patients, prolonged therapy is associated with fewer recurrent emboli than a shorter duration therapy according to randomized trials, and this effect appears to be larger than the increased risk of bleeding as described separately. (See "Venous thromboembolism: Initiation of anticoagulation (first 10 days)" and "Treatment, prognosis, and follow-up of acute pulmonary embolism in adults" and "Venous thromboembolism: Anticoagulation after initial management" and "Rationale and indications for indefinite anticoagulation in patients with venous thromboembolism".)


The next step after the initiation of anticoagulant therapy is the evaluation of all patients with CTEPH for pulmonary thromboendarterectomy. Surgery is the only definitive therapy for CTEPH and pulmonary thromboendarterectomy is the surgical procedure of choice. The evaluation should occur even if the symptoms, hemodynamic abnormalities, or ventilatory impairment seem mild because early surgery may prevent development of an irreversible vasculopathy [1]. The decision to proceed to pulmonary thromboendarterectomy is based upon four criteria: the surgical accessibility of the thrombi; the presence of hemodynamic and/or ventilatory impairment; the impact of the patient's comorbidities on the risks of the surgery; and the willingness and motivation of the patient to undergo surgery [1].

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Literature review current through: Nov 2017. | This topic last updated: Dec 08, 2017.
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