Chronic Thromboembolic Pulmonary Hypertension

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US Cardiology, 2006;3(2):1-5

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Chronic thromboembolic pulmonary hypertension (CTEPH) results from obstruction of the major pulmonary arteries by unresolved or organized pulmonary emboli that have become incorporated into the vessel wall, ultimately causing increased pulmonary vascular resistances.Without intervention, CTEPH is a progressive, fatal disease for which there is no proven effective medical therapy. Pulmonary endarterectomy (PE) is the treatment of choice. Lung transplantation is indicated only in few cases when PE is not feasible.

Pulmonary hypertension accounts for substantial morbidity and mortality. Except when lung or heart lung transplantation is considered, CTEPH is the only form of potentially surgically curable PH, making its recognition crucial. Chronic thromboembolic obstruction of the major pulmonary arteries occurs as a result of diagnosed or unrecognized acute pulmonary embolism (PEm) and may lead to PH and cor pulmonale. CTEPH should be considered in patients with unexplained dyspnea even when no definitive history of acute PEm is evident.1

Epidemiology and Pathophysiology

A recent prospective, long-term, follow-up study assessed the incidence of symptomatic CTEPH in consecutive patients after a single acute episode of PE.2 The cumulative incidence of symptomatic CTEPH was 1% at six months, 3.1% at one year, and 3.8% at two years,much higher than previously suspected.The most likely factor to increase the risk of CTEPH was previous PE (odds ratio (OR); 19.0), although younger age, a larger perfusion defect, and idiopathic PE at presentation also increased the risk. Acute PE is likely the initiating event even when there is no documented history of acute venous thromboembolism. Although serial angiographic studies are limited to small numbers of patients, incomplete resolution is visible in many patients as long as three weeks after an acute pulmonary embolic event.3 When serial perfusion scans have been performed, up to 66% of patients show persistence of abnormal perfusion on scans performed several months after the primary event4 although perfusion scanning often underestimates the extent of angiographic obstruction in chronic PE.5 It is unclear why some patients do not resolve the acute embolism and then develop PH. A thrombophilic disorder is found in only a minority of patients.8 A lupus anticoagulant is present in 10-20% of patients with CTEPH.6,7 Abnormal fibrinolysis or pulmonary endothelial pathology could be responsible for incomplete thrombus resolution, but this remains unclear.9,10

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