Current and future management of chronic thromboembolic pulmonary hypertension: from diagnosis to treatment responses

Abstract

Although pulmonary endarterectomy (PEA) has been proven a very effective treatment for chronic thromboembolic pulmonary hypertension, it cannot be performed in a substantial proportion of patients. Here, we outline a proposed treatment algorithm, outlining therapeutic alternatives: (1) PEA should be considered as the first treatment option, where possible; (2) medical intervention is a possible option in inoperable patients and those with significant arteriopathy, although only chronic anticoagulation has been widely used to date (advanced medical treatment options could include prostanoids, endothelin receptor antagonists, or phosphodiesterase-5 inhibitors, but randomized clinical trials are required); (3) pulmonary hypertension is likely to persist after PEA in patients with significant small-vessel arteriopathy, resulting in poor clinical outcome and increased perioperative mortality (medical therapy could also be applied here); (4) anticoagulation therapy and, possibly, advanced medical treatment with careful monitoring may provide benefits in patients with mild or asymptomatic disease; (5) if medical therapy begins to fail, PEA should be offered without delay to avoid progression to severe, secondary arteriopathy; (6) in the absence of severe comorbidity, lung transplantation may be undertaken where PEA has failed, in nonresponders to medical therapy, and in patients with progressive arteriopathy; (7) in patients not eligible for PEA due to collateral and/or surgically inaccessible lesions, balloon angioplasty may be a possible alternative at some centers, but is experimental and requires further assessment. Continued research and clinical trials investigating possible applications of new medical treatments are required.