Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) occurs as a consequence of a series of events that includes arterial obstruction by embolic material, secondary in situ thrombosis, cytokine activation and inflammation, and small vessel angiopathy. Medical therapies have a limited efficacy. Only the guanylate cyclase stimulator, riociguat, is approved for this condition. Surgical pulmonary endarterectomy is the definitive treatment for patients with proximal disease, but one third of patients with CTEPH are considered ineligible for surgery. Another third have significant residual pulmonary hypertension postoperatively. Balloon pulmonary angioplasty is an option for these patients. The procedure has a low procedural mortality and high efficacy in experienced centres but has not yet been subjected to rigorous evaluation in clinical trials. Alternative options for percutaneous management include atrial septostomy and pulmonary artery denervation. Experience with these procedures is accumulating, but adequately powered, controlled trials have not yet been performed.

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