Treating the “Untreatable”. Recent advances in chronic thromboembolic pulmonary hypertension

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is thought to result from incomplete resolution of pulmonarterial lumina with collagenous obstructions. The treatment of choice is pulmonary endarterectomy (PEA) with low post-operative mortality and good long-term survival in expert hands. Patients ineligible for PEA or who have recurrent or persistent pulmonary hypertension after surgery have been previously the "Untreatable". Modern CTEPH man agement has now a solution for those previous no-option patients: balloon pulmonary angioplasty (BPA), and targeted medical therapies. Riociguat has been approved for inoperable CTEPH, and persistent-recurrent PH after PEA, and report that BPA improves haemodynamic, symptoms and functional capacity in patients with CTEPH, but controlled trials with long-term follow-up are needed. Complications include haemoptysis, wire injury, vessel dissection, vessel review summarises available evidence for PEA, BPA, and medical therapy, patient selection, peri-procedural imaging