Treating the “Untreatable”. Recent advances in chronic thromboembolic pulmonary hypertension
Inbal Shafran1 and Irene Lang2
1The Pulmonary institute, Chaim Sheba Medical Center, Tel-Hashomer, Israel and the
2Department of Internal Medicine II, Division of Cardiology, Medical University of Vienna, Vienna, Austria
Correspondence: Irene Lang, Medical University of Vienna, Internal Medicine II, Division of Cardiology, Währinger Gürtel 18–20, 1090 Vienna, Austria
Main treatment for CTEPH is Pulmonary Endarterectomy, a complex surgical procedure, that is performed in roughly half of all CTEPH patients. Refinements of Medical Treatments and Balloon Pulmonary Angioplasty have emerged as effective treatment strategies for the remaining patients who had no options in the past.
Chronic thromboembolic pulmonary hypertension (CTEPH) is thought to result from incomplete resolution of pulmonary thromboemboli that undergo organisation into fibrous tissue within pulmonary arterial branches, filling pulmonary arterial 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 management 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 SC Treprostinil has been efficacious in a randomized controlled trial of very severe CTEPH. Observational studies 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 rupture, reperfusion pulmonary oedema, pulmonary parenchymal bleeding and haemorrhagic pleural effusions. This review summarises available evidence for PEA, BPA, and medical therapy, patient selection, peri-procedural imaging and technical refinements achieved by CTEPH teams, and discusses future management of CTEPH.