Chronic thromboembolic pulmonary hypertension

Abstract

Since the last World Symposium on Pulmonary Hypertension in 2008, we have witnessed numerous and exciting developments in chronic thromboembolic pulmonary hypertension (CTEPH). Emerging clinical data and advances in technology have led to reinforcing and updated guidance on diagnostic approaches to pulmonary hypertension, guidelines that we hope will lead to better recognition and more timely diagnosis of CTEPH. We have new data on treatment practices across international boundaries as well as long-term outcomes for CTEPH patients treated with or without pulmonary endarterectomy. Furthermore, we have expanded data on alternative treatment options for select CTEPH patients, including data from multiple clinical trials of medical therapy, including 1 recent pivotal trial, and compelling case series of percutaneous pulmonary angioplasty. Lastly, we have garnered more experience, and on a larger international scale, with pulmonary endarterectomy, which is the treatment of choice for operable CTEPH. This report overviews and highlights these important interval developments as deliberated among our task force of CTEPH experts and presented at the 2013 World Symposium on Pulmonary Hypertension in Nice, France.

Keywords: ACP; CT; CTEPH; CTPA; DHCA; ECMO; MRI; PAH; PAP; PEA; PH; PTPA; PVR; RCTs; VQ; antegrade cerebral perfusion; chronic thromboembolic pulmonary hypertension; computed tomography; computed tomography pulmonary angiogram; deep hypothermic circulatory arrest; extracorporeal membrane oxygenation; magnetic resonance imaging; percutaneous transluminal pulmonary angioplasty; pulmonary angiogram; pulmonary arterial hypertension; pulmonary artery pressure; pulmonary endarterectomy; pulmonary hypertension; pulmonary vascular resistance; randomized controlled trials; ventilation/perfusion; ventilation/perfusion scan.