State-of-the-Art Paper
Chronic Thromboembolic Pulmonary Hypertension

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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.

Key Words

chronic thromboembolic pulmonary hypertension
pulmonary endarterectomy
ventilation/perfusion scan
pulmonary angiogram

Abbreviations and Acronyms

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

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Dr. Kim has received consultancy fees for work on steering committees for Actelion and Bayer; and research support from Actelion, Aires, Gilead Sciences, Lung LLC, and United Therapeutics. Dr. Delcroix has received fees for serving as investigator, speaker, consultant, or steering committee member from Actelion, Bayer, GlaxoSmithKline, Novartis, Pfizer, and United Therapeutics; educational grants from Actelion, GlaxoSmithKline, and Pfizer; research grants from Actelion, Pfizer, and GlaxoSmithKline; and she is also holder of the Actelion Chair for Pulmonary Hypertension and of the GlaxoSmithKline chair for research and education in pulmonary vascular pathology at the KU Leuven. Dr. Jenkins has received fees for lecturing and/or serving as a trial adjudicator and steering committee member from Actelion, Bayer, GlaxoSmithKline, and Pfizer. Dr. Channick has received research grants from and/or consulted for companies that have treatments for pulmonary hypertension, including Actelion Pharmaceuticals, Bayer, United Therapeutics, and Gilead. Dr. Jansa has received fees for serving as investigator, consultant, and speaker from Actelion, Bayer, United Therapeutics, AOP Orphan Pharmaceuticals, Pfizer, and GlaxoSmithKline. Dr. Lang has received fees for serving as investigator, speaker, consultant, or steering committee member from Actelion, Bayer, GlaxoSmithKline, Novartis, Pfizer, AOP Orphan Pharmaceuticals, and United Therapeutics; educational grants from Actelion; and research grants from Actelion, AOP Orphan Pharmaceuticals, Bayer, and United Therapeutics. Dr. Madani has served as consultant for Bayer and GlaxoSmithKline; and received fees as speaker for Bayer. Dr. Pengo is on the advisory board of Daiichi-Sankyo; and has received lecture fees from Bayer AG and Roche Diagnostics. Dr. Mayer received fees for serving as speaker, consultant, or steering or adjudication committee member from Actelion, Bayer, GlaxoSmithKline, Pfizer, and AOP Orphan Pharmaceuticals. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.