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Review
. 2019 Jan 24;53(1):1801915.
doi: 10.1183/13993003.01915-2018. Print 2019 Jan.

Chronic thromboembolic pulmonary hypertension

Nick H Kim  1 , Marion Delcroix  2 , Xavier Jais  3 , Michael M Madani  4 , Hiromi Matsubara  5 , Eckhard Mayer  6 , Takeshi Ogo  7 , Victor F Tapson  8 , Hossein-Ardeschir Ghofrani  6   9   10   11 , David P Jenkins  12   11
Affiliations
Review

Chronic thromboembolic pulmonary hypertension

Nick H Kim et al. Eur Respir J. .

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of pulmonary embolism and a major cause of chronic PH leading to right heart failure and death. Lung ventilation/perfusion scintigraphy is the screening test of choice; a normal scan rules out CTEPH. In the case of an abnormal perfusion scan, a high-quality pulmonary angiogram is necessary to confirm and define the pulmonary vascular involvement and prior to making a treatment decision. PH is confirmed with right heart catheterisation, which is also necessary for treatment determination. In addition to chronic anticoagulation therapy, each patient with CTEPH should receive treatment assessment starting with evaluation for pulmonary endarterectomy, which is the guideline recommended treatment. For technically inoperable cases, PH-targeted medical therapy is recommended (currently riociguat based on the CHEST studies), and balloon pulmonary angioplasty should be considered at a centre experienced with this challenging but potentially effective and complementary intervention.

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Conflict of interest statement

Conflict of interest: N.H. Kim reports personal fees for consultancy, steering committee work and speaker bureau membership from Actelion and Bayer; personal fees for consultancy from Merck; and is a board member of the International CTEPH Association, CTEPH.com. Conflict of interest: M. Delcroix is an investigator, speaker, consultant or steering committee member for Actelion, Bayer AG, Bellerophon, Eli Lilly, GSK, MSD, Pfizer and Reata; and has received an institutional research grant from Actelion. Conflict of interest: X. Jais received grants and personal fees from Actelion, GSK, Bayer and MSD. Conflict of interest: M.M. Madani has received consultancy fees from MSD/Bayer, Wexler Surgical and Actelion, and is an executive board member of the International CTEPH Association, CTEPH.com. Conflict of interest: H. Matsubara has received lecture fees from Actelion Pharmaceuticals Japan, Ltd, AOP orphan Pharmaceuticals AG, Bayer Yakuhin, Ltd, Pfizer Japan, Inc, Nippon Shinyaku, Co, Ltd and Kaneka Medix Corporation, outside the submitted work. Conflict of interest: E. Mayer has received consultancy fees from Actelion, Bayer and MSD; and is on the speaker bureau for Actelion, Bayer, MSD and Pfizer. Conflict of interest: T. Ogo is a member of the speaker bureau for Actelion and Bayer. Conflict of interest: V.F. Tapson received research support from Actelion, Arena, Bayer, BiO2, Edwards Scientific, Ekos/BTG, Janssen, Inari, Portola, Reata, United Therapeutics, Daiichi and Penumbra; consultancy fees from Actelion, Bayer, BiO2, Ekos/BTG, Inari, Janssen, Portola, United Therapeutics, VWave, Daiichi and Penumbra; speaker bureau for Actelion, Bayer and Janssen. Conflict of interest: H-A. Ghofrani reports personal fees for advisory board work payment for lectures including service on speaker bureaus from Actelion, Bayer, GSK, Novartis and Pfizer; consultancy fees from Actelion, Bayer, Bellerophon Pulse Technologies, GSK, MSD, Novartis and Pfizer; and grants from Deutsche Forschungsgemeinschaft (DFG), outside the submitted work. Conflict of interest: D.P. Jenkins worked as adjudicator for Actelion and Bayer for MERIT and CHEST-1 studies; received honoraria for speaking from Actelion and Bayer; and is a board member of the International CTEPH Association, CTEPH.com.

Figures

FIGURE 1
FIGURE 1
Chronic thromboembolic pulmonary hypertension (CTEPH): revised treatment algorithm. BPA: balloon pulmonary angioplasty. #: multidisciplinary: pulmonary endarterectomy surgeon, PH expert, BPA interventionist and radiologist; : treatment assessment may differ depending on the level of expertise; +: BPA without medical therapy can be considered in selected cases.
See this image and copyright information in PMC

Comment in

  • doi: 10.1183/13993003.02148-2018

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