Chronic thromboembolic pulmonary hypertension: realising the potential of multimodal management.

Journal: The Lancet. Respiratory medicine
Published Date:

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism. Important advances have enabled better understanding, characterisation, and treatment of this condition. Guidelines recommending systematic follow-up after acute pulmonary embolism, and the insight that CTEPH can mimic acute pulmonary embolism on initial presentation, have led to the definition of CTEPH imaging characteristics, the introduction of artificial intelligence diagnosis pathways, and thus the prospect of easier and earlier CTEPH diagnosis. In this Series paper, we show how the understanding of CTEPH as a sequela of inflammatory thrombosis has driven successful multidisciplinary management that integrates surgical, interventional, and medical treatments. We provide imaging examples of classical major vessel targets, describe microvascular targets, define available tools, and depict an algorithm facilitating the initial treatment strategy in people with newly diagnosed CTEPH based on a multidisciplinary team discussion at a CTEPH centre. Further work is needed to optimise the use and combination of multimodal therapeutic options in CTEPH to improve long-term outcomes for patients.

Authors

  • Marion Delcroix
    Clinical Department of Respiratory Diseases, University Hospitals of Leuven and Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), Department of Chronic Diseases and Metabolism (CHROMETA), KU Leuven-University of Leuven, Leuven, Belgium. Electronic address: marion.delcroix@uzleuven.be.
  • Marc de Perrot
    Division of Thoracic Surgery, Toronto General Hospital, Toronto, ON, Canada.
  • Xavier Jaïs
    Assistance Publique-Hôpitaux de Paris (AP-HP), Service de Pneumologie, Hôpital Bicêtre, Université Paris-Saclay, Le Kremlin-Bicêtre, France.
  • David P Jenkins
    Department of Cardiothoracic Surgery, Royal Papworth Hospital, Cambridge, UK.
  • Irene M Lang
    Division of Cardiology, Department of Internal Medicine II, Vienna General Hospital, Centre for CardioVascular Medicine, Medical University of Vienna, Vienna, Austria.
  • Hiromi Matsubara
    National Hospital Organization Okayama Medical Center, Okayama, Japan.
  • Lilian J Meijboom
    Department of Radiology and Nuclear Medicine, Amsterdam Cardiovascular Sciences, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands.
  • Rozenn Quarck
    Clinical Department of Respiratory Diseases, University Hospitals of Leuven and Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), Department of Chronic Diseases and Metabolism (CHROMETA), KU Leuven-University of Leuven, Leuven, Belgium.
  • Gérald Simonneau
    Assistance Publique-Hôpitaux de Paris (AP-HP), Service de Pneumologie, Hôpital Bicêtre, Université Paris-Saclay, Le Kremlin-Bicêtre, France.
  • Christoph B Wiedenroth
    Department of Thoracic Surgery, Kerckhoff Clinic, 61231 Bad Nauheim, Germany.
  • Nick H Kim
    Division of Pulmonary, Critical Care and Sleep Medicine, University of California San Diego, La Jolla, CA, USA.