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Canadian Respiratory Journal
Volume 17, Issue 6, Pages 301-334
Special Article

Diagnostic Evaluation and Management of Chronic Thromboembolic Pulmonary Hypertension: A Clinical Practice Guideline

Sanjay Mehta,1 Doug Helmersen,2 Steeve Provencher,3 Naushad Hirani,2 Fraser D Rubens,4 Marc De Perrot,5 Mark Blostein,6 Kim Boutet,7 George Chandy,8 Carole Dennie,9 John Granton,10 Paul Hernandez,11 Andrew M Hirsch,12 Karen Laframboise,13 Robert D Levy,14 Dale Lien,15 Simon Martel,3 Gerard Shoemaker,1 John Swiston,14 and Justin Weinkauf15

1Southwest Ontario Pulmonary Hypertension Clinic, London Health Sciences Centre, Schulich School of Medicine & Dentistry, University of Western Ontario, London, Ontario, Canada
2University of Calgary Pulmonary Hypertension Clinic, Division of Respiratory Medicine, Peter Lougheed Hospital, Calgary, Alberta, Canada
3Pulmonary Hypertension Program, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Université Laval, Québec, Québec, Canada
4University of Ottawa Heart Institute, Division of Cardiac Surgery, University of Ottawa, Ottawa, Canada
5Toronto Pulmonary Endarterectomy Program, University of Toronto, Division of Thoracic Surgery, Toronto General Hospital, Toronto, Ontario, Canada
6Division of Hematology, Department of Medicine, Sir Mortimer B Davis Jewish General Hospital, McGill University, Montréal, Canada
7Centre for Pulmonary Vascular Disease, Sir Mortimer B Davis Jewish General Hospital, McGill University, and Sacré-Coeur Hospital Respiratory Division, Centre Hospitalier Universitaire de Montreal, Montréal, Québec, Canada
8University of Ottawa Heart Institute Pulmonary Hypertension Clinic, Respirology Division, The Ottawa Hospital, University of Ottawa, Ottawa, Canada
9Thoracic and Cardiac Imaging Sections, The Ottawa Hospital, Cardiac Radiology, University of Ottawa Heart Institute, University of Ottawa, Ottawa, Canada
10University Health Network Pulmonary Hypertension Program, Faculty of Medicine, University of Toronto, Division of Respirology, Toronto, Ontario, Canada
11Pulmonary Hypertension Clinic, Respirology Division, Department of Medicine, QEII Health Sciences Centre, Dalhousie University, Halifax, Nova Scotia, Canada
12Center for Pulmonary Vascular Disease, Sir Mortimer B Davis Jewish General Hospital, McGill University, Montréal, Québec, Canada
13Division of Respirology, Critical Care and Sleep Medicine, University of Saskatchewan, Saskatoon, Saskatchewan, Canada
14Pulmonary Hypertension Program, Respirology Division, Vancouver General Hospital, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada
15University of Alberta Pulmonary Hypertension Clinic, University of Alberta, Edmonton, Alberta, Canada

Copyright © 2010 Canadian Thoracic Society. This open-access article is distributed under the terms of the Creative Commons Attribution Non-Commercial License (CC BY-NC) (, which permits reuse, distribution and reproduction of the article, provided that the original work is properly cited and the reuse is restricted to noncommercial purposes.


BACKGROUND: Pulmonary embolism is a common condition. Some patients subsequently develop chronic thromboembolic pulmonary hypertension (CTEPH). Many care gaps exist in the diagnosis and management of CTEPH patients including lack of awareness, incomplete diagnostic assessment, and inconsistent use of surgical and medical therapies.

METHODS: A representative interdisciplinary panel of medical experts undertook a formal clinical practice guideline development process. A total of 20 key clinical issues were defined according to the patient population, intervention, comparator, outcome (PICO) approach. The panel performed an evidence-based, systematic, literature review, assessed and graded the relevant evidence, and made 26 recommendations.

RESULTS: Asymptomatic patients postpulmonary embolism should not be screened for CTEPH. In patients with pulmonary hypertension, the possibility of CTEPH should be routinely evaluated with initial ventilation/ perfusion lung scanning, not computed tomography angiography. Pulmonary endarterectomy surgery is the treatment of choice in patients with surgically accessible CTEPH, and may also be effective in CTEPH patients with disease in more ‘distal’ pulmonary arteries. The anatomical extent of CTEPH for surgical pulmonary endarterectomy is best assessed by contrast pulmonary angiography, although positive computed tomography angiography may be acceptable. Novel medications indicated for the treatment of pulmonary hypertension may be effective for selected CTEPH patients.

CONCLUSIONS: The present guideline requires formal dissemination to relevant target user groups, the development of tools for implementation into routine clinical practice and formal evaluation of the impact of the guideline on the quality of care of CTEPH patients. Moreover, the guideline will be updated periodically to reflect new evidence or clinical approaches.