Pulmonary hypertension: diagnosis and management

BMJ 2013; 346 doi: http://dx.doi.org/10.1136/bmj.f2028 (Published 16 April 2013)
Cite this as: BMJ 2013;346:f2028

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  1. David G Kiely, consultant respiratory physician1,
  2. Charlie A Elliot, consultant respiratory physician1,
  3. Ian Sabroe, consultant respiratory physician12,
  4. Robin Condliffe, consultant respiratory physician 1
  1. 1National Pulmonary Hypertension Service (Sheffield), Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S10 2JF, UK
  2. 2Academic Unit of Respiratory Medicine, Department of Infection and Immunity, Faculty of Medicine, Dentistry and Health, Medical School, University of Sheffield, Sheffield, UK
  1. Correspondence to: D G Kiely david.kiely{at}sth.nhs.uk

Summary points

  • Pulmonary hypertension has many causes so prognoses and treatments vary

  • The condition is diagnosed by systematically evaluating the breathless patient and screening patients at high risk

  • Patients at high risk of severe and treatable pulmonary hypertension include those with systemic sclerosis, portal hypertension, congenital heart disease, and previous pulmonary embolism

  • Specialist centres provide access to tailored investigative and treatment pathways and support networks for patients

  • Patients with severe pulmonary hypertension can deteriorate rapidly—do not delay referral to perform specialist investigations

  • Only selected patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension benefit from interventions directed at pulmonary vasculature; for most patients treatment is aimed at the underlying condition

Pulmonary hypertension was previously considered a rare untreatable condition. The past two decades have seen major changes in our understanding of the spectrum of disease affecting the pulmonary circulation. More than 30 randomised controlled trials (RCTs) of treatments have been performed, and surgery for patients with chronic thromboembolic pulmonary hypertension has been developed. Advances in imaging have enabled more detailed patient assessment, but pulmonary hypertension continues to be a life shortening condition, and there is often a delay of around two years from onset of symptoms to diagnosis.1 This article focuses on adult pulmonary hypertension in primary and secondary care, its diagnosis, and management.

Sources and selection criteria

This review is based on our personal experience, personal archives of references, and a PubMed search using terms including pulmonary hypertension, chronic thromboembolic disease, and prognosis. We consulted international guidelines from the European Society of Cardiology, European Respiratory Society, and American College of Chest Physicians.

What is pulmonary hypertension and how common is it?

Pulmonary hypertension is defined at cardiac catheterisation as a mean pulmonary artery pressure of 25 mm Hg or more. The initial clinical classification in 1973 arose from a World Health Organization sponsored international meeting after an epidemic related to use of …

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