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  • Review Article
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Early detection of pulmonary arterial hypertension

Nature Reviews Cardiology volume 12, pages 143–155 (2015)Cite this article

Subjects

Key Points

  • Pulmonary arterial hypertension (PAH) remains a devastating disease without a cure, despite therapeutic innovations

  • Most patients are diagnosed at a very advanced stage of the disease

  • Specific populations of patients, such as those with systemic sclerosis and carriers of PAH-causing mutations (such as in the BMPR2 gene) are at high risk of developing PAH

  • Screening of high-risk populations for PAH is recommended by current guidelines and is an important strategy to improve clinical outcomes

  • Optimal screening algorithms for the early detection of PAH will continue to evolve with ongoing research

Abstract

Pulmonary arterial hypertension (PAH) remains an incurable disease associated with an unacceptably high early mortality, despite advances in therapeutic options. The disease is clinically silent until late in its natural history, when most of the distal pulmonary arteries have been obliterated. Early diagnosis of PAH is associated with improved long-term survival, and screening of at-risk populations is, therefore, a rational strategy to improve outcomes in this condition. Doppler echocardiography is the most widely used screening tool in current clinical practice. The role of evidence-based screening strategies has been clarified by research such as the DETECT study in patients with systemic sclerosis. A multimodal approach, using a range of noninvasive tests, improves the performance of screening algorithms. Right heart catheterization is mandatory to confirm a diagnosis of PAH. Uncertainties exist about the definition and prognostic relevance of pulmonary hypertension during exercise, but accumulating evidence suggests that stress testing of the pulmonary circulation can unmask clinically important early disease. Novel tools for the early detection of pulmonary vascular disease are urgently needed, given the substantial limitations of currently available techniques.

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Figure 1: The main components of how early detection of PAH might improve clinical outcomes.
Figure 2: Relationship between PH on exercise and resting PAH.

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Authors and Affiliations

  1. University of Sydney, Sydney Medical School, Missenden Road, Camperdown, 2050, NSW, Australia

    Edmund M. T. Lau & David S. Celermajer

  2. Université Paris-Sud, Hôpital Bicêtre, AP-HP, Inserm UMR-S999, 78 Rue du Général Leclerc, 94270, Le Kremlin-Bicêtre, France

    Marc Humbert

Authors
  1. Edmund M. T. Lau
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  2. Marc Humbert
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  3. David S. Celermajer
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Contributions

All the authors researched data for the article. E.M.T.L. wrote the manuscript, and all the authors reviewed and edited the article before submission.

Corresponding author

Correspondence to David S. Celermajer.

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Competing interests

E.M.T.L. has received support from Actelion for speaking engagements. M.H. has relationships with the following companies: Actelion, Aires, Bayer, BMS, GSK, Novartis, Pfizer, and United Therapeutics. In addition to being an investigator in trials involving these companies, relationships include consultancy services and membership of scientific advisory boards. D.S.C. has received support from Actelion, including serving on the speaker bureau and funding for research.

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Lau, E., Humbert, M. & Celermajer, D. Early detection of pulmonary arterial hypertension. Nat Rev Cardiol 12, 143–155 (2015). https://doi.org/10.1038/nrcardio.2014.191

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