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Pulmonary hypertension

Nature Reviews Disease Primers volume 10, Article number: 1 (2024) Cite this article

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A Publisher Correction to this article was published on 17 January 2024

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Abstract

Pulmonary hypertension encompasses a range of conditions directly or indirectly leading to elevated pressures within the pulmonary arteries. Five main groups of pulmonary hypertension are recognized, all defined by a mean pulmonary artery pressure of >20 mmHg: pulmonary arterial hypertension (rare), pulmonary hypertension associated with left-sided heart disease (very common), pulmonary hypertension associated with lung disease (common), pulmonary hypertension associated with pulmonary artery obstructions, usually related to thromboembolic disease (rare), and pulmonary hypertension with unclear and/or multifactorial mechanisms (rare). At least 1% of the world’s population is affected, with a greater burden more likely in low-income and middle-income countries. Across all its forms, pulmonary hypertension is associated with adverse vascular remodelling with obstruction, stiffening and vasoconstriction of the pulmonary vasculature. Without proactive management this leads to hypertrophy and ultimately failure of the right ventricle, the main cause of death. In older individuals, dyspnoea is the most common symptom. Stepwise investigation precedes definitive diagnosis with right heart catheterization. Medical and surgical treatments are approved for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. There are emerging treatments for other forms of pulmonary hypertension; but current therapy primarily targets the underlying cause. There are still major gaps in basic, clinical and translational knowledge; thus, further research, with a focus on vulnerable populations, is needed to better characterize, detect and effectively treat all forms of pulmonary hypertension.

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Fig. 1: Diverse profile of pulmonary hypertension and pulmonary arterial hypertension across regions of the world.
Fig. 2: Molecular mechanisms in pulmonary arterial hypertension.
Fig. 3: Current pulmonary arterial hypertension therapies.

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Acknowledgements

The authors acknowledge the assistance and intellectual input of N. Breault and I. Emon in drawing Fig. 2.

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Authors and Affiliations

  1. Faculdade de Medicina, Universidade Eduardo Mondlane, Maputo, Moçambique

    Ana Mocumbi

  2. Instituto Nacional de Saúde, EN 1, Marracuene, Moçambique

    Ana Mocumbi

  3. Service de Pneumologie et Soins Intensifs Respiratoires, Hôpital Bicêtre (Assistance Publique Hôpitaux de Paris), Université Paris–Saclay, INSERM UMR_S 999, Paris, France

    Marc Humbert

  4. ERN-LUNG, Le Kremlin Bicêtre, Paris, France

    Marc Humbert

  5. Sharma University of Health Sciences, Haryana, New Delhi, India

    Anita Saxena

  6. Department of Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, China

    Zhi-Cheng Jing

  7. Cape Heart Institute, Faculty of Health Science, University of Cape Town, Cape Town, South Africa

    Karen Sliwa

  8. Department of Medicine, Groote Schuur Hospital, Faculty of Health Science, University of Cape Town, Cape Town, South Africa

    Friedrich Thienemann

  9. Department of Internal Medicine, University Hospital Zurich, University of Zurich, Zurich, Switzerland

    Friedrich Thienemann

  10. Department of Medicine, Queen’s University, Kingston, Ontario, Canada

    Stephen L. Archer

  11. Institute of Health Research, University of Notre Dame, Fremantle, Western Australia, Australia

    Simon Stewart

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Contributions

Conceptualization and writing of the first draft (A.M.); Introduction (A.M. and S.S.); Epidemiology (A.S., S.S., F.T. and A.M.); Mechanisms and pathophysiology (S.L.A. and Z.-C.J.); Diagnosis, screening and prevention (S.S., A.S., K.S. and A.M.); Management (M.H.); Quality of life (S.S.); Outlook (A.M., S.S. and M.H.). All authors reviewed the last version of the paper.

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Correspondence to Ana Mocumbi.

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Competing interests

M.H. declares institutional, speaker, consultant or steering committee fees from Aerovate, Altavant, AOP Orphan, Bayer, Ferrer, Janssen (Actelion), MSD (Acceleron) and United Therapeutics. The other authors declare no competing interests.

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Nature Reviews Disease Primers thanks S. Abman, J.-L. Vachiery, C. Vizza and the other, anonymous, reviewer(s) for their contribution to the peer review of this work.

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Mocumbi, A., Humbert, M., Saxena, A. et al. Pulmonary hypertension. Nat Rev Dis Primers 10, 1 (2024). https://doi.org/10.1038/s41572-023-00486-7

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