Epidemiology and treatment of pulmonary arterial hypertension

doi:10.1038/nrcardio.2017.84

Review

. 2017 Oct;14(10):603-614.

doi: 10.1038/nrcardio.2017.84. Epub 2017 Jun 8.

Epidemiology and treatment of pulmonary arterial hypertension

Edmund M T Lau¹, Eleni Giannoulatou^{2

3}, David S Celermajer^{4

5}, Marc Humbert^{6

7

8}

Affiliations

¹ Department of Respiratory Medicine, Royal Prince Alfred Hospital, Missenden Road, Camperdown, NSW 2050, Australia.
² Computational Genomics Laboratory, Victor Chang Cardiac Research Institute, Liverpool Street, Darlinghurst, NSW 2010, Australia.
³ St Vincent's Clinical School, University of New South Wales, Victoria Street, Darlinghurst, NSW 2010, Australia.
⁴ Sydney Medical School, University of Sydney, Missenden Road, Camperdown, NSW 2050, Australia.
⁵ Department of Cardiology, Royal Prince Alfred Hospital, Missenden Road, Camperdown, NSW 2050, Australia.
⁶ AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, DHU Thorax Innovation, Hôpital Bicêtre, Rue du Général Leclerc, 94270 Le Kremlin-Bicêtre, France.
⁷ INSERM UMR_S 999, LabEx LERMIT, Centre Chirurgical Marie Lannelongue, Univ. Paris-Sud, Université Paris-Saclay, Avenue de la Résistance, 92350 Le Plessis-Robinson, France.
⁸ Faculté de Médecine, Univ. Paris-Sud, Université Paris-Saclay, Rue Gabriel Peri, 94270 Le Kremlin-Bicêtre, France.

PMID: 28593996
DOI: 10.1038/nrcardio.2017.84

Review

Epidemiology and treatment of pulmonary arterial hypertension

Edmund M T Lau et al. Nat Rev Cardiol. 2017 Oct.

. 2017 Oct;14(10):603-614.

doi: 10.1038/nrcardio.2017.84. Epub 2017 Jun 8.

Authors

Edmund M T Lau¹, Eleni Giannoulatou^{2

3}, David S Celermajer^{4

5}, Marc Humbert^{6

7

8}

Affiliations

¹ Department of Respiratory Medicine, Royal Prince Alfred Hospital, Missenden Road, Camperdown, NSW 2050, Australia.
² Computational Genomics Laboratory, Victor Chang Cardiac Research Institute, Liverpool Street, Darlinghurst, NSW 2010, Australia.
³ St Vincent's Clinical School, University of New South Wales, Victoria Street, Darlinghurst, NSW 2010, Australia.
⁴ Sydney Medical School, University of Sydney, Missenden Road, Camperdown, NSW 2050, Australia.
⁵ Department of Cardiology, Royal Prince Alfred Hospital, Missenden Road, Camperdown, NSW 2050, Australia.
⁶ AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, DHU Thorax Innovation, Hôpital Bicêtre, Rue du Général Leclerc, 94270 Le Kremlin-Bicêtre, France.
⁷ INSERM UMR_S 999, LabEx LERMIT, Centre Chirurgical Marie Lannelongue, Univ. Paris-Sud, Université Paris-Saclay, Avenue de la Résistance, 92350 Le Plessis-Robinson, France.
⁸ Faculté de Médecine, Univ. Paris-Sud, Université Paris-Saclay, Rue Gabriel Peri, 94270 Le Kremlin-Bicêtre, France.

PMID: 28593996
DOI: 10.1038/nrcardio.2017.84

Abstract

In the past 2 decades, major changes have occurred in the epidemiological and treatment landscape of pulmonary arterial hypertension (PAH). Previously regarded as a disease of the young and middle-aged, contemporary registries from the Western world have demonstrated an increase in the age of patients with PAH, many of whom are elderly with multiple comorbidities. Another important observation is the improvement in survival of patients with PAH in the modern treatment era compared with historical cohorts, before the availability of advanced therapy. The management of PAH has also become more complex, and numerous drugs are now approved that target the endothelin 1, nitric oxide, and prostacyclin pathways. Combining drugs from different classes is now considered the standard of care and has been demonstrated to improve outcomes. Furthermore, the current treatment paradigm is the early use of combination therapy, often at the time of diagnosis, particularly in patients with severe disease. This Review provides a comprehensive update on the epidemiology and pharmacotherapy of PAH.

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Comment in

Hypertension: Promising mitochondria-targeting drug for PAH.
Fernández-Ruiz I. Fernández-Ruiz I. Nat Rev Cardiol. 2018 Jan;15(1):4-5. doi: 10.1038/nrcardio.2017.188. Epub 2017 Nov 16. Nat Rev Cardiol. 2018. PMID: 29143809 No abstract available.

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[1] Chest. 2013 Sep;144(3):952-958 - PubMed

[2] Chest. 2013 Sep;144(3):952-958 - PubMed

[3] Eur Respir J. 2007 Dec;30(6):1103-10 - PubMed

[4] Eur Respir J. 2007 Dec;30(6):1103-10 - PubMed

[5] Ann Rheum Dis. 2017 Jul;76(7):1219-1227 - PubMed

[6] Ann Rheum Dis. 2017 Jul;76(7):1219-1227 - PubMed

[7] Ann Intern Med. 1991 Sep 1;115(5):343-9 - PubMed

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[9] Eur Respir J. 2014 Jun;43(6):1691-7 - PubMed

[10] Eur Respir J. 2014 Jun;43(6):1691-7 - PubMed

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Epidemiology and treatment of pulmonary arterial hypertension

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Epidemiology and treatment of pulmonary arterial hypertension

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