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Review
. 2020 Nov;36(6):583-587.
doi: 10.6515/ACS.202011_36(6).20200507A.

Management of Pulmonary Arterial Hypertension Patients with World Health Organization Functional Class II

Cheng-Chung Hung  1 , Chin-Chang Cheng  1   2   3 , Wei-Chun Huang  1   2   3   4 , Kun-Chang Lin  1 , Feng-Yu Kuo  1 , Shu-Hung Kuo  1 , Tse-Hsuan Yang  1 , Hsin-Li Liang  1 , Guang-Yuan Mar  1
Affiliations
Review

Management of Pulmonary Arterial Hypertension Patients with World Health Organization Functional Class II

Cheng-Chung Hung et al. Acta Cardiol Sin. 2020 Nov.

Abstract

Pulmonary arterial hypertension (PAH) is an incurable chronic and progressive debilitating disease associated with significant morbidity and mortality. The World Health Organization functional class (WHO FC) at diagnosis and at follow-up remains one of the strongest predictors of survival in PAH. Studies have shown improved long-term outcomes in PAH patients who received PAH-specific treatment, as monotherapy or as combination therapy, early in their disease course. Studies have also shown that without treatment, PAH rapidly deteriorates even in patients with less advanced (low risk) disease state. In this article, we review evidence from randomized controlled clinical trials to support our position on the importance of early PAH management in WHO FC II patients. The growing importance of combination therapy in the early treatment of PAH and recommendations by the most recent guidelines for the diagnosis and treatment of pulmonary hypertension are also discussed in this article.

Keywords: Early treatment; Pulmonary artery hypertension; World Health Organization Functional Class.

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References

    1. Hsu CH, Ho WJ, Huang WC, et al. 2014 Guidelines of Taiwan Society of Cardiology (TSOC) for the management of pulmonary arterial hypertension. Acta Cardiol Sin. 2014;30:401–444. - PMC - PubMed
    1. Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37:67–119. - PubMed
    1. Daccord C, Letovanec I, Yerly P, et al. First histopathological evidence of irreversible pulmonary vascular disease in dasatinib-induced pulmonary arterial hypertension. Eur Respir J. 2018;51:1701694. - PubMed
    1. Drozd K, Ahmadi A, Deng Y, et al. Effects of an endothelin receptor antagonist, macitentan, on right ventricular substrate utilization and function in a Sugen 5416/hypoxia rat model of severe pulmonary arterial hypertension. J Nucl Cardiol. 2017;24:1979–1989. - PubMed
    1. Huang WC, Ke MW, Cheng CC, et al. Therapeutic benefits of induced pluripotent stem cells in monocrotaline-induced pulmonary arterial hypertension. PLoS One. 2016;11:e0142476. - PMC - PubMed

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