Neth Heart J (2015) 23:278–284 DOI 10.1007/s12471-015-0666-9

O r i g i n a l a rt i c l e

Decrease in quality of life predicts mortality in adult patients with pulmonary arterial hypertension due to congenital heart disease I.M. Blok · A.C.M.J. van Riel · M.J. Schuuring · M.G. Duffels · J.C. Vis · A.P.J. van Dijk · E.S. Hoendermis · B.J.M. Mulder · B.J. Bouma

Published online: 4 March 2015 © The Author(s) 2015. This article is published with open access at Springerlink.com

Abstract Background  Decrease in quality of life (QoL) in left-sided heart failure precedes poor survival, which can be reversed with exercise training. We investigated whether QoL is associated with mortality in pulmonary arterial hypertension due to congenital heart disease (PAH-CHD) patients. Methods  In this observational study, PAH-CHD adults referred for PAH-specific therapy were included. QoL surveys (SF36) were recorded during 2 years of therapy. Based on shift in SF36 scores during this period, patients had either

Electronic supplementary material The online version of this article (doi:10.1007/s12471-015-0666-9) contains supplementary material, which is available to authorized users. I.M. Blok, A.C.M.J. van Riel, M. J. Schuuring, M.G. Duffels, J.C. Vis, A.P.J. van Dijk, E.S. Hoendermis, B.J.M. Mulder, B.J. Bouma, the authors take responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation. B.J. Bouma () · I.M. Blok · A.C.M.J. van Riel · M.G. Duffels · J.C. Vis · B.J.M. Mulder Department of Cardiology, Academic Medical Centre, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands e-mail: [email protected] I.M. Blok · A.C.M.J. van Riel · M.J. Schuuring · B.J.M. Mulder ICIN, Netherlands Heart Institute, Utrecht, The Netherlands A.P.J. van Dijk Department of Cardiology, Radboud University Medical Centre, Nijmegen, The Netherlands E.S. Hoendermis Department of Cardiology, University Medical Centre Groningen, Groningen, The Netherlands

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decreased or non-decreased QoL. Subsequently, the patients were followed for mortality. Results  Thirty-nine PAH-CHD patients (mean age 42, 44 % male, 49 % Down’s syndrome) were analysed. Following PAH-specific therapy, SF36 physical component summary (PCS) decreased in 13 (35–31 points, p = 0.001) and showed no decrease in 26 patients (34–43 points, mean values, p 

Decrease in quality of life predicts mortality in adult patients with pulmonary arterial hypertension due to congenital heart disease.

Decrease in quality of life (QoL) in left-sided heart failure precedes poor survival, which can be reversed with exercise training. We investigated wh...
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