Decrease in quality of life predicts mortality in adult patients with pulmonary arterial hypertension due to congenital heart disease |
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Authors: | IM Blok ACMJ van Riel MJ Schuuring MG Duffels JC Vis APJ van Dijk ES Hoendermis BJM Mulder BJ Bouma |
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Institution: | .Department of Cardiology, Academic Medical Centre, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands ;.ICIN, Netherlands Heart Institute, Utrecht, The Netherlands ;.Department of Cardiology, Radboud University Medical Centre, Nijmegen, The Netherlands ;.Department of Cardiology, University Medical Centre Groningen, Groningen, The Netherlands |
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Abstract: | BackgroundDecrease 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.MethodsIn 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 decreased or non-decreased QoL. Subsequently, the patients were followed for mortality.ResultsThirty-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 < 0.001). Post-initiation phase, median follow-up was 4.5 years, during which 12 deaths occurred (31 %), 10 (56 %) in the decreased and 2 (10 %) in the non-decreased group (p = 0.002). Cox regression showed a decrease in SF36 PCS predicted mortality (HR 3.4, 95 % CI 1.03–11, p = 0.045).ConclusionsIn PAH-CHD patients, decrease in SF36 PCS following initiation of PAH-specific therapy is a determinant of mortality.Electronic supplementary materialThe online version of this article (doi:10.1007/s12471-015-0666-9) contains supplementary material, which is available to authorized users. |
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Keywords: | Congenital heart defect Pulmonary arterial hypertension Quality of life Serial Mortality |
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