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

doi:10.1007/s12471-015-0666-9

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

Research output: Contribution to journal › Article › Academic › peer-review

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 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 < 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).

CONCLUSIONS: In PAH-CHD patients, decrease in SF36 PCS following initiation of PAH-specific therapy is a determinant of mortality.

Original language	English
Pages (from-to)	278-84
Number of pages	7
Journal	Netherlands Heart Journal
Volume	23
Issue number	5
DOIs	https://doi.org/10.1007/s12471-015-0666-9
Publication status	Published - May 2015
Externally published	Yes

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Blok, I. M., van Riel, A. C. M. J., Schuuring, M. J., Duffels, M. G., Vis, J. C., van Dijk, A. P. J., Hoendermis, E. S., Mulder, B. J. M., & Bouma, B. J. (2015). Decrease in quality of life predicts mortality in adult patients with pulmonary arterial hypertension due to congenital heart disease. Netherlands Heart Journal, 23(5), 278-84. https://doi.org/10.1007/s12471-015-0666-9

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title = "Decrease in quality of life predicts mortality in adult patients with pulmonary arterial hypertension due to congenital heart disease",

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 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 < 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).CONCLUSIONS: In PAH-CHD patients, decrease in SF36 PCS following initiation of PAH-specific therapy is a determinant of mortality.",

author = "Blok, {I M} and {van Riel}, {A C M J} and Schuuring, {M J} and Duffels, {M G} and Vis, {J C} and {van Dijk}, {A P J} and Hoendermis, {E S} and Mulder, {B J M} and Bouma, {B J}",

year = "2015",

month = may,

doi = "10.1007/s12471-015-0666-9",

language = "English",

volume = "23",

pages = "278--84",

journal = "Netherlands Heart Journal",

issn = "1568-5888",

publisher = "Bohn Stafleu van Loghum",

number = "5",

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TY - JOUR

T1 - Decrease in quality of life predicts mortality in adult patients with pulmonary arterial hypertension due to congenital heart disease

AU - Blok, I M

AU - van Riel, A C M J

AU - Schuuring, M J

AU - Duffels, M G

AU - Vis, J C

AU - van Dijk, A P J

AU - Hoendermis, E S

AU - Mulder, B J M

AU - Bouma, B J

PY - 2015/5

Y1 - 2015/5

N2 - 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 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 < 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).CONCLUSIONS: In PAH-CHD patients, decrease in SF36 PCS following initiation of PAH-specific therapy is a determinant of mortality.

AB - 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 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 < 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).CONCLUSIONS: In PAH-CHD patients, decrease in SF36 PCS following initiation of PAH-specific therapy is a determinant of mortality.

U2 - 10.1007/s12471-015-0666-9

DO - 10.1007/s12471-015-0666-9

M3 - Article

C2 - 25911012

SN - 1568-5888

VL - 23

SP - 278

EP - 284

JO - Netherlands Heart Journal

JF - Netherlands Heart Journal

IS - 5

ER -

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

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