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, 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

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.

Figures

Fig. 1
Fig. 1
Flowchart of patient inclusion
Fig. 2
Fig. 2
a Individual SF36 PCS regression lines of 39 PAH-CHD patients. b Decreased SF36 PCS regression lines, 13 patients. c Non-decreased SF36 PCS regression lines, 26 patients. All QoL measurements were recorded in the initiation phase after the start of PAH-specific standardised therapy. The thick lines represent the mean regression lines
Fig. 3
Fig. 3
Variability plot showing median, 25 and 75 % values of QoL scores during the initiation phase. a Variability of SF36 physical component summary scores. b Variability of SF36 mental component summary scores
Fig. 4
Fig. 4
Kaplan-Meier survival curve between PAH-CHD patients with decreased and non-decreased SF36 PCS, 2 years after initiation of PAH-specific therapy

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Source: PubMed

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