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.
authors.affiliation: Department of Cardiology, Academic Medical Centre, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands.
authors.forename: I M
authors.initials: IM
authors.lastname: Blok
authors.forename: A C M J
authors.initials: AC
authors.lastname: van Riel
authors.forename: M J
authors.initials: MJ
authors.lastname: Schuuring
authors.forename: M G
authors.initials: MG
authors.lastname: Duffels
authors.forename: J C
authors.initials: JC
authors.lastname: Vis
authors.forename: A P J
authors.initials: AP
authors.lastname: van Dijk
authors.forename: E S
authors.initials: ES
authors.lastname: Hoendermis
authors.forename: B J M
authors.initials: BJ
authors.lastname: Mulder
authors.forename: B J
authors.initials: BJ
authors.lastname: Bouma