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

• DOI: 10.1007/s12471-015-0666-9
• Publication Date: 2015-03-03T12:59:35Z
• Authors: 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
• Keywords: Quality of life; Serial; Congenital heart defect; BOSENTAN; ASSOCIATION; Pulmonary arterial hypertension; CONTROLLED-TRIAL; DOWNS-SYNDROME; EXERCISE CAPACITY; 3. Good health; DOUBLE-BLIND; 03 medical and health sciences; Radboudumc 16: Vascular damage RIHS: Radboud Institute for Health Sciences; 0302 clinical medicine; FAILURE; ATRIAL SEPTAL-DEFECT; Original Article; EISENMENGER-SYNDROME; FIBRILLATION; Mortality; Cardiology and Cardiovascular Medicine

Decrease in quality of life (QoL) left-sided heart failure precedes poor survival, which can be reversed with exercise training. We investigated whether QoL is associated mortality pulmonary arterial hypertension due to congenital disease (PAH-CHD) patients.In this observational study, PAH-CHD adults referred for PAH-specific therapy were included. surveys (SF36) recorded during 2 years therapy. Based on shift SF36 scores period, patients had either decreased or non-decreased QoL. Subsequently, the followed mortality.Thirty-nine (mean age 42, 44 % male, 49 Down's syndrome) analysed. Following therapy, physical component summary (PCS) 13 (35-31 points, p = 0.001) and showed no decrease 26 (34-43 mean values, < 0.001). Post-initiation phase, median follow-up was 4.5 years, 12 deaths occurred (31 %), 10 (56 %) (10 group (p 0.002). Cox regression a PCS predicted (HR 3.4, 95 CI 1.03-11, 0.045).In patients, following initiation determinant mortality.