TY - JOUR
T1 - Health-related quality of life and survival in pulmonary arterial hypertension
AU - Mathai, Stephen C.
AU - Suber, Tomeka
AU - Khair, Rubina M.
AU - Kolb, Todd M.
AU - Damico, Rachel L.
AU - Hassoun, Paul M.
N1 - Publisher Copyright:
© Copyright 2016 by the American Thoracic Society.
PY - 2016/1
Y1 - 2016/1
N2 - Rationale: Pulmonary arterial hypertension is a progressive disease with high morbidity and mortality despite advances in medical therapy. The relationship between patient-related outcomes, such as healthrelated quality of life (HRQOL), and survival is not well described. Objective: To assess the relationship between HRQOL and outcomes in patients with pulmonary arterial hypertension. Methods:Consecutivepatientswith right heart catheterization-proven pulmonary arterial hypertension who completed the Medical Outcomes Survey Short Form-36 survey (SF-36) were included. Demographic, clinical, physiological, and hemodynamic data were collected at baseline. Survival was assessed from the time of diagnosis of pulmonary arterial hypertension. Cox proportional hazard models were constructed to assess the relationship between HRQOL and transplant-free survival. Measurements and Main Results: Eighty-seven patients with pulmonary arterial hypertension were enrolled and followed prospectively for a median of 3.8 years. At baseline, HRQOL was significantly worse than U.S. normal values for six of eight domains of the SF-36. Several domains demonstrated moderate correlation (r value>0.40) with 6-minute-walk distance and World Health Organization functional class; there were no significant associations with hemodynamics. In univariable Cox proportional hazard models, six of eight domains and both summary scores were significantly associated with survival. In multivariable models, adjusted for age, disease type, and cardiac function, these relationships largely persisted. Conclusions: In this cohort of patients with pulmonary arterial hypertension, HRQOL, as assessed by the SF-36, was strongly associated with transplant-free survival. These relationships persisted when controlling for potential confounders such as disease type and disease severity. These findings suggest thatHRQOLmay be an important predictor of outcomes in pulmonary arterial hypertension and therefore a target for future therapeutic interventions.
AB - Rationale: Pulmonary arterial hypertension is a progressive disease with high morbidity and mortality despite advances in medical therapy. The relationship between patient-related outcomes, such as healthrelated quality of life (HRQOL), and survival is not well described. Objective: To assess the relationship between HRQOL and outcomes in patients with pulmonary arterial hypertension. Methods:Consecutivepatientswith right heart catheterization-proven pulmonary arterial hypertension who completed the Medical Outcomes Survey Short Form-36 survey (SF-36) were included. Demographic, clinical, physiological, and hemodynamic data were collected at baseline. Survival was assessed from the time of diagnosis of pulmonary arterial hypertension. Cox proportional hazard models were constructed to assess the relationship between HRQOL and transplant-free survival. Measurements and Main Results: Eighty-seven patients with pulmonary arterial hypertension were enrolled and followed prospectively for a median of 3.8 years. At baseline, HRQOL was significantly worse than U.S. normal values for six of eight domains of the SF-36. Several domains demonstrated moderate correlation (r value>0.40) with 6-minute-walk distance and World Health Organization functional class; there were no significant associations with hemodynamics. In univariable Cox proportional hazard models, six of eight domains and both summary scores were significantly associated with survival. In multivariable models, adjusted for age, disease type, and cardiac function, these relationships largely persisted. Conclusions: In this cohort of patients with pulmonary arterial hypertension, HRQOL, as assessed by the SF-36, was strongly associated with transplant-free survival. These relationships persisted when controlling for potential confounders such as disease type and disease severity. These findings suggest thatHRQOLmay be an important predictor of outcomes in pulmonary arterial hypertension and therefore a target for future therapeutic interventions.
KW - Outcomes
KW - Pulmonary hypertension
KW - Quality of life
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U2 - 10.1513/AnnalsATS.201412-572OC
DO - 10.1513/AnnalsATS.201412-572OC
M3 - Article
C2 - 26492065
AN - SCOPUS:84989170889
SN - 2325-6621
VL - 13
SP - 31
EP - 39
JO - Annals of the American Thoracic Society
JF - Annals of the American Thoracic Society
IS - 1
ER -