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Quality of Life in PAH: Clinical and Functional Correlates

Myung Park


M. Chesney

T. Aje

Gautam Ramani


S. Synowski

J. Frederick

W. Kop

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Conference: 2010 International PHA Conference and Scientific Sessions

Release Date: 06.24.2010

Presentation Type: Abstracts

Park M, Chesney M, Aje T, Ramani G, Synowski S, Frederick J, Kop W. 
University of Maryland School of Medicine, Baltimore, MD, USA

BACKGROUNDPulmonary artery hypertension (PAH) is associated with adverse long-term clinical outcomes and poor quality of life (QOL). Determinants of QOL in PAH are insufficiently understood and this study examined clinical predictors of general health-related QOL and PAH-specific QOL.

METHODSThirty-six PAH patients were enrolled [age=59±14 yrs, 67% women; 14 idiopathic, 10 connective tissue disease (CTD), 4 congenital heart disease, 8 other PAH]. General health-related QOL was measured with the SF-12, and PAH-specific QOL with the CAMPHOR. SF-12 scores (range 0-100) were standardized to allow comparisons with population reference values for Physical and Mental Summary QOL scores (PSQOL and MS-QOL). A novel scale measuring patient-reported PAH responses (PAH-R) was designed for the purpose of this investigation.

RESULTSQOL was significantly impaired in PAH patients compared to reference values (mean PS-QOL and MS-QOL scores 36.2±8.2 and 42.7±5.7, resp.). CTD patients reported lower general physical QOL (p<0.05) (Fig 1). Continuous oxygen use was related to poor general QOL (p<0.05) but not other domains of QOL. Younger age was associated with poorer mental health (p=0.035). CAMPHOR score significantly correlated with physical (r=0.60) and mental (0.79) QOL (p<0.001). Treatment with parenteral PAH medications (n=10) did not result in poorer QOL ratings on any of the measures. 6MWD and hemodynamic parameters were also not predictive of QOL.

CONCLUSIONCTD and continuous oxygen use adversely affected physical QOL in PAH. Reduced mental QOL was associated with younger age. Further longitudinal research is needed to determine the clinical impact of QOL assessments in the clinical management of PAH.