Evaluation of disease-specific health-related quality of life in patients with pulmonary arterial hypertension.

摘要: Summary Background Pulmonary arterial hypertension (PAH) remains a debilitating and life-threatening disease despite improvements in hemodynamics, exercise capacity survival with recent therapeutic advances. Health-related quality of life (HRQOL) has, therefore, been proposed as an important outcome for evaluating care. Relatively little, however, is known regarding HRQOL or its determinants PAH. The Minnesota Living Heart Failure questionnaire was recently adapted validated measurement We applied this pulmonary hypertension-specific version (MLHF-PH) to larger population PAH patients. Methods Ninety-three consecutive outpatients completed the MLHF-PH. Scores were assessed correlations demographics, symptoms, hemodynamics treatments. Results Patients had significantly impaired by disease-specific Each physical emotional component, well total scores on MLHF-PH indicated severely depressed HRQOL. As compared other diagnoses, associated scleroderma worst WHO functional Class II symptoms reported better than III Fatigue, weakness abdominal discomfort each more HRQOL, current epoprostenol use. With sole exception right atrial pressure, hemodynamic measurements did not correlate scores. Simultaneous evaluation non-disease-specific (SF-36) revealed similarly status, although identified fewer associations patient-specific factors. Conclusion Severely present patients evaluated questionnaire. availability may enable further targeted investigations factors that might improve outcomes.