Abstract
BackgroundPulmonary arterial hypertension (PAH) remains a life-threatening condition, despite modern therapies. We prospectively investigated the therapeutic health-related quality of life (HRQOL) effects of goal-oriented sequential combination therapy based on exercise capacity in patients newly diagnosed with PAH.MethodsTo examine the changes in HRQOL in PAH patients, we treated 30 patients newly diagnosed with PAH with goal-oriented sequential combination therapy based on exercise capacity. We monitored exercise capacity by cardiopulmonary exercise testing and observed the benefit of using a peak VO2 cut-off of 15 mL/kg/min to guide combination therapy. First-line treatment was an endothelin receptor antagonist (ERA); second-line treatment was the addition of a phosphodiesterase-5 inhibitor (PDE-5I). At baseline and at 3, 6, and 12 months, HRQOL was evaluated by using the eight-item Medical Outcomes Survey Short Form Health Survey.ResultsAt 12 months, 100% of PAH patients were receiving an ERA, and 82% an ERA + PDE-5I. The mean physical component summary (PCS) score was 33.5 at baseline, 41.2 at 3 months, 40.8 at 6 months, and 42.0 at 12 months, and the mean mental component summary (MCS) scores were 45.6, 47.0, 50.0, and 50.1, respectively. PCS score was significantly greater at 3 months than at baseline (P = 0.035). MCS score was comparable at 3 months and at baseline, but was significantly greater at 6 and 12 months than at baseline (P = 0.033, P = 0.028, respectively). Thus, PCS score improved soon after initiation of therapy, and MCS score improved later.ConclusionsTogether, these results suggest that goal-oriented sequential combination therapy based on exercise capacity improves HRQOL in patients with PAH.
Highlights
Pulmonary arterial hypertension (PAH) is a life-threatening condition that is associated with poor prognosis [1], despite the availability of many therapeutic options
Since the reliability and validity of the Japanese version of the − 36 (SF-8) was confirmed in the general Japanese population [17], it has been used in several Japanese studies as a measure of health-related quality of life (HRQOL) outcomes [18,19,20]
Patients with any of the following conditions at enrollment were excluded: 1) pulmonary hypertension corresponding to group 2, 3, 4, or 5 in the classification or PAH with congenital heart disease; 2) pregnancy; 3) serum creatinine > 2.0 mg/dL; 4) history of serious chronic obstructive pulmonary disease or restrictive lung disease; 5) inability to walk without personal assistance; 6) currently receiving PAH-targeted therapy such as an endothelin receptor antagonists (ERAs), a phosphodiesterase-5 inhibitors (PDE-5Is), or intravenous epoprostenol; 7) any other condition that, by judgment of the physicians in charge, made enrollment inappropriate because of concerns for patient safety
Summary
Pulmonary arterial hypertension (PAH) is a life-threatening condition that is associated with poor prognosis [1], despite the availability of many therapeutic options. Hirashiki et al Health and Quality of Life Outcomes (2019) 17:103 cardiopulmonary exercise testing (CPX) for guiding treatment in patients with pulmonary hypertension [5, 7,8,9,10]. The 36-item Medical Outcomes Study Short Form Health Surveys (SF-36) are general, non-diseasespecific instruments for assessing QOL and are considered useful for evaluating treatment efficacy in patients with pulmonary hypertension [13,14,15]. Pulmonary arterial hypertension (PAH) remains a life-threatening condition, despite modern therapies. We prospectively investigated the therapeutic health-related quality of life (HRQOL) effects of goal-oriented sequential combination therapy based on exercise capacity in patients newly diagnosed with PAH
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