In patients with severe LC-FAOD, UX007 interim study results demonstrated improved exercise endurance and tolerance, and were associated with positive changes in self-reported HR-QoL.
Background The minute ventilation-carbon dioxide production (VE/VCO 2 ) slope, obtained during exercise testing, possesses prognostic value in heart failure (HF). The VE-VCO 2 relationship is generally linear thereby hypothetically producing similar slope values regardless of the exercise-test time interval used for calculation. Design This study assesses the ability of the VE/VCO 2 slope, calculated at different time intervals throughout a progressive exercise test, to predict 1-year cardiac-related hospitalization and mortality in subjects with HF. Methods Seventy-two subjects underwent symptom-limited exercise testing with ventilatory expired gas analysis. Mean age and left ventricular ejection fraction for 44 male and 28 female subjects were 51.2 years ( ± 13.0) and 27.0% ( ± 12.3) respectively. The VE/VCO 2 slope was calculated from time 0 to 25, 50, 75 and 100% of exercise time and subsequently used to create five randomly selected VE/VCO 2 slope categories. Results (The intraclass correlation coefficient found calculation of the VE/VCO 2 slope, when divided into quartiles, to be a reliable measure (alpha = 0.94, P < 0.0001). Univariate Cox regression analysis revealed all VE/VCO 2 slope categories (25-100% and random selections) were significant predictors of cardiac-related hospitalization and mortality over a 1-year period. Multivariate Cox regression analysis revealed all VE/VCO 2 slope categories outperformed peak oxygen consumption (VO 2 ) in predicting hospitalization and mortality at 1 year. Conclusions Although the different classification schemes were not identical, these results suggest VE/VCO 2 slope maintains prognostic significance regardless of exercise-test time interval. Calculation of VE/VCO 2 slope may therefore still be valuable in subjects putting forth a sub-maximal effort while effort-dependent measures, such as peak VO 2 , are not.
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