Background Unlike adult patients, the utility of cardiopulmonary exercise testing (CPET) in children as a prognostic tool is unclear. We sought to examine the associations of CPET with outcomes in children with dilated cardiomyopathy (DCM). Methods This was a single-center, retrospective review of children with DCM who underwent CPET. The primary endpoint for this study was a time-dependent composite outcome of hospitalization for management of decompensated heart failure, initiation of mechanical circulatory support, heart transplant, or death. Results We examined 52 children with DCM who underwent CPET at median age 12.6 years (interquartile range [IQR], 9.9-14.6 years). At first CPET, the median peak heart rate was 80% (IQR, 70-88%) of predicted, median peak oxygen consumption 62% (IQR, 45-77%) of predicted, and median minute ventilation/carbon dioxide production slope 34.9 (IQR, 27.9-39.4). Eighteen (35%) patients reached the composite outcome during follow-up. Univariable factors associated with the composite outcome included: Lower peak heart rate predicted, lower blood pressure response, lower peak oxygen consumption predicted, and higher minute ventilation/carbon dioxide production slope. The association between exercise performance and composite outcome was linear; thus, no reliable cutoff point could be identified. Serial CPET had been performed in 30 patients; clinically, those with deterioration of exercise capacity had poorer outcomes. Conclusions Cardiopulmonary exercise testing is feasible in children with DCM and is useful to predict outcomes. The finding of lower exercise capacity and lower blood pressure response should prompt closer follow-up. In those with serial testing, a decline in exercise capacity may be a marker of clinical deterioration.
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