The Utility of Cardiopulmonary Exercise Testing for the Prediction of Outcomes in Ambulatory Children With Dilated Cardiomyopathy

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.