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Chylothorax is a consequence of a thoracic duct injury that can occur during surgical procedures in patients with congenital heart disease. It is associated with high rates of morbimortality and increased use of clinical and hospital resources. The aim of this study was to evaluate the risk factors, distribution, manifestations, complications, and treatments for chylothorax in patients undergoing cardiac surgery in a tertiary pediatric hospital in southern Brazil. This is a retrospective, quantitative study, in which all medical records (n = 166) of patients with chylothorax after pediatric cardiac surgery between January 2014 and December of 2020 and a matched control group (n = 166) were analyzed. Over the study period, there was an increase in incidence of chylothorax from 4.5% in 2014 to 7.6% in 2020, a trend that has been reported in the literature. After multivariate analysis, the following were identified as risk factors for the diagnosis of chylothorax: genetic syndrome (OR 2.298); prolonged cardiopulmonary bypass time (greater than 120 min) (OR 2.410); fluid overload in the immediate postoperative period (OR 1.110); and SIRS (OR 2.527). Mortality was two times greater (p = 0.021) and there was a higher rate (34.4%) of infection (p < 0.001) in patients who developed chylothorax. In addition, a sensitivity analysis was performed comparing patients with low- and high-output chylothorax (> 20 mL/kg), which confirmed unfavorable outcomes for the latter group. Herein, we show that hemodynamic alterations were important factors for diagnosis. Understanding the risk factors, outcomes, and complications helps early identification and enables the reduction of morbidity and mortality. [ABSTRACT FROM AUTHOR]