Operative and nonoperative outcomes in patients with trisomy 13 and 18 with congenital heart diseaseCentral MessagePerspective

Objective: To evaluate the short- and long-term outcomes of cardiac repair versus nonoperative management in patients with trisomy 13 and trisomy 18 with congenital heart disease. Methods: An institutional review board-approved, retrospective review was undertaken to identify all patients admitted with trisomy 13/18 and congenital heart disease. Patients were divided into 2 cohorts (operated vs nonoperated) and compared. Results: Between 1985 and 2023, 62 patients (34 operated and 28 nonoperated) with trisomy 13 (n = 9) and trisomy 18 (n = 53) were identified. The operated cohort was 74% girls, underwent mainly The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1 procedures (n = 24 [71%]) at a median age of 2.5 months (interquartile range [IQR], 1.3-4.5 months). This compares with the nonoperative cohort where 64% (n = 18) would have undergone The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1 procedures if surgery would have been elected. The most common diagnosis was ventricular septal defect. Postoperative median intensive care unit stay was 6.5 days (IQR, 3.7-15 days) with a total hospital length of stay of 15 days (IQR, 11-49 days). Thirty-day postoperative survival was 94%. There were 5 in-hospital deaths in the operated and 7 in the nonoperated cohort. Median follow-up was 15.4 months (IQR, 4.3-48.7 months) for the operated and 11.2 months (IQR, 1.2-48.3 months) for the nonoperated cohorts. One-year survival was 79% operated versus 51.5% nonoperated (P < .003). Nonoperative treatment had an increased risk of mortality (hazard ratio, 3.28; 95% CI, 1.46-7.4; P = .004). Conclusions: Controversy exists regarding the role of primary cardiac repair in patients with trisomy 13/18 and congenital heart disease. Cardiac repair can be performed safely with low early mortality and operated patients had higher long-term survival compared with nonoperated in our cohort

Brain injury and neurodevelopmental outcomes in children undergoing surgery for congenital heart diseaseCentral MessagePerspective

Objectives: Brain injury is commonly seen on magnetic resonance imaging in infants with complex congenital heart disease. The impact of perioperative brain injury on neurodevelopmental outcomes is not well understood. We evaluate the association of brain injury and other markers on neurodevelopmental outcomes in patients undergoing surgery for congenital heart surgery during infancy. Methods: Term newborns with infant cardiac surgery performed between 2008 and 2019 at a single tertiary center, and both preoperative and postoperative brain magnetic resonance imaging were included. Those with underlying genetic conditions were excluded. Brain injury was characterized using an magnetic resonance imaging scoring system. Neurodevelopmental outcomes were assigned using the Pediatric Stroke Outcome Measure and Glasgow Outcome Scale Extended. Independent risk factors for poor neurodevelopmental outcomes were determined by multivariable Cox regression. Results: A total of 122 patients were included. New or progressive postoperative brain injury was noted in 69 patients (57%). A total of 101 patients (83%) had at least 1 neurodevelopmental assessment (median age 36 months) with an early assessment (5-24 months) performed in 95 children. Multivariable Cox regression analysis of early neurodevelopmental outcomes identified new stroke on postoperative magnetic resonance imaging to be an independent predictor of poor neurodevelopmental outcome. Postoperative peak lactate was an independent predictor of poor outcome assessed by the Pediatric Stroke Outcome Measure and Glasgow Outcome Scale Extended. Conclusions: Our study reveals that evidence of new stroke on magnetic resonance imaging after infant congenital heart surgery is a predictor of poor neurodevelopmental outcomes in early childhood. Postoperative lactic acidosis is associated with poor neurodevelopmental outcome and may be a surrogate biomarker for ischemic brain injury