RESUMEN
Contemporary multicenter data regarding midterm outcomes for neonates with pulmonary atresia with intact ventricular septum are lacking. We sought to describe outcomes in a contemporary multicenter cohort, determine factors associated with end-states, and evaluate the effect of right ventricular coronary dependency and coronary atresia on transplant-free survival. Neonates treated during 2009-2019 in 19 United States centers were reviewed. Competing risks analysis was performed to determine cumulative risk of each end-state, and multivariable regression analyses were performed to identify factors associated with each end-state and transplant-free survival. We reviewed 295 patients. Median tricuspid valve Z-score was - 3.06 (25%, 75%: - 4.00, - 1.52). Final end-state was biventricular repair for 45 patients (15.2%), one-and-a half ventricle for 16 (5.4%), Fontan for 75 (25.4%), cardiac transplantation for 29 (9.8%), and death for 54 (18.3%). Seventy-six patients (25.7%) remained in mixed circulation. Cumulative risk estimate of death was 10.9%, 16.1%, 16.9%, and 18.8% at 1, 6 months, 1 year, and 5 years, respectively. Tricuspid valve Z-score was inversely, and coronary atresia positively associated with death or transplantation [odds ratio (OR) = 0.46, (95% confidence interval (CI) = 0.29-0.75, p < 0.001) and OR = 3.75 (95% CI 1.46-9.61, p = 0.011), respectively]. Right ventricular coronary dependency and left coronary atresia had a significant effect on transplant-free survival (log-rank p < 0.001). In a contemporary multicenter cohort of patients with PAIVS, consisting predominantly of patients with moderate-to-severe right ventricular hypoplasia, we observed favorable survival outcomes. Right ventricular coronary dependency and left, but not right, coronary atresia significantly worsens transplant-free survival.
RESUMEN
BACKGROUND: We aimed to determine whether infants undergoing cardiac surgery would more efficiently attain negative fluid balance postoperatively with passive peritoneal drainage as compared to traditional pleural drainage. METHODS: A prospective, randomized study including children undergoing repair of tetralogy of Fallot (TOF) or atrioventricular septal defect (AVSD) was completed between September 2011 and June 2013. Patients were randomized to intraoperative placement of peritoneal catheter or right pleural tube in addition to the requisite mediastinal tube. The primary outcome measure was fluid balance at 48 hours postoperatively. Variables were compared using t tests or Fisher exact tests as appropriate. RESULTS: A total of 24 patients were enrolled (14 TOF and 10 AVSD), with 12 patients in each study group. Mean fluid balance at 48 hours was not significantly different between study groups, -41 ± 53 mL/kg in patients with periteonal drainage and -9 ± 40 mL/kg in patients with pleural drainage (P = .10). At 72 hours however, postoperative fluid balance was significantly more negative with peritoneal drainage, -52.4 ± 71.6 versus +2.0 ± 50.6 (P = .04). On subset analysis, fluid balance at 48 hours in patients with AVSD was more negative with peritoneal drainage as compared to pleural, -82 ± 51 versus -1 ± 38 mL/kg, respectively (P = .02). Fluid balance at 48 hours in patients with TOF was not significantly different between study groups. CONCLUSION: Passive peritoneal drainage may more effectively facilitate negative fluid balance when compared to pleural drainage after pediatric cardiac surgery, although this benefit is not likely universal but rather dependent on the patient's underlying physiology.