Long-Term Outcomes of Children With Trisomy 13 and 18 After Congenital Heart Disease Interventions

• Published in: The Annals of thoracic surgery Vol. 103; no. 6; pp. 1941 - 1949
• Main Authors: Peterson, Jennifer K., MS, Kochilas, Lazaros K., MD, MSCR, Catton, Kirsti G., MSN, Moller, James H., MD, Setty, Shaun P., MD
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Inc 01-06-2017
• Subjects: Canada; Cardiothoracic Surgery; Cause of Death; Chromosome Disorders - complications; Chromosome Disorders - mortality; Chromosomes, Human, Pair 13; Chromosomes, Human, Pair 18; Female; Follow-Up Studies; Heart Defects, Congenital - complications; Heart Defects, Congenital - mortality; Heart Defects, Congenital - surgery; Hospital Mortality; Humans; Infant; Infant, Newborn; Male; Retrospective Studies; Surgery; Trisomy; Trisomy 13 Syndrome; Trisomy 18 Syndrome; United States; Canada; United States; National Death Index; Risk Adjustment in Congenital Heart Surgery, version 1; trisomy 13; CI; VSD; TOF; IQR; interquartile range; tetralogy of Fallot; congenital heart defect; NDI; STAT; PCCC; HIPAA; T13; Pediatric Cardiac Care Consortium; confidence interval; T18; ventricular septal defect; Health Insurance Portability and Accountability Act; CHD; trisomy 18; RACHS-1; Society of Thoracic Surgeons/European Association for Cardiothoracic Surgery Mortality
• ISSN: 0003-4975; 1552-6259
• DOI: 10.1016/j.athoracsur.2017.02.068

Background The purpose of this study is to report short- and long-term outcomes after congenital heart defect (CHD) interventions in patients with trisomy 13 or 18. Methods A retrospective review of the Pediatric Cardiac Care Consortium (PCCC) identified children with trisomy 13 or 18 with interventions for CHD between 1982 and 2008. Long-term survival and cause of death were obtained through linkage with the National Death Index. Results A total of 50 patients with trisomy 13 and 121 patients with trisomy 18 were enrolled in PCCC between 1982 and 2008; among them 29 patients with trisomy 13 and 69 patients with trisomy 18 underwent intervention for CHD. In-hospital mortality rates for patients with trisomy 13 or trisomy 18 were 27.6% and 13%, respectively. Causes of in-hospital death were primarily cardiac (64.7%) or multiple organ system failure (17.6%). National Death Index linkage confirmed 23 deaths after discharge. Median survival (conditioned to hospital discharge) was 14.8 years (95% confidence interval [CI]: 12.3 to 25.6 years) for patients with trisomy 13 and 16.2 years (95% CI: 12 to 20.4 years) for patients with trisomy 18. Causes of late death included cardiac (43.5%), respiratory (26.1%), and pulmonary hypertension (13%). Conclusions In-hospital mortality rate for all surgical risk categories was higher in patients with trisomy 13 or 18 than that reported for the general population. However, patients with trisomy 13 or 18, who were selected as acceptable candidates for cardiac intervention and who survived CHD intervention, demonstrated longer survival than previously reported. These findings can be used to counsel families and make program-level decisions on offering intervention to carefully selected patients.