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Background The purpose of this study is to report short and long term outcomes following 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 was obtained through linkage with the National Death Index. Results A total of 50 patients with Trisomy 13 and 121 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 for patients with Trisomy 13 or Trisomy 18 was 27.6% and 13%, respectively. Causes of in-hospital mortality were primarily cardiac (64.7%) or multiple organ system failure (17.6%). National Death Index linkage confirmed 23 post-discharge deaths. Median survival (conditioned to hospital discharge) was 14.8 years (95% CI 12.3–25.6 years) for patients with Trisomy 13 and 16.2 years (95% CI 12–20.4 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 for all surgical risk categories was higher in patients with Trisomy 13 or 18 than 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.

The location and ligation of right ventricle to coronary artery connections can be reliably accomplished off bypass. Coronary flow became antegrade, improving myocardial oxygenation. No myocardial damage was observed. Inapparent right ventricle to coronary artery connections occasionally enlarge secondarily after right ventricular decompression, making early follow-up evaluation necessary after ligation. Despite the initial presence of significant right ventricle to coronary artery connections, 2-ventricle repairs are possible with long-term benefits.

In this diverse setting, race/ethnicity was not associated with increased LOS, age at first echocardiogram, length of ventilation, or complications. Surgical complexity, chronic conditions, and complications were associated with increased LOS. We discuss some interventions to reduce disparities in congenital heart surgery outcomes.