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.

Objective Despite overall improvements in congenital heart disease outcomes, racial and ethnic disparities have continued. The purpose of this study is to examine the effect of race and ethnicity, as well as other risk factors on congenital heart surgery length of stay and in-hospital mortality. Design From the 2012 Healthcare Cost and Utilization Project Kids Inpatient Database (KID), we identified 13,130 records with Risk Adjustment in Congenital Heart Surgery complexity score-eligible procedures. Multivariate logistic and linear regression modeling with survey weights, stratification and clustering was used to examine the relationships between predictor variables and length of stay as well as in-hospital mortality. Results No significant mortality differences were found among all race and ethnicity groups across each age group. Black neonates and black infants had a longer length of stay (neonatal Estimate = 8.73 days, p = .0034; infant Estimate 1.10 days, p = 0.0253), relative to whites. Government-sponsored insurance was associated with increased odds of neonatal mortality (odds ratio = 1.51, p = .0055), increased length of stay in neonates (Estimate = 4.26 days, p = .0009) and infants (Estimate = 1.52 days, p = .0181), relative to private insurance. Government-sponsored insurance was associated with increased number of chronic conditions, which were also associated with increased LOS (estimate 8.39 days, p < 0.001 in neonates; estimate 3.60 days, p < 0.001 in infants; estimate 1.87 days, p < 0.001 children). Conclusions Racial/ethnic disparities in congenital heart surgical outcomes may be changing compared to previous studies using the KID database. Increased length of stay in children with government-sponsored insurance may reflect expansion of individual states government-sponsored insurance eligibility criteria for children with complex chronic medical conditions. These findings warrant cautious optimism regarding racial and ethnic disparities in congenital heart surgery outcomes.

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.

Clinical case reports (CCRs) provide an important means of sharing clinical experiences about atypical disease phenotypes and new therapies. However, published case reports contain largely unstructured and heterogeneous clinical data, posing a challenge to mining relevant information. Current indexing approaches generally concern document-level features and have not been specifically designed for CCRs. To address this disparity, we developed a standardized metadata template and identified text corresponding to medical concepts within 3,100 curated CCRs spanning 15 disease groups and more than 750 reports of rare diseases. We also prepared a subset of metadata on reports on selected mitochondrial diseases and assigned ICD-10 diagnostic codes to each. The resulting resource, Metadata Acquired from Clinical Case Reports (MACCRs), contains text associated with high-level clinical concepts, including demographics, disease presentation, treatments, and outcomes for each report. Our template and MACCR set render CCRs more findable, accessible, interoperable, and reusable (FAIR) while serving as valuable resources for key user groups, including researchers, physician investigators, clinicians, data scientists, and those shaping government policies for clinical trials.