Does Down syndrome influence the outcomes of congenital cardiac surgery? A systematic review and meta-analysis

Background Congenital cardiac surgery for individuals with Down syndrome (DS) has historically occurred at a reduced frequency. Little data are available regarding long-term post-congenital cardiac surgical outcomes. Limited sample sizes and clinical heterogeneity require a pooled analysis approach. Aims To compare long-term outcomes post-congenital heart surgery between adults with and without DS. Methods Databases (Medline, Embase, and PubMed) were searched utilizing terms related to DS and congenital heart disease. Studies that enrolled adults (>18 years) with operated congenital heart disease and compared long-term outcomes with respect to DS presence were included. All study designs were included, but those with limited/peri-operative follow-up, non-English texts, case studies, and literature reviews were excluded. Blinded screening, data extraction, and quality assessment were independently conducted by two reviewers. QUIPS criteria were used for risk of bias analysis. Both random- and fixed-effects models were used for meta-analysis. Results A total of 23 studies (n = 10 466) were included. Risk of bias was frequently high due to unblinded retrospective study designs and analyses limited in adjustment for other prognostic factors. Meta-analysis demonstrated no effect of DS on long-term mortality [hazard ratio (HR) 0.86, 95% confidence interval (95% CI) 0.6-1.23], to a maximum described follow-up of 38 years. Lower cardiac reoperation risk (HR 0.6, 95% CI 0.46-0.78) for individuals with DS was found on pooled analysis. Meta-analysis was limited by between-study variation. Conclusion DS does not affect post-congenital cardiac surgical survival in adulthood. Reduced reoperation may reflect challenges in assessing functional and symptomatic status and/or concerns regarding perceived reoperation difficulties or likely benefits.

Automated summary

This study aims to compare the long-term outcomes after cardiac surgery in adults with and without Down syndrome (DS). The meta-analysis showed that DS does not affect long-term survival after congenital cardiac surgery in adulthood, but individuals with DS have a lower risk of cardiac reoperation. The study is limited by limited sample sizes and clinical heterogeneity.