Impacto del síndrome de deleción 22q11.2 en la mortalidad de los pacientes pediátricos con defectos conotruncales corregidos

Abstract

Introduction and objectives

The 22q11.2 deletion syndrome (22q11.2 DS), known as DiGeorge syndrome, is phenotypically associated with conotruncal heart defects. The aim of this review was to evaluate mortality in pediatric patients with corrected conotruncal defects.

Methods

A literature search was conducted in the databases PubMed, ScienceDirect, Google Scholar, and Redalyc databases, covering the last 10 years. Original articles with pediatric population diagnosed with 22q11.2 DS and conotruncal congenital heart defects were included. A statistical analysis of 5 studies was performed, evaluating the odds ratio (OR), 95% confidence intervals (95%CI), and statistical significance using the chi-square test for each exposure variable. The ROBINS-E tool was used to assess the risk of bias.

Results

A total of 18 articles were collected, including a total population of 10 993 patients (3225 with 22q11.2 DS). The most frequent cardiac defect was tetralogy of Fallot (n = 655). The most common intraoperative complications were arrhythmias, and the most common postoperative complications were infections. Mortality in patients with 22q11.2 DS was assessed. The OR was 0.74 (95%CI, 0.38-1.45; P = .38). Heterogeneity test yielded an I² = 66%.

Conclusions

22q11.2 DS is not associated with post-surgical mortality in patients treated for conotruncal congenital heart defects.