Impact of 22q11.2 Deletion on the Postoperative Course of Children After Cardiac Surgery

Abstract

The primary objective of this study was to describe the impact of 22q11.2 deletion (del22q11) on the clinical characteristics, postoperative course, and short-term outcomes of children undergoing surgery for congenital heart disease. The charts of all children ages 1 day-18 years who received cardiac surgery for interrupted aortic arch (IAA), tetralogy of Fallot (TOF), or truncus arteriosus (TA) repair from 1 January 2001 to 31 December 2011 were retrospectively reviewed. The patients were divided into two groups: the 22q11 group including children with del22q11 undergoing surgery for TOF, IAA, or TA and the non-22q11 or control group including children with no chromosomal or genetic abnormality undergoing surgery for TOF, IAA, or TA. Demographic information, cardiac diagnoses, noncardiac abnormalities, preoperative factors, intraoperative details, surgical procedures performed, postoperative complications, and in-hospital deaths were collected. The outcome data collected included days of inotrope use, need for dialysis, length of mechanical ventilation, intensive care unit (ICU) length of stay (LOS), hospital LOS, and mortality. The study enrolled 173 patients: 65 patients in the 22q11 group and 108 patients in the control group. Of the 65 patients in the 22q11 group, 36 (55 %) underwent repair for TOF, 13 (20 %) for IAA, and 16 (25 %) for TA. The two groups did not differ in terms of age or weight. The preexisting conditions were similar in the two groups. Unplanned noncardiac operations were more common in the children with del22q11, but delayed chest closure was similar in the two groups. The incidence of postoperative noncardiac complications such as reintubation, vocal cord paralysis, and diaphragmatic paralysis was similar in the two groups. However, increasing numbers of patients in del22q11 group needed dialysis in one form or the other during the immediate postoperative stay. The incidence of fungal infection and wound infection was higher in the del22q11 group than in the control group. Duration of mechanical ventilation, ICU LOS, and hospital LOS were similar in the two groups, except in certain subgroups. Mortality did not differ significantly between the two groups. In conclusion, children with del22q11 have a higher risk of postoperative complications after cardiac surgery, with no difference in length of mechanical ventilation, ICU LOS, hospital LOS, or mortality. However, short-term outcomes may differ in certain subgroups.

Predictors of Length of Hospital Stay After Complete Repair for Tetralogy of Fallot: A Prospective Cohort Study

“…Extracardiac malformations and 22q11.2 deletion were not associated with THD. This finding was consistent with our prior report but contrary to other studies that reported increased THD in TOF patients with 22q11.2 deletion or in patients with congenital heart defects and genetic syndromes . The lack of significance in our study could be ascribed in part to the fact that 22q11.2 deletion was not assessed in the entire cohort.…”

22q11.2 deletion syndrome and congenital heart disease

“…In the same study, those with truncus arteriosus and a 22q11.2 deletion underwent surgical intervention at an earlier age but still experienced more severe pulmonary hypertension than their non‐deleted counterparts. Likewise, McDonald et al () reported more frequent unplanned noncardiac operations, use of dialysis and infections in the patients with 22q11.2 deletion syndrome.…”

Associated genetic syndromes and extracardiac malformations strongly influence outcomes of fetuses with congenital heart diseases

“…This is the case for atrioventricular septal defect in Down syndrome, which has a more favorable anatomy compared with that of fetuses without chromosomal anomalies [1,4]. In the majority of studies, CHDs associated with extracardiac anomalies have worse outcomes [10][11][12][13][14][15]. However, relatively few studies have evaluated the overall effect of known genetic syndromes and major ECMs on pregnancy outcome and mortality in fetal CHD [16,17].…”