Although single individual reports have documented outcomes in children with pentalogy of are few data available for postoperative outcome of this cohort of patients after cardiac surgery. The aim of this study was to retrospectively review the clinical details of patients with
pentalogy of Cantrell managed at two centers. Two cardiac surgical institutions retrospectively studied all patients with
pentalogy of Cantrell and significant
congenital heart disease who underwent surgical intervention, excluding PDA
ligation, between 1992 and 2004. Seven children with
pentalogy of Cantrell underwent surgical intervention at a median age of 60 days (range, 1-11 months). Three patients had
tetralogy of Fallot, two
double outlet right ventricle, one patient had
tricuspid atresia, and one patient a perimembranous
ventricular septal defect. The mean duration of postoperative ventilation was 112.8 days (range, 4-335 days) but three patients required ventilation for more than 100 days. Patients who had a preoperative diaphragmatic plication required a longer duration of ventilation (mean = 186.5 days [range, 100-273 days] compared with mean = 132 days [range, 4-335 days]). Four patients survived, with three patients weaned from ventilation. Three patients had withdrawal of care following failure to wean from ventilation, following multisystem organ failure, and at the request of their parents. In conclusion, the
postoperative care of children with
pentalogy of Cantrell after cardiac surgery is often complicated by prolonged need for ventilatory support and multiple postoperative complications. Earlier surgical intervention does not necessarily reduce morbidity and mortality. These data may help in the counseling of parents prior to surgical intervention.