Outcome of Children with Pentalogy of Cantrell Following Cardiac Surgery

Abstract

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.

Cantrell syndrome with complex cardiac malformations: a case report

“…Five distinct body folds fuse at the base of the umbilical cord. Defects of the cephalic fold could result in the core features of the pentalogy [6]. There is no etiologic study that either links the congenital defects to maternal risk factors or explains the underlying development mechanisms.…”

“…High morbidity ensues even if the surgery is successful and the child survives [6]. It has been found previously that survivors underwent operation on average at 4.1 years old, and nonsurvivors, at 0.7 years old [4].…”

Multidetector Computed Tomographic Angiography Imaging of Pentalogy of Cantrell

“…3 Our case represented the full spectrum of the pentalogy of Cantrell. Our case presented with a complex combination of thoracoabdominal ectopia cordis in association with single ventricle, atrial septal defect, and absent left pulmonary artery originating from the undersurface of the aortic arch by way of patent ductus arteriosus; thus, the prognosis was poor.…”

Cantrell Sendromlu Bir Yenidoğan

“…Ventriküler septal defekt (VSD) tüm olgularda görülmüştür. Bunun yanında bazı olgularda Fallot tetralojisi, atrial septal defekt (ASD), pulmoner arter hipoplazisi, büyük damarlarda transpozisyon ve sol ventrikül divertükülü gibi kalp anomalileri bildirilmiştir (6,7 (6,7). Olgumuzda ekokardiyografi ve BT anjiyo kombinasyonu ile cerrahi planlamayı gerçekleştirdik.…”