Primary definitive repair of interrupted aortic arch with ventricular septal defect

Abstract

The optimal surgical management (primary or staged repair) of interrupted aortic arch (IAA) with ventricular septal defect (VSD) remains to be determined. A consecutive series of 14 neonates, aged 3-18 days (mean: 10 +/- 6 days) underwent primary complete repair. Mean weight was 3.3 +/- 0.4 kg. Eleven patients had IAA type B, 2 had type A and 1 had type C. Six infants had the Di George syndrome. Preoperative management (mean: 5 +/- 4 days) included prostaglandin E1 (14/14), intubation and ventilation (13/14), and inotropic support (11/14). Surgery was performed under deep hypothermia and circulatory arrest and involved resection of all ductal tissue, direct end-to-side aortic arch anastomosis and patch closure of the VSD. There were 2 early deaths (14%, 70% CL: 5%-31%): low cardiac output (1), residual VSD (1). Four patients (33%, 70% CL: 13%-52%) underwent reoperation for recurrent aortic obstruction (3 patients, 1 death) or left ventricular outflow tract obstruction (LVOTO) (1 patient). The results improved with time: no death and no recurrent aortic obstruction in the last 8 patients. At last follow-up (11 patient, mean follow-up = 24 +/- 9 months), all patients were free of cardiac symptoms; none had persistent aortic obstruction; 4 had LVOTO (gradient greater than 20 mm Hg) and 1 (with the Di George syndrome) had severe mental disorders. Primary complete repair provides satisfactory results in most infants born with IAA and VSD. An adequate direct aortic arch anastomosis should entail a low risk of recurrent obstruction. LVOTO develops in many cases and may require further surgery.

Management strategies for interrupted aortic arch with associated anomalies

“…The aim is to improve lower body perfusion, improve renal function, diminish acidosis, and maximise the pulmonary/systemic resistance ratio across the ductus arteriosus [7]. A high index of suspicion is needed for necrotising enterocolitis [32]. Cardiac catheterisation should be avoided and MRI scan considered if echo is inconclusive [7].…”

“…Resection of all ductal tissues and extensive mobilisation of the descending aorta is important to prevent restenosis [7,32]. The techniques employed to repair the aortic arch are direct anastomosis between ascending and descending aorta; direct anastomosis with patch augmentation or a conduit interposition [7].…”

“…Preoperative management includes prostaglandin, and may include assisted mechanical ventilation and dopamine infusion [7,32]. The aim is to improve lower body perfusion, improve renal function, diminish acidosis, and maximise the pulmonary/systemic resistance ratio across the ductus arteriosus [7].…”

“…Recent advances in neonatal cardiac surgery have made it feasible to repair IAA-VSD within the first month after birth [6][7][8]. During the last several years, results of both primary and two-stage repair of IAA have improved, and early mortality in some centres approaches 8-10% [7,[31][32][33].…”

“…However, it carries the risk of recurrent obstruction due to tension at the anastomosis site or inadequate resection of ductal tissues [31,32]. It can also result in bronchial compression [31].…”