Single-stage repair for coarctation with ventricular septal defect: results of 100 cases at a single centre.

OBJECTIVES: The outcomes of the single-stage surgical repair of aortic arch hypoplasia (AAH) and/or coarctation of the aorta (CoA) associated with ventricular septal defect (VSD) remain controversial, especially in a lower middle-income country. This study reports the results of a single-stage repair protocol at our institution for AAH/CoA with VSD using selective cerebral perfusion. METHODS: This retrospective study included 100 consecutive patients who underwent single-stage repair via median sternotomy using selective cerebral perfusion for AAH/CoA with VSD from July 2010 to March 2017. RESULTS: The patients consisted of 65 males and 35 females. The median age of the patients was 67 days (range 4-2266 days); the median weight was 3.8 kg (range 2.1-15 kg). The average cardiopulmonary bypass time was 132 ± 28 min, the aortic cross-clamp time was 92 ± 23 min and the selective cerebral perfusion time was 33 ± 10 min. The survival rate of all patients was 94.7 ± 2.3%, with an in-hospital mortality of 5% and no late mortality at a median follow-up of 37 months (range 4-96 months). Four patients required reoperation due to recoarctation. The overall event-free survival rate following surgery was 87.1%. The median pressure gradient across the anastomosis at the last follow-up was 8.3 ± 2.8 mmHg. Multivariate logistic regression analysis revealed proximal aortic arch obstruction as a predictor of mortality (odds ratio = 3.8). The aortic isthmus diameter was identified as a predictor for reintervention by Cox regression (hazard ratio = 6.7). CONCLUSIONS: Single-stage repair for AAH/CoA with VSD is safe and feasible in a developing country.

A rare complication of myocardial ischaemia following single-stage repair in a case of Berry syndrome.

Berry syndrome is a rare congenital cardiac lesion consisting of a distal aortopulmonary window, the aortic origin of the right pulmonary artery (PA), intact ventricular septum and an interrupted or hypoplastic aortic arch. Different repair techniques have been described in the literature. We report a case of Berry syndrome, in whom myocardial ischaemia developed following direct implantation of the right PA to the main PA, which was resolved using an interposition tube graft.