Background: Anomalous origin of pulmonary artery branch from ascending aorta(AOPA) in the presence of two separate semilunar valves is a rare but important entity necessitating early diagnosis and surgery to prevent development of irreversible pulmonary vascular disease. We evaluated our experience with the technique and outcome of eleven patients with this condition. Methods: Between January 2000 and December 2018, eleven patients were diagnosed with AOPA. Echocardiographic data were collected from the records, regarding the site of origin of the anomalous pulmonary artery, additional defects, biventricular function and pulmonary artery pressures. Intraoperative charts were reviewed for the details of the surgical procedure and cardiopulmonary bypass. Postoperative data included survival, ventilatory support and duration of hospital stay. After discharge, children were reviewed at 1 month, 3 months, 6 months and then at yearly intervals. Results: Of the eleven patients, male to female ratio was 4:7 with a median age of six months(15 days - 28 years) and median weight of 5.7 kg(1.8 kg - 40 kg). Nine patients underwent direct re-implantation of anomalous pulmonary artery branch to main pulmonary artery. The survival rate was 91% in our series. On follow-up, no re-operations or re-interventions were required and all patients who underwent surgical correction were in stable clinical condition. Conclusion: Early and midterm outcomes of children who underwent surgery for AOPA is good. Early direct re-implantation of the anomalous branch PA to main pulmonary artery is the optimal surgical strategy for these patients

Double-outlet left ventricle (DOLV) is a rare congenital cardiac anomaly. The aorta and the main pulmonary arterial trunk arises predominantly from the left ventricle(LV) and is associated with a malaligned ventricular septal defect(VSD), various degrees of hypoplasia of the right ventricle, and presence or absence of pulmonary stenosis. Bi-ventricular repair is the preferred treatment option whenever possible. Various techniques for bi-ventricular repair have been described. The best option for DOLV correction is by translocating the pulmonary root to the right ventricle(RV)[1]. In this series, we report four patients who underwent biventricular repair of DOLV in our institute with excellent outcomes. All patient details were collected from the institute patient record system. Echocardiographic data were obtained from the records. Intraoperative charts were reviewed for further information on the surgical procedure and cardiopulmonary bypass. Postoperative data included survival, functional status and followup echocardiography. Of the four children, three underwent pulmonary root translocation and one child underwent Reparation al etage Ventriculaire(REV) procedure. There was no mortality and all children are in stable clinical condition in the recent follow-up and no re-operations or interventions were required following primary surgical correction. Thus DOLV is anatomically and surgically a challenging subset. Pulmonary root translocation in this anatomy is technically challenging but safe and superior option when compared to other alternative surgical procedures and it can be performed with excellent results, even in infants.