All four patients were female, with an average age of 28.8 days and an average weight of 3.64 kg. Only case 4 was born prematurely at 34 W+5 and was treated conservatively until 71 days to complete operation. All the others completed primary corrective surgery in the neonatal period, and all survived after operation. Two different surgical techniques were used to repair the aortic-pulmonary window and the aortic origin of the right pulmonary artery, including 1 case using the aortic internal baffle technique and another 3 cases underwent replantation of the right pulmonary artery (1 case was reconstructed in situ, and the other 2 cases were reconstructed by moving the right pulmonary artery in the anterior of aorta). Case 2 who received aortic internal baffle technique underwent two reoperation because of right pulmonary artery stenosis. While, right pulmonary artery of cases 3 and 4 developed well after being reconstructed the right pulmonary artery anterior translocation. After the diagnosis of Berry syndrome through cardiac color ultrasound and CT angiography, a primary surgical radical treatment should be performed as soon as possible at an experienced pediatric cardiac center. Although the surgery is high-risk and complex, it is safe and effective.

Objective This study was aimed to discuss the safety, feasibility and availability of perimembranous ventricular septal defects(PmVSD) closure via super minimal intercostal incision under transesophageal echocardiography(TEE) guidance in children.Methods There were 81 cases of PmVSD via super minimally invasive transintercostal device closure operation(length of incision ≤ 1cm) from August 2014 to August 2016.TEE was used to guide and monitor the entire procedure.Assessed the effectiveness of device closure by postoperative regular follow-up.Results 80 patients were successfully operated by super minimally invasive transintercostal device closure in all 81 cases.Operation success rate reached 98.77%.The average diameter of ventricular septal defects was(3.72±0.96)mm.The average diameter of amplatzer occluder was(4.88±0.95)mm.Postoperative follow-up time was from 6 months to 24 months.One of 16 cases that with aneurysm of membranous septum had more than two shunts, remained a mild residual shunt beside of the amplatzer occluder but self cured during follow-up.Mild pericardial effusion was found in one patients after the operation and disappeared during follow-up.There were no cases of perioperative death, device deformation or displacement, residual shunt, complete atrioventricular block and valve involvement in the patients whom were successfully operated.One patient transferred to ventricular septal defect repair operation under direct visualization with a cardiopulmonary bypass, because ventricular fibrillation happened when guide wire passed the ventricular septal.Conclusion Super minimally invasive transintercostal device closure of PmVSD in children guided by TEE was safety and availability, that could avoid x-ray radiation and sternotomy, and operate simply, with small incision and low complication.