The patient, male, 1 year, was admitted to our hospital with cardiac murmur. Cardiac ultrasonography showed "complete atrioventricular septal defect (C-AVSD), secondary orifice atrial septal defect (ASD), patent ductus arteriosus (PDA), left superior vena cava, and pulmonary hypertension". The patient got follow-up at the age of 3, 6, 9 months and 1 year, with no feeding difficulties, no obvious underdevelopment and no history of repeated respiratory infections. Cardiac ultrasonography showed that the ventricular septal defect (VSD) healed spontaneously at 9 months of age. At 1 year of age, he was admitted to the hospital with "partial atrioventricular septal defect (P-AVSD)" and accepted surgery. Intraoperative exploration showed that the primary orifice ASD was 12 mm, the atrioventricular valve was divided into two groups, and the left atrioventricular valve had three leaflets: anterior, posterior, and lateral one. A cleft was between the anterior and posterior leaflets. The annulus was not enlarged with diameter of 13 mm. The right atrioventricular valve developed well, with fibrous hyperplasia and adhesion under the septal valve. No VSD was seen. The cleft was sutured intermittently. Autologous pericardial patch was used to repair the primary orifice ASD, and the coronary sinus was separated into the right atrium. Self-healing of VSD patients with C-AVSD is very rare, suggesting that patients with C-AVSD with normal range of development, and without obvious clinical symptoms and secondary damage, should be followed up and accept elective surgery in clinical practice.
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 and were followed up to now. 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 used 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. One-stage surgical repair of Berry syndrome is a high-risk and complicated operation, but it is safe in an experienced heart center.