Objective To investigate diagnostic and treatment strategies of patients with congenital heart diseases and right aortic arch. Methods Clinical data of 27 children who underwent surgical correction for congenital heartdiseases and right aortic arch in Children’s Heart Center of Beijing Children’s Hospital from January 1，2012 to April 1，2013 were retrospectively analyzed. There were 20 male and 7 female patients with their age of 10.96±12.08 months and body weight of 7.70±3.13 kg. All the patients had right aortic arch，including 14 children with tetralogy of Fallot，9 childrenwith ventricular septal defect，1 child with patent ductus arteriosus，1 child with pulmonary artery sling and 2 children with isolated double aortic arch. Thirteen children were found to have a vascular ring including aberrant left subclavian artery and double aortic arch. All the patients received surgical correction for their intracardiac anomalies and concomitant transposition of the left subclavian artery for aberrant left subclavian artery or division of the nondominal arch for double aortic arch. Results Three children died perioperatively including 1 child who was unable to be weaned from cardiopulmonary bypass，1 child without vascular ring who died 7 days postoperatively，and 1 child with double aortic arch who died of acuterespiratory distress syndrome. There was another child who gave up further postoperative treatment. Twenty-three children were followed up for 3-17 months after discharge. Echocardiography showed satisfactory correction of their intracardiac anomalies. All the patients alive recovered well with good pulses in arteries of both arms. Conclusion Careful strategiesare needed for surgical correction of patients with congenital heart diseases and concomitant right aortic arch. Comprehensivepreoperative evaluation including contrast-enhanced CT and magnetic resonance imaging is helpful for clinical decision making in diagnosis and treatment. Clinical outcomes of 1-stage surgical correction of vascular ring and other concomitant intracardiac anomalies are satisfactory with good short-term results.
ObjectiveTo investigate the best anatomical classification, surgical timing, procedure and clinical outcomes of congenital vascular ring.MethodsThe clinical data of 58 patients who underwent congenital vascular ring surgery in Pediatric Surgery Center, Fuwai Hospital between 2014 and 2019 were retrospectively analyzed. There were 32 (55.2%) males and 26 (44.8%) females with a median age of 16.5 (2-73) months. Preoperative symptoms, imaging examinations, anatomical classifications, surgical procedures and postoperative recovery were assessed.ResultsThere were 20 (34.5%) patients of double aortic arch, 22 (37.9%) patients of right aortic arch with left arterial duct or ligament, 15 (25.9%) patients of left aortic arch with aberrant right subclavian artery, and 1 (1.7%) patient of circumflex aorta with cervical aorta arch. The median ventilator supporting time was 6.0 (0-648) h, and the median hospital stay time was 14.5 (7-104) d. One patient with coarctation of aorta died of severe pulmonary infection during perioperative period, and the others survived without symptoms and reoperation after discharge. The median follow-up time was 7.0 (1-62) months.ConclusionFor children with unexplained dyspnea and dysphagia, or with right aortic arch, preoperative imaging examinations such as computed tomography or magnetic resonance imaging are required to confirm the diagnosis of vascular ring. Surgical correction of congenital vascular ring is safe and reliable, and can effectively relieve symptoms. The mortality rate and reoperation rate are low, and the follow-up results are satisfactory.