Abstract:

Objective To evaluate the clinical characteristics, diagnosis and prognosis of congenital vascular ring(CVR) in children.Methods The CVR parting ,clinical data,imaging examinations and treatment of 99 cases with CVR in Children′s Hospital of Chongqing Medical University from January 2006 to December 2015 were retrospectively analyzed.According to the prognosis, patients were divided into survival group and death or giving up the treatment groups, the risk factors of poor prognosis were analyzed. Results A total of 99 cases diagnosed as CVR were enrolled,including 55 males and 44 females, with median age of 7.2 months.The diagnosis included pulmonary artery sling in 51 cases,double aorta arch in 32 cases,right aortic arch with left ductus arteriosus in 15 cases,left aoaic arch with vagus right subclavian artery in 1 case. Only 51 cases (51.5%) had respiratory system performance, 8 cases (8.1%) had cardiovascular system performance, 2 cases (2.0%) had digestive system performance, 3 cases (33.3%) had breathing combined cardiovascular system performance 3, 5 patients without clinical symptoms. Merge heart malformations were found in 64 cases (64.6%), of them 31 cases with ASD, 2 cases with VSD, 9 cases with ASD + VSD and 22 cases with complex cardiac anomalies. Tracheobronchial stenosis was found in 69 cases, esophageal stenosis in 14 cases, 10 cases with laryngeal cartilage dysplasia, 9 cases with congenital bronchial pulmonary hypoplasia.The diagnosis accuracy of echocardiographic was 57.4% and that of computed tomography angiography was 91.6%,26 cases checked with the fiber bronchoscope were found airway stenosis; 14/17 (82.3%) accepting line barium esophageal imaging examination were found esophageal pressure distortion and narrow. Fifty-seven cases were treated with surgical operation, of them 4 cases died, six cases with difficult postoperative offline gave up treatment. 42 cases with non-surgical treatment, 1 case died and 10 cases with difficult offline gave up treatment. Age and proportion of the pulmonary artery sling with pulmonary dysplasia of dead or abandoned children group was significantly higher than those of survival group.Conclusion Eehocardiography combined with CTA is the best way to diagnose CVR.Type of CVR,age and the combining pulmonary hypoplasia are related to prognosis.