부산대학교 도서관

Background The great majority of patients with complete vascular rings and pulmonary artery sling present before their first birthday. This retrospective report summarises the surgical experience of a single institution with the majority of vascular ring group presenting after infancy and having associated congenital cardiac anomalies. Possible reasons of delayed presentation of vascular ring, the asymptomatic left pulmonary artery sling and the individualised surgical management of each subgroup are discussed. Methods Fifteen consecutive patients, aged 2 months to 9 years (mean+-SD=41.33+-31.55 months) underwent surgical correction of vascular rings and sling during the last 12 years, including one infant. All patients underwent chest roentgenogram, barium esophagogram, two dimensional echocardiogram and cardiac catheterisation. Magnetic resonance imaging was employed in three patients. Four patients with isolated complete vascular ring were symptomatic (group A), one patient with left pulmonary artery sling was asymptomatic (group B) and 10 symptomatic patients with complete vascular rings had associated congenital cardiac anomalies (group C). The operative approach was through a left posterolateral thoracotomy (n=5), a median sternotomy (n=8) and a combination of median sternotomy and left thoracotomy (n=2). Operations of various types were performed. Results The operative mortality was 6.6% and there was no late death. All survivors are doing well and asymptomatic after a mean follow up period of 66.87 months (range=1 month to 143 months). Postoperative barium esophagogram in all survivors revealed no stasis of contrast in the upper esophagus and marked diminution in the degree of esophageal indentations. Conclusions Accuracy in diagnosis is important to determine the optimal surgical approach. Individualization of surgical management provides gratifying results. Asymptomatic left pulmonary artery sling without associated anomalies may be left untreated.