Contemporary surgical outcome and symptomatic relief following vascular ring surgery in children: effect of prenatal diagnosis

OBJECTIVES Our goal was to describe postoperative complications and outcomes in a large contemporary cohort of children with an isolated double aortic arch (DAA) or a right aortic arch (RAA) with left arterial ligament and to assess the impact of foetal diagnosis on outcomes. METHODS We performed a retrospective analysis of all patients who underwent surgery for DAA or RAA with left arterial ligament between 2005 and 2019. RESULTS A total of 132 children were operated on for a DAA (n = 77) or a RAA (n = 55). Prenatal diagnosis was made in 100/132 (75.8%). Median age at surgery for DAA was 5.0 (1.7-13.3) months and for RAA was 13.9 (6.4-20.1) months. There was no difference in the age at surgery between the prenatal and postnatal cases (8.6 [4.0-15.6] vs 5.4 months [1.8-17.7]; P = 0.37). No surgical deaths occurred. Vocal cord palsy was the most common complication, occurring in 12/132 (9%): of these, 11 resolved spontaneously and 1 required a temporary tracheostomy. Logistic regression demonstrated that older age at operation was the only predictor for a postoperative complication (P = 0.02). Overall, 21/67 (31%) of prenatally detected, symptomatic cases reported residual symptoms/signs 1 year after surgery compared to 18/28 (64%) of postnatally detected cases. Postnatal diagnosis was associated with persistent postoperative symptoms/signs [P = 0.006, odds ratio = 3.9 (95% confidence interval 1.5-9.4)]. CONCLUSIONS Surgery to relieve a vascular ring resolves trache-oesophageal compressive symptoms in most cases, but parents/patients should be aware that symptoms/signs may persist in the first postoperative year despite effective release of the vascular ring. Earlier surgery and prenatal diagnosis may improve outcomes.

Automated summary

This study aimed to describe the postoperative complications and outcomes in children with isolated double aortic arch or right aortic arch with left arterial ligament and assess the impact of fetal diagnosis on outcomes. The most common complication was vocal cord palsy, and older age at surgery was the only predictor for postoperative complications. Prenatal diagnosis was associated with a lower rate of postoperative symptoms.