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

Eur J Cardiothorac Surg. 2022 May 27;61(6):1260-1268. doi: 10.1093/ejcts/ezab527.


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.

Keywords: Cardiac surgery; Congenital heart disease; Double aortic arch; Prenatal diagnosis; Right aortic arch; vascular ring.

MeSH terms

  • Aorta, Thoracic / diagnostic imaging
  • Aorta, Thoracic / surgery
  • Aortic Arch Syndromes* / diagnosis
  • Child
  • Female
  • Humans
  • Infant
  • Pregnancy
  • Prenatal Diagnosis
  • Retrospective Studies
  • Treatment Outcome
  • Ultrasonography, Prenatal
  • Vascular Ring* / diagnosis