Outcomes of surgical repair of congenital vascular rings in children.

OBJECTIVES

Vascular rings are rare anomalies of congenital cardiovascular. This study aimed to assess the outcome of surgical repair of congenital vascular rings in children.

METHODS

This retrospective study reviewed data from 237 patients with congenital vascular rings who underwent surgical division from January 2008 to October 2022.

RESULTS

The mean age of patients was 12 months (Interquartile range, IQR, 6, 32 months) and their mean weight was 9.5 kg (IQR, 8, 14 kg). The data revealed that 153 patients (65%) were boys. The complete vascular rings consisted of a Double aortic arch (DAA, 33%), Right aortic arch (RAA) with left ligamentum arteriosum (LLA, 39%), and circumflex aorta (0.8%). The incomplete vascular rings consisted of a pulmonary artery sling (PAS, 26%), innominate artery compression syndrome (0.4%), and Left aortic arch (LAA) with Aberrant right subclavian artery (ARSCA, 0.8%). There were 3 hospital deaths (1.3%) and only one late death during the study. The overall mortality was 1.7% (4 patients). Eighteen patients underwent reintervention for complications. The overall survival rates at 1, 5, and 10 years were 98.3%, 98.3%, and 98.3%, respectively. The rate of freedom from residual symptoms at 1, 5, and 10 years was 97%, 91.3%, and 86.2%, respectively.

CONCLUSION

The results show that surgical repair of congenital vascular rings in pediatric patients yields excellent outcomes: most patients' symptoms improved and only a small proportion required reintervention. Although the pulmonary artery sling forms an incomplete vascular ring, it is associated with a higher incidence of tracheal stenosis and an increased risk of persistent postoperative respiratory symptoms. These observations suggest that more proactive intraoperative management of tracheal stenosis should be considered during the primary operation.