A case of pulmonary artery sling, unilateral lung hypoplasia, and congenital heart disease

Abstract

Vascular rings are rare congenital anomalies that primarily result from an embryological derangement of the paired aortic arches or branching pulmonary arteries. They make up <1% of all cardiac defects. Double aortic arch and right-sided aortic arch with the left ligamentum arteriosum are the most common types, making up 85– 95% of cases, with pulmonary artery slings making up about 10% of cases. The prevalence is estimated as 59 per million children with very few cases reported worldwide. The clinical presentation is variable and this includes severe acute life-threatening episodes, recurrent apnea, severe respiratory distress, feeding difficulties persistent cough, wheezing, or stridor. In some cases, affected individuals may be completely asymptomatic. A high index of suspicion is needed to suspect and diagnose vascular rings as they are uncommon but surgically treatable. We present a case of a 5-day-old term neonate referred to our facility with respiratory distress associated with stridor which started 12 h post-delivery.