Don’t Forget the Head and Neck Vessels

Abstract

An 8-week-old baby girl was diagnosed with aortopulmonary (AP) window after presenting to her local hospital with failure to thrive and respiratory distress. Echocardiogram also identified a right-sided aortic arch. Unfortunately, there was incomplete imaging of the arch branching pattern prior to surgery. Her early post-operative recovery period was complicated by two cardiac arrests, she remained in hospital for a long time, and after discharge continued to fail to thrive with frequent respiratory infections. Further investigations revealed a right-sided aortic arch with aberrant left subclavian artery forming a vascular ring. A vascular ring is a rare congenital anomaly of the great vessels, and the severity of symptoms is related to the degree of compression of the trachea and oesophagus. Some varieties of vascular ring are highly associated with other congenital cardiac defects. Currently there is no gold standard diagnostic imaging for vascular ring; however, computed tomography angiography (CTA) and cardiac magnetic resonance imaging (MRI) are superseding echocardiography because of their ability to provide detailed anatomy and relationship to other structures. A symptomatic untreated vascular ring carries significant risk of mortality and diagnosis is often delayed due to attribution of the respiratory symptoms to more common paediatric disorders.