Vascular ring: a commonly overlooked cause of neonatal respiratory distressVascular ring: a commonly overlooked cause of neonatal respiratory distress

Abstract

Objective: To report a case of a newborn with complete vascular ring caused by the right-sided aortic arch with retroesophageal left subclavian artery and Kommerell diverticulum. Case Report: A preterm newborn female (36 weeks 3 days) with low birth weight (1670 g) was delivered by cesarean section, with Apgar scores of 7/8. Physical examination revealed dyspnea, cyanosis, and inspiratory stridor with systolic murmur (2/6 grade) at the left upper sternal border. She was transferred to the neonatal intensive care unit, with improvement in her cyanosis and respiratory distress, immediately after orotracheal intubation. Chest X-ray demonstrated mediastinal enlargement with a right-sided aortic arch. The transthoracic echocardiogram showed situs solitus, levocardia, foramen ovale, tortuous and right-sided aortic arch, enlarged ascending aorta, large patent ductus arteriosus (PDA), left-to-right shunt at atrial septal and PDA levels, and Doppler signs of right pulmonary artery flow obstruction probably caused by vascular compression. Angiotomography confirmed the vascular ring and detailed his obstruction anatomy: the right-sided aortic arch with the retroesophageal left subclavian artery arising from the Kommerell diverticulum and left-sided PDA. The anomalous left subclavian artery caused the tracheo-esophageal, right pulmonary artery, and right bronchus compression. The left-sided large PDA completed the vascular ring and its combination caused early symptoms. Conclusion: Vascular ring it’s a congenital malformation that should be suspected in all patients associated with aortic arch anomaly presenting with respiratory symptoms (dyspnea/inspiratory stridor and/or chronic sibilance).