A Severe Form of Arterial Tortuosity Syndrome Presenting With Significant Airway Obstruction in an Infant. Issue 2 (March 2020)

• Record Type: Journal Article
• Authors: Dodge-Khatami, Jannika; Simpson, Scott A.; Dodge-Khatami, Ali
• Is Part Of: World journal for pediatric & congenital heart surgery. Volume 11:Issue 2(2020)
• Page Start: 238
• Page End: 240
• Publication Date: 2020-03
• Subjects: Pediatric cardiology -- Periodicals; Congenital heart disease in children -- Periodicals; Heart -- Abnormalities -- Surgery -- Periodicals; Heart -- Surgery -- Periodicals; Heart Defects, Congenital -- surgery -- Periodicals; Cardiac Surgical Procedures -- Periodicals; Child -- Periodicals; Adult -- Periodicals; 618.9212
• DOI: 10.1177/2150135119829009
• Languages: English
• ISSNs: 2150-1351

Abstract

We describe a severe form of arterial tortuosity syndrome in a newborn, in which the tortuous course of the aorta masqueraded as a pulmonary artery sling on fetal echocardiogram. The newborn presented with respiratory distress after birth. The clinical course was complicated by extrinsic airway obstruction requiring cardiopulmonary resuscitation. Timely diagnostic work-up in patients with arterial tortuosity syndrome is necessary to plan eventual intervention, and hopefully to prevent complications related to the abnormal vasculature.