right aortic arch (RAA), an elongated aortic arch compresses the distal trachea The frontal chest radiograph (A) shows an RAA The ascending aorta occupies a far leftward position in the upper mediastinum Magnetic resonance image in axial transverse plane (B) shows posterior displacement and narrowing of the distal trachea Note that the RAA is unusually elongated as it connects the far leftward ascending aorta to the descending aorta on the right side SCV, Superior caval vein Absent pulmonary valve syndrome, also associated with bronchial compression, is characterized by severe dilation of the pulmonary arteries in the mediastinum and the hilums of the lungs (Fig 47.31).154,155 It is most typically associated with tetralogy of Fallot, and in most cases the arterial duct is absent Most patients present early in life with severe respiratory distress due to the bronchial compression and tracheobronchomalacia The lungs are typically hyperinflated, with or without scattered areas of segmental or lobar collapse FIG 47.31 Computed tomograms showing mild narrowing of the right ventricular outflow tract (RVO) by the anteriorly deviated outlet septum (OS), a ventricular septal defect (VSD) (A), and markedly dilated central pulmonary arteries, consistent with tetralogy of Fallot and absent pulmonary valve syndrome The left and right bronchi are compressed by the right and left pulmonary arteries (B) Reformatted images of the airway in the coronal plane (C) show severe narrowing of the left main bronchus, with trapping of air in the left lung LPA, Left pulmonary artery; LV, left ventricle; MPA, main pulmonary artery; RPA, right pulmonary artery; RV, right ventricle As in vascular rings and slings, all these related conditions need a tailored surgical approach and procedure Contrast-enhanced computed tomography is best suited for investigation of critically ill patients, whereas magnetic resonance imaging can be used in older and less symptomatic patients Annotated References Edwards JE Anomalies of the derivatives of the aortic arch system Med Clin North Am 1948;32:925–948 In this seminal paper, the pioneer cardiac pathologist Jesse E Edwards introduced a hypothetical model of the double aortic arch that explains the morphogenesis of most anomalies Dodge-Khatami A, Tulevski II, Hitchcock JF, de Mol BA, Bennink GB Vascular rings and pulmonary arterial sling: from respiratory collapse to surgical cure, with emphasis on judicious imaging in the hi-tech era Cardiol Young 2002;12:96–104 This review paper presents an excellent overview of the morphology, clinical manifestations, diagnosis, and surgical approaches to various vascular rings and the pulmonary arterial sling Backer CL, Mavroudis C, Rigsby CK, Holinger LD Trends in vascular ring surgery J Thorac Cardiovasc Surg 2005;129:1339–1347 This original paper reviews how diagnostic imaging, operative techniques, and clinical outcomes of vascular rings have evolved in the past 60 years in a large referral center Alsenaidi K, Gurofsky R, Karamlou T, Williams WG, McCrindle BW Management and outcomes of double aortic arch in 81 patients Pediatrics 2006;118:e1336–e1341 This original paper reviews the clinical manifestations, associated cardiac and noncardiac anomalies, operative techniques, and outcomes in a large cohort of patients with double aortic arch Yoo SJ, Min JY, Lee YH, et al Fetal sonographic diagnosis of aortic arch anomalies Ultrasound Obstet Gynecol 2003;22:535–546 Patel CR, Lane JR, Spector ML, Smith PC Fetal echocardiographic diagnosis of vascular rings J Ultrasound Med 2006;25:251–257 These two papers illustrate the fetal echocardiographic findings of various anomalies involving the aortic arches In the first paper, fetal and postnatal sonographic findings are correlated with morphogenetic mechanisms Murdison KA, Andrews BA, Chin AJ Ultrasonographic display of complex vascular rings J Am Coll Cardiol 1990;5:1645–1653 This original paper illustrates how a suprasternal sweep can assist in echocardiographic evaluation ... Alsenaidi K, Gurofsky R, Karamlou T, Williams WG, McCrindle BW Management and outcomes of double aortic arch in 81 patients Pediatrics 2006;118:e1336–e1341 This original paper reviews the clinical manifestations, associated cardiac and