History
The history of patients with right aortic arch and vascular ring depends on several factors, including the severity of tracheal compression, esophageal compression, or both and whether associated anomalies are present. Note the following:
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Among patients with a vascular ring, those with right aortic arch tend to present slightly later than those with a double aortic arch, but this is a minor difference and is not a reliable distinguishing feature. The classic history of a patient with a vascular ring is noisy breathing noted by the parents during the first few weeks of life.
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Young patients may have experienced episodes often referred to as apparent life-threatening events (ALTE) or "death spells," in which they experience acute apneic or severe obstructive events accompanied by cyanosis. Patients with less severe tracheal compression may provide a history of persistent respiratory symptoms without frank stridor, often treated as asthma or bronchiolitis, or they may present with recurrent lower respiratory infections. Esophageal symptoms include emesis, choking, or dysphagia and are more common in older infants and children than in young infants following the introduction of solid foods.
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Occasionally, patients may reach older childhood or adulthood before developing persistent or progressive symptoms of dysphagia or respiratory symptoms or both.
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In newborn infants with associated cardiac or noncardiac anomalies, the ring may be diagnosed incidentally during the course of evaluation for the other anomalies.
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Abnormalities of aortic arch sidedness and branching are common in patients with a chromosome 22q11 deletion. [11] A substantial proportion of individuals diagnosed with a chromosome 22q11 deletion beyond age 6 months have been found to have aortic arch anomalies, including clinically occult vascular rings.
Physical Examination
Physical examination findings can vary, often in accordance with the historic presentation.
Newborns with associated anomalies may have no evidence of a vascular ring upon physical examination, but this situation is the exception because most patients have readily recognizable physical signs.
The classic sign of vascular rings in general is nonpositional stridor; however, many young infants with a ring have adventitious expiratory breath sounds as well as the characteristic inspiratory stridor. The respiratory findings generally do not improve with nebulized bronchodilator therapy and are usually more prominent with agitation or crying.
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Left: Schematic diagram of the primitive pharyngeal arch system showing the left (L) and right (R) external carotid (EC) and internal carotid (IC) arteries, the fourth (IV) and sixth (VI) pharyngeal arches, distal pulmonary arterial segments (PA), dorsal aortas (DA), and seventh intersegmental arteries (VII). The proximal (p) sixth arches develop into the proximal pulmonary arteries, and the distal (d) sixth arches become the arterial ducts. The seventh intersegmental arteries develop into the subclavian arteries. Right: Schematic diagram depicting the segments of the pharyngeal arch system that regress (shown in black) in order for normal development of the great arteries and their thoracic branches (common carotid artery [CCA], left pulmonary artery [LPA], ductus arteriosus [PDA], right pulmonary artery [RPA], subclavian artery [SCA]).
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Left: Schematic diagram depicting the segments of the pharyngeal arch system that regress (shown in black) in order for the development of a right aortic arch with aberrant left subclavian artery. Abbreviations are as in the first image. Right: Mature anatomy of a vascular ring formed by a right aortic arch with an aberrant left subclavian artery arising from a retroesophageal diverticulum with a left-sided ligamentum arteriosum to the left pulmonary artery.
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Left: Schematic diagram depicting the segments of the pharyngeal arch system that regress (shown in black) in order for the development of a right aortic arch with mirror-image branching of the brachiocephalic vessels and a left-sided ductus arteriosus from the descending aorta to the left pulmonary artery. Abbreviations are as in the first image. Right: Mature anatomy of a vascular ring formed by a right aortic arch with mirror-image branching of the brachiocephalic vessels and a left-sided ductus arteriosus from the descending aorta to the left pulmonary artery.
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Left: Schematic diagram depicting the segments of the pharyngeal arch system that regress (shown in black) in order for the development of a right aortic arch with aberrant retroesophageal left innominate artery. Abbreviations are as in the first image. Right: Mature anatomy of a vascular ring formed by a right aortic arch with an aberrant retroesophageal left innominate artery with a left-sided ligamentum arteriosum to the left pulmonary artery.
