Right Aortic Arch in Vascular Ring Defects Clinical Presentation

Updated: Jan 10, 2020
  • Author: Doff B McElhinney, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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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:

  • 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.

  • 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.

  • Occasionally, patients may reach older childhood or adulthood before developing persistent or progressive symptoms of dysphagia or respiratory symptoms or both.

  • Vascular rings may be diagnosed prenatally based on fetal ultrasonography echocardiography findings. [7, 8, 9, 10]

  • In newborn infants with associated cardiac or noncardiac anomalies, the ring may be diagnosed incidentally during the course of evaluation for the other anomalies.

  • 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.