Congenital Disease of the Aortic Arch: Coarctation and Arch Anomalies

  • Jeffrey C. Hellinger
  • Luisa F. Cervantes
  • L. Santiago Medina

Key Points


■ Evaluation of upper versus lower extremity blood pressure is an important diagnostic tool in the evaluation of coarctation. Systolic blood pressure of upper extremities greater than that of lower extremities merits further diagnostic workup (limited to moderate evidence).

■ Associated cardiovascular anomalies are common in patients with coarctation and impact the presenting clinical symptoms, the age of clinical presentation, and the clinical outcome (moderate evidence).

■ In newborns and infants with appropriate acoustic window, echocardiogram is the study of choice with sensitivity >90% (limited to moderate evidence).

■ In older children, contrast-enhanced MR angiography (MRA) is superior to transthoracic echocardiography and other MR imaging techniques for the diagnosis of congenital coarctation and obstructive aortic arch anomalies (moderate to strong evidence).

■ CT angiography (CTA) offers comparative performance to MRA and can be used for the diagnosis and surgical planning when MRA is not available or is contraindicated (limited evidence).

■ Conventional angiography is no longer routinely used for diagnosing coarctation but is reserved for endovascular treatment with balloon angioplasty or stent placement (limited evidence).

Aortic Arch Anomalies: Vascular rings and Pulmonary Slings

■ The clinical presentation of a symptomatic vascular ring or a pulmonary sling may result from tracheal compression, esophageal compression, or both. Symptoms and signs are typically respiratory in infants and children, whereas they are esophageal in adults (limited to moderate evidence).

■ Associated cardiac abnormalities occur in up to one-third of patients with aortic arch anomalies (moderate evidence).

■ Preoperative evaluation of aortic arch anomalies should also address the presence of tracheomalacia. Unrecognized tracheomalacia can lead to negative surgical outcome and persistent symptoms.

■ MRI–MRA and CTA have the highest diagnostic performance (sensitivity and specificity) followed closely by the esophagram in evaluating aortic arch vascular rings (limited to moderate evidence).

■ Esophagram can readily detect vascular ring but has lower sensitivity for pulmonary sling (limited evidence).

■ Conventional angiography is no longer routinely used for the diagnosis of vascular rings (limited evidence).


Aortic Arch Vascular Ring Tracheal Ring Moderate Evidence Interrupted Aortic Arch 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Jeffrey C. Hellinger
    • 1
  • Luisa F. Cervantes
    • 2
  • L. Santiago Medina
    • 3
    • 2
    • 4
  1. 1.Department of RadiologyChildren’s Hospital of Philadelphia, University of Pennsylvania School of MedicinePhiladelphiaUSA
  2. 2.Department of RadiologyMiami Children’s HospitalMiamiUSA
  3. 3.Division of Neuroradiology and Brain ImagingDirector of the Health Outcomes, Policy, and Economics (HOPE) CenterMiamiUSA
  4. 4.Harvard Medical SchoolBostonUSA

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