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Right aortic arch and coarctation: a rare association

Fraz A Ismat1, Paul M Weinberg, Jack Rychik

  • 1Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA. ismat@email.chop.edu

Congenital Heart Disease
|March 3, 2007
PubMed

Insights

Coarctation of the aorta is rare in patients with a right aortic arch, often presenting with long-segment hypoplasia and other congenital anomalies. Noninvasive imaging can diagnose this condition, which is frequently repaired with patch angioplasty.

Area of Science:

  • Cardiovascular Surgery
  • Pediatric Cardiology
  • Congenital Heart Disease

Background:

  • Coarctation of the aorta is a rare condition in individuals with a right aortic arch.
  • Previous studies have not extensively detailed the anatomical and clinical associations of this rare presentation.

Purpose of the Study:

  • To understand the anatomical and clinical correlates of coarctation in patients with a right aortic arch.
  • To review a single institutional experience with associated diagnoses, diagnostic methods, and surgical interventions.

Main Methods:

  • Retrospective review of echocardiography, magnetic resonance imaging, catheterization, and surgical databases.
  • Study period spanned from 1988 to 2001.

Main Results:

  • Out of 240 patients with a right aortic arch, 10 (4.1%) had coarctation.
  • Most cases (90%) exhibited long-segment hypoplasia, frequently associated with aberrant left subclavian artery (60%) and vascular rings (60%).
  • Noninvasive imaging (MRI/echocardiography) successfully diagnosed all cases; most were repaired with patch angioplasty.

Conclusions:

  • Coarctation with a right aortic arch is a rare congenital heart defect, distinct from left aortic arch coarctation.
  • Long-segment hypoplasia without bicuspid aortic valve is characteristic, often occurring within broader syndrome complexes.
  • Noninvasive diagnosis is feasible, and surgical repair typically involves patch angioplasty.
Abstract

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