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
Summary
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.
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