Right aortic arch and coarctation: a rare association.
OBJECTIVE
Understand anatomical and clinical correlatives to coarctation in right aortic arch.


BACKGROUND
Coarctation of the aorta is rare in patients with a functional right aortic arch. We reviewed a single institutional experience, examining associated diagnoses, diagnostic methodology, and surgical approaches.


METHODS
A retrospective study was performed of our echocardiographic, magnetic resonance imaging, catheterization, and surgical databases from 1988 to 2001.


RESULTS
Of 240 patients with right aortic arch, 10 (4.1%) had coarctation, constituting 1.9% of all native coarctations (n = 524). Nine (90%) had long-segment hypoplasia. Six (60%) had an aberrant left subclavian artery or retroesophageal diverticulum, 3 (30%) had mirror image branching, and 1 (10%) had a double arch with an atretic left arch. Other congenital heart defects were seen in 6 (60%) comprising 3 with ventricular septal defects, and one each with double-outlet right ventricle, cor triatriatum, and pulmonary valve abnormality. No patients with long-segment hypoplasia had bicuspid aortic valve. Six (60%) had vascular rings, and 5 (50%) had other associated syndromes. Magnetic resonance imaging and/or echocardiography successfully diagnosed all of these patients. Although long-segment right aortic arch coarctation courses behind the trachea posteriorly, only 2 needed an extra-anatomic (jump) graft; the remainders were repaired with patch angioplasty.


CONCLUSIONS
Coarctation with right aortic arch is rare, constituting 4.1% of all patients with right aortic arch, compared with 5-8% of patients with left aortic arch and congenital heart disease. Nearly all had long-segment hypoplasia without bicuspid aortic valve, and half were part of other syndrome complexes. This association can be diagnosed noninvasively and can often be repaired by patch angioplasty.
Ismat
Fraz A
FA
Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA. ismat@email.chop.edu
Weinberg
Paul M
PM
Rychik
Jack
J
Karl
Tom R
TR
Fogel
Mark A
MA
D001013:Aorta, Thoracic / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D001017:Aortic Coarctation / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000601:surgery; D006328:Cardiac Catheterization; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D015150:Echocardiography, Doppler; D019560:Echocardiography, Three-Dimensional; D005260:Female; D006331:Heart Diseases / Q000453:epidemiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies