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Tex Heart Inst J. 2000; 27(4): 398–400.
PMCID: PMC101111

Double Aortic Arch in D-Transposition of the Great Arteries: Confirmation of Dominant Arch by Magnetic Resonance Imaging

Colin J. McMahon, MB, BAO, BCh, MRCPI, Louis I. Bezold, MD, and Giles W. Vick, III, MD, PhD

Abstract

We report the case of a female infant who had the rare combination of double aortic arch and D-transposition of the great arteries. The double aortic arch was diagnosed at the time of her arterial switch procedure. Magnetic resonance imaging identified the left arch as dominant, and the right arch was successfully ligated.

Key words: Double aortic arch, heart defects, congenital/diagnosis, magnetic resonance imaging, transposition of great vessels

The occurrence of D-transposition of the great arteries (D-TGA) in association with double aortic arch is exceptionally rare. We report a case of double aortic arch in association with D-TGA, which was diagnosed at the time of the arterial switch procedure. Magnetic resonance imaging enabled identification of the dominant arch prior to successful ligation of the non-dominant arch.

Case Report

A prenatal diagnosis of D-transposition of the great arteries (D-TGA), ventricular septal defect, and right aortic arch was made at 28 weeks' gestation. The female infant was born in April 1999 at 36 weeks' gestation and weighed 2.5 kg. Apgar scores were 8 and 9 (-1 color) at 1 and 5 minutes, respectively.

Physical examination revealed a heart rate of 135 bpm, a respiratory rate of 60, and blood pressure (in the right arm) of 65/34 mmHg. Oxygen saturation was 73% in room air; this increased to 82% in 100% inspired oxygen. The pulses were normal, and the apex beat was located in the left 4th intercostal space midclavicular line. There was a right ventricular heave, a narrowly split 2nd heart sound, and a grade 2/6 systolic ejection murmur at the left upper sternal edge. Chest radiography demonstrated levocardia, right aortic arch, and upturned apex; images in the anterior-posterior projection showed a narrow anterior mediastinum. Echocardiography demonstrated atrial situs solitus, atrioventricular concordance, and ventriculo-arterial discordance. The ventricular septum was intact. There was continuous left-to-right shunting through a patent foramen ovale and low-velocity bidirectional shunting through a moderate-size ductus arteriosus. The aortic arch appeared to be right-sided. A bedside balloon atrial septostomy was performed using a 5-F septostomy balloon. Thereafter, oxygen saturation improved to 88% in room air. Prostaglandin was not administered.

On day of life 9, the infant underwent an arterial switch procedure with Lecompte maneuver, closure of the atrial septal defect, and ligation of the ductus arteriosus (Fig. 1). She was found to have a double aortic arch; there was no obvious discrepancy in size between the 2 arches. Ligation of either arch was postponed until the dominant arch could be determined. The patient's postoperative course was complicated by failed extubation and by persistent stridor and tachypnea secondary to upper airway obstruction. Bronchoscopy failed to determine any evidence of airway obstruction. Magnetic resonance imaging (MRI) of the aortic arches showed a dominant left aortic arch and tracheal compression (Figs. 2–4). Each arch gave rise to individual ipsilateral carotid and subclavian arteries (Fig. 5). The patient was returned to the operating room at 5 weeks of age for ligation of her right aortic arch. She was discharged home 1 week after this procedure. She had persistent mild stridor in the postoperative period. This was substantially improved at the most recent follow-up examination, 12 months after surgery.

figure 15FF1
Fig. 1 Axial cine gradient echo image at the level of the pulmonary arteries. Note the position of the main pulmonary artery (MPA) anterior to the ascending aorta (Asc Ao), subsequent to arterial switch surgery for transposition of the great arteries. ...
figure 15FF5
Fig. 5 Axial cine gradient echo image above the level of the aortic arches. Locations of the right common carotid artery (RCCA), right subclavian artery (RSCA), left common carotid artery (LCCA), and left subclavian artery (LSCA) are as noted.

Discussion

The combined finding of D-TGA and double aortic arch is something of an embryologic curiosity. To the best of our knowledge, only 1 patient has previously been described with double aortic arch and bilateral patent ducti arteriosi associated with transposition of the great arteries, lending support to Edwards's hypothesis of aortic arch development. 1 There are very few reports of double aortic arch in association with transposition of the great arteries: 3 cases described in living patients, 2–4 and 1 diagnosis made postmortem. 5

Transposition of the great arteries is the 2nd most common cardiac lesion presenting in infancy. Approximately 10% of cases have an associated aortic arch anomaly, most often coarctation, 6 interruption, 7 or right aortic arch. 8 Double aortic arch was 1st described by Hommel in 1737. 9 The presenting symptoms are typically secondary to tracheal compression and upper airway obstruction. Associated intracardiac malformations, although rare, are usually conotruncal malformations—typically tetralogy of Fallot (11 of 16 patients in one study). 5 Double aortic arch is treated surgically by ligation of the smaller arch (usually the left), distal to the subclavian artery, through a posterolateral thoracotomy. 3

Determination of the dominant arch is vital prior to surgical ligation. Useful diagnostic techniques include echocardiography, angiography, and, more recently, MRI. If examination by 1 of these techniques leaves any doubt concerning the dominant arch, further evaluation is mandatory. Cases of coarctation have been reported to involve 1 10 or both aortic arches 11 prior to ligation. Erroneous ligation of the opposite arch in a patient with coarctation may have catastrophic results. In patients whose arch dominance is equivocal, such as ours, MRI provides excellent definition of the arch anatomy.

Footnotes

Address for reprints: Colin J. McMahon, MB, BAO, BCh, MRCPI, Division of Pediatric Cardiology, Texas Children's Hospital, 6621 Fannin, MC 2-2280, Houston, TX 77030

References

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