Johnston TA, Farra H.
Double Lumen Aortic Arch in Association with Tetralogy of Fallot.
Images Paediatr Cardiol 2006;26:5-7
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| Division of Pediatric Cardiology, Department of Pediatrics, University
of Washington Children’s Hospital & Regional Medical Center, Seattle,
Washington. |
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MeSH
Aorta, Heart defects, congenital, Tetralogy of Fallot
The patient, an 11-month-old girl born with Cornelia de Lange syndrome
and tetralogy of Fallot, underwent placement of a right modified Blalock-Taussig
shunt as a neonate. She had done well clinically with acceptable oxygen
saturations. Echocardiography suggested an abnormality of the aortic arch.
She underwent cardiac catheterization to address branch pulmonary artery
stenosis prior to complete repair. Angiography demonstrated the rare finding
of a double lumen left aortic arch.
The double lumen is felt to arise from persistence of the embryologic
fifth aortic arch.1 The fifth arch is situated inferior to the
true aortic arch. The brachiocephalic arteries arise from the true aortic
arch. This may be an isolated abnormality and have no clinical significance.
This case illustrates an association with tetralogy of Fallot. It also
has been described in association with coarctation of the aorta.
Figure 1: Color Doppler image echocardiographic image from the
suprasternal notch demonstrates the separation of flow into the double
lumens. Two separate color signals represent the parallel lumens with the
true arch superior (arrow) to the persistent fifth arch (asterisk).
Figure 2: Echocardiogram image from the suprasternal notch demonstrates
the double lumen aorta. The superior arch (arrow) gives rise to the head
and neck arteries. The persistent fifth arch (asterisk) is directly inferior
to the true aortic arch.
Figure 3: Lateral projection of the left ventricular angiogram
demonstrates the double lumen left aortic arch. The persistent fifth ach
(arrow) runs inferior and parallel to the true aortic arch (*). The pulmonary
artery fills from the right modified Blalock-Taussig shunt.
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References
Van Pragh R, Van Pragh S. Persistent fifth arterial arch in man. Congenital
double lumen aortic arch. Am J Cardiol 1969;24:279-282.
Culham JAG, Reed MH. Persistent fifth aortic arch with coarctation of the
aorta. Cardiovasc Intervent Radiol 1985;8:137-139.
Contact information
Troy Alan Johnston,
M.D.
Children’s Hospital & Regional Medical Center
4800 Sand Point Way, N.E.
P.O. Box 5371/4G-1
Seattle, Washington 98105-0371
Phone: 206 987-2015
Fax: 206 987-3839
Troy.Johnston@seattlechildrens.org
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