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Aortic coarctation in an infant with tetralogy and pulmonary atresia

Published online by Cambridge University Press:  18 November 2005

Robert M. Freedom,
Lee N. Benson and
Haverj Mikailian
Robert M. Freedom
Affiliation:
Division of Cardiology, Department of Paediatrics, University of Toronto, Toronto, Ontario, CanadaDepartment of Diagnostic Imaging, The Hospital for Sick Children and Departments of Medical Imaging and Paediatrics, University of Toronto, Toronto, Ontario, Canada
Lee N. Benson
Affiliation:
Division of Cardiology, Department of Paediatrics, University of Toronto, Toronto, Ontario, Canada
Haverj Mikailian
Affiliation:
Department of Diagnostic Imaging, The Hospital for Sick Children and Departments of Medical Imaging and Paediatrics, University of Toronto, Toronto, Ontario, Canada
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Abstract

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Information

Type
Images in Congenital Heart Disease
Information
Cardiology in the Young , Volume 15 , Issue 6 , December 2005, pp. 667 - 668
Copyright
© 2005 Cambridge University Press

This three-month-old infant presented with cyanosis and a soft continuous murmur over the left chest. Cross-sectional echocardiography suggested tetralogy with pulmonary atresia. The heart was left-sided, normal atrial arrangement, concordant atrioventricular connections, and a single outlet via the aorta. Cardiac angiography (Fig. 1), performed in the morphologically right ventricle (RV) via the inferior caval vein (ICV) and visualized in the frontal (a) and lateral (b) projections, revealed the presence of a blind infundibulum (INF), a dilated overriding aorta (AO), a left aortic arch, the outlet septum (OS), and the right atrium (RA).

Figure 1.

Injection of contrast into a tortuous narrowed (long arrow) collateral vessel (C) originating from the left subclavian artery (Fig. 2), as seen in frontal projection, demonstrated the vascular supply to the entire left lung (LPA) and some segments to the lower lobe of the right lung (RLLPA), also showing proximal narrowing of the collateral artery (arrowhead). Further injection of contrast into the transverse aortic arch (TAA), as seen in lateral projection (Fig. 3), revealed coarctation of the aorta (arrow) just distal to the origin of the left subclavian artery. At this site, there was a gradient of 22 millimetres of mercury to the descending thoracic aorta (DAO).

Figure 2.

Figure 3.

We interpret this rare combination of congenital heart defects as an attempt by nature to enhance the flow of blood to the lungs. Interestingly, before the era of unifocalization, banding of the aorta was once suggested1 as a possible therapeutic manoeuvre in this difficult situation.

References

Iida J, Lin E. Experimental and clinical studies on “aorta banding” for severe cyanotic heart disease. Jpn Heart J 1970; 11: 130–140.Google Scholar