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Abdominal aorta interruption and CHD

Published online by Cambridge University Press:  30 December 2014

Lia Oliveira,
Erica Torres and
Rui Anjos
Lia Oliveira*
Affiliation:
Serviço de Cardiologia Pediátrica, Hospital de Santa Cruz – Centro Hospitalar Lisboa Ocidental, Lisboa, PortugalServiço de Pediatria Médica, Departamento de Pediatria, Hospital de Santa Maria – Centro Hospitalar Lisboa Norte, Centro Académico de Medicina de Lisboa, Lisboa, Portugal
Erica Torres
Affiliation:
Serviço de Cardiologia Pediátrica, Hospital de Santa Cruz – Centro Hospitalar Lisboa Ocidental, Lisboa, PortugalServiço de Pediatria, Centro Hospitalar do Algarve, Faro, Portugal
Rui Anjos
Affiliation:
Serviço de Cardiologia Pediátrica, Hospital de Santa Cruz – Centro Hospitalar Lisboa Ocidental, Lisboa, PortugalServiço de Cardiologia Pediátrica, Hospital de Santa Cruz – Centro Hospitalar Lisboa Ocidental, Lisboa, Portugal
*
Correspondence to: L. C. F. Oliveira, MD, Departamento de Pediatria, Serviço de Pediatria Médica, Hospital de Santa Maria, Avenida Prof. Egas Moniz, 1649-035 Lisbon, Portugal. Tel: +0 035 121 780 5229; Fax: +0 035 121 780 5229; E-mail: lcfoliveira@gmail.com
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Abstract

We report a rare case of CHD and abdominal aorta interruption. Renal hypertension was the first sign of the abdominal aortic malformation. The aetiology of abdominal aortic interruption remains unclear, it could be congenital or acquired.

Keywords

CHDabdominal aorta interruptionrenal hypertension

Information

Type
Images in Congenital Cardiac Disease
Information
Cardiology in the Young , Volume 25 , Issue 5 , June 2015, pp. 992 - 993
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Copyright
© Cambridge University Press 2014 

We present the case of girl with a history of unrestrictive perimembranous ventricular septal defect, severe valvular and subvalvular aortic stenosis, and type B interrupted aortic arch.

The patient underwent Damus-Stensel-Kaye surgery and aortic arch repair as a neonate. The status of the patient after surgery was uneventful. At 30-month-old, the child represented with arterial hypertension. There were no signs of re-coarctation.

A magnetic resonance angiography of the aorta showed a right renal artery stenosis and a dilated sac-like termination below the renal arteries. The abdominal aorta continued through a smaller calibre vessel emerging from its anterior surface (Fig 1), giving origin to both iliac arteries. A CT angiography confirmed this diagnosis (Fig 2) and an angiography was performed, which confirmed the aortic malformation and right renal artery duplication, with diffuse hypoplastic arteries (Fig 3). An isotopic renal scan revealed a decrease in right kidney function (40%). Screening for systemic diseases was negative. Arterial hypertension was controlled pharmacologically and the child remains asymptomatic, and thus surgical bypass has not yet been considered.

Figure 1

Magnetic resonance angiography, posterior view, showing the abdominal aorta terminating in a larger sac-like structure (arrow) and its continuation through a smaller calibre vessel (*). Perfusion of the right kidney is decreased.

Figure 2

CT angio-scan, confirming the diagnosis of abdominal aorta malformation.

Figure 3

Aortography, anterior view, confirmed the aortic malformation, after the origin of the renal arteries, and a collateral vessel (arrow) arising from the anterior surface of the aorta, and from which both common iliac arteries emerged (*). There is duplication and hypoplasia of right renal vessels.

This case is a very uncommon association, and its origin is not clear: congenital or acquired. A dissection/thrombosis of the distal part of the aorta could have been the trigger, as the patient had a femoral arterial line inserted at the time of cardiac surgery. Mid-aortic syndrome, characterised by thoracic or abdominal aortic narrowing or occlusion, and often associated with stenosis of efferent vessels,Reference Levart and Klokocovnik 1 including the renal arteries, could also be a possibility, although this is not a typical presentation.

Acknowledgements

The authors acknowledge the support of the Hospital de Santa Cruz (Centro Hospitalar Lisboa Ocidental, Lisboa) for providing supporting images.

Financial Support

This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.

Conflicts of Interest

None.

References

1 Levart, TK, Klokocovnik, T. Mid‐aortic syndrome in a 3‐year‐old girl: successfully treated by aorto–aortic grafting and renal artery implantation into the graft. Tex Heart Inst J 2012; 39: 657–661.Google Scholar
Figure 0

Figure 1Magnetic resonance angiography, posterior view, showing the abdominal aorta terminating in a larger sac-like structure (arrow) and its continuation through a smaller calibre vessel (*). Perfusion of the right kidney is decreased.

Figure 1

Figure 2CT angio-scan, confirming the diagnosis of abdominal aorta malformation.

Figure 2

Figure 3Aortography, anterior view, confirmed the aortic malformation, after the origin of the renal arteries, and a collateral vessel (arrow) arising from the anterior surface of the aorta, and from which both common iliac arteries emerged (*). There is duplication and hypoplasia of right renal vessels.