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Cor triatriatum dexter and coarctation of the aorta – a rare association in a 7-year-old child with type 1 neurofibromatosis

  • Ikenna Omeje (a1), Georgi Christov (a2), Sachin Khambadkone (a2) and Tain-Yen Hsia (a1)

Abstract

Cor triatriatum dexter is an extremely rare congenital anomaly of the right atrium. It occurs because of the persistence of the right sinus venosus valve, resulting in partitioning of the right atrium. Most of the described cases of cor triatriatum dexter in the literature were incidental findings on echocardiogram or at necropsy.

We present a case report of a 7-year-old girl who was referred to us for further assessment, with a possible diagnosis of coarctation of the aorta. Initial investigations confirmed not only the presence of a long segment coarctation of the aorta, but also a large obstructive membrane in the right atrium.

A catheter intervention was performed to stent the coarctation segment, and the fibro-muscular shelf in the right atrium was surgically resected.

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Corresponding author

Correspondence to: Dr I. Omeje, MD, PhD, Cardiothoracic Unit, Great Ormond Street Hospital for Children, NHS Foundation Trust, London WC1N 3JH, United Kingdom. Tel: +02074059200, ext 5730; Fax: +02074301281; E-mail: Omejei@gosh.nhs.uk

References

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