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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

Published online by Cambridge University Press:  17 December 2013

Ikenna Omeje ,
Georgi Christov ,
Sachin Khambadkone  and
Tain-Yen Hsia
Ikenna Omeje*
Affiliation:
Department of Cardiothoracic Surgery, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, United Kingdom
Georgi Christov
Affiliation:
Department of Cardiology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, United Kingdom
Sachin Khambadkone
Affiliation:
Department of Cardiology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, United Kingdom
Tain-Yen Hsia
Affiliation:
Department of Cardiothoracic Surgery, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, United Kingdom
*
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
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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.

Keywords

Cor triatriatumright atriumcoarctation of the aorta
Type
Original Articles
Information
Cardiology in the Young , Volume 25 , Issue 2 , February 2015 , pp. 308 - 311
Copyright
© Cambridge University Press 2013 

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