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Rapid progression of long-segment coarctation in a patient with Williams' syndrome

Published online by Cambridge University Press:  03 May 2005

Cammon Arrington ,
Martin Tristani-Firouzi  and
Michael Puchalski
Cammon Arrington
Affiliation:
University of Utah, Department of Pediatric Cardiology, Salt Lake City, Utah, United States of America
Martin Tristani-Firouzi
Affiliation:
University of Utah, Department of Pediatric Cardiology, Salt Lake City, Utah, United States of America
Michael Puchalski
Affiliation:
University of Utah, Department of Pediatric Cardiology, Salt Lake City, Utah, United States of America
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Abstract

Over a period of 6 days, a three-week-old male developed a long-segment coarctation, with sub-total obstruction of the descending aorta, immediately distal to the left subclavian artery. On the 24th day of life, the stenotic region was repaired by placement of a pulmonary allograft patch measuring 3 centimetres in length. Severe diffuse vascular medial thickening was discovered at the operation. Subsequent fluorescence in-situ hybridization proved positive for Williams' syndrome. To our knowledge, this is the first report of rapidly progressive infantile arteriopathy in the setting of Williams' syndrome.

Keywords

Supravalvar aortic stenosiselastinneonategenetics
Type
Brief Report
Information
Cardiology in the Young , Volume 15 , Issue 3 , June 2005 , pp. 312 - 314
Copyright
© 2005 Cambridge University Press

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