Prosthetic graft replacement of a large subclavian aneurysm in a child with Loeys-Dietz syndrome: a case report

European Heart Journal. Case Reports
Mohamed SobhJens Scheewe

Abstract

Loeys-Dietz syndrome (LDS) is a genetic connective tissue disorder, which is characterized by rapid development of aortic and peripheral arterial aneurysms. Loeys-Dietz syndrome has some overlapping phenotypic features with other inherited aortopathies such as Marfan syndrome. However, LDS has a more aggressive vascular course with patient morbidity and mortality occurring at an early age. We present the rare case of an 11-year-old girl with LDS who underwent valve sparing aortic root replacement at the age of 2.9 years with good results. She had routine follow-up cardiovascular magnetic resonance imaging and was found to have a large aneurysm of the right subclavian artery. After multidisciplinary team discussion, successful surgical resection with prosthetic graft replacement of the right subclavian artery was performed. This case illustrates that large aneurysms of aortic branches can already develop in childhood and underlines the need for frequent follow-ups including cross-sectional imaging and multidisciplinary team management.

References

Aug 25, 2006·The New England Journal of Medicine·Bart L LoeysHarry C Dietz
Jan 30, 2007·The Annals of Thoracic Surgery·Jason A WilliamsDuke E Cameron
May 28, 2009·Pediatric Radiology·Ajay Malhotra, Per-Lennart Westesson
May 6, 2014·The Annals of Thoracic Surgery·Erik BeckmannAndreas Martens
Feb 14, 2015·The Annals of Thoracic Surgery·Jason A WilliamsG Chad Hughes
Dec 14, 2016·The Journal of Thoracic and Cardiovascular Surgery·Nishant D PatelDuke E Cameron
Oct 4, 2017·Neurology·Sung-Min ChoAndrew B Buletko
Jan 11, 2018·Radiographics : a Review Publication of the Radiological Society of North America, Inc·William W LoughboroughMark C K Hamilton

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Methods Mentioned

BETA
dissections
sedation
dissection
surgical resection

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