Prosthetic graft replacement of a large subclavian aneurysm in a child with Loeys-Dietz syndrome: a case report.
Cardiovascular magnetic resonance imaging
Case report
Loeys–Dietz syndrome
Subclavian artery aneurysm
Journal
European heart journal. Case reports
ISSN: 2514-2119
Titre abrégé: Eur Heart J Case Rep
Pays: England
ID NLM: 101730741
Informations de publication
Date de publication:
Oct 2020
Oct 2020
Historique:
received:
03
03
2020
revised:
09
05
2020
accepted:
19
05
2020
entrez:
18
11
2020
pubmed:
19
11
2020
medline:
19
11
2020
Statut:
epublish
Résumé
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.
Sections du résumé
BACKGROUND
BACKGROUND
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.
CASE SUMMARY
METHODS
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.
DISCUSSION
CONCLUSIONS
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.
Identifiants
pubmed: 33204949
doi: 10.1093/ehjcr/ytaa163
pii: ytaa163
pmc: PMC7649448
doi:
Types de publication
Case Reports
Langues
eng
Pagination
1-4Informations de copyright
© The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology.
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