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Anesthetic management of a child with Loeys-Dietz syndrome undergoing complete aortic arch replacement.

Cirugia y cirujanos - 1 Jan 2023

Olabarrieta-Zarain Unai, Martínez-Santos Leire, Alberdi-Enríquez Alex, Guereca-Gala Ane, Bravo-Sevilla Blanca, Martínez-Ruiz Alberto

Abstract excerpt

Loeys-Dietz syndrome (LDS) is a connective tissue disease related to β-transforming growth factor mutations, which causes aneurysms formation, vascular tortuosity and skeletal manifestations. The prognosis is very poor, and mortality occurs at the age of 27 in patients without surgical treatment. Despite being diagnosed in childhood, is not usual surgical aortic replacement in children. We report a case of 12...

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PMID: 37677958
DOI: 10.24875/CIRU.21000786

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