Valve-Sparing Aortic Root Replacement in an 8-Month-Old Infant With Loeys-Dietz Syndrome.


Journal

The Annals of thoracic surgery
ISSN: 1552-6259
Titre abrégé: Ann Thorac Surg
Pays: Netherlands
ID NLM: 15030100R

Informations de publication

Date de publication:
May 2019
Historique:
received: 11 07 2018
accepted: 24 07 2018
pubmed: 14 10 2018
medline: 20 12 2019
entrez: 14 10 2018
Statut: ppublish

Résumé

The clinical experience with Loeys-Dietz syndrome (LDS) reveals fateful natural history with intracerebral incidents and aortic dissections. A newborn child was referred to our hospital with significantly dilated aortic root and clinical signs of LDS phenotype later genetically confirmed as LDS type I. A therapy with antihypertensive medicines was initiated to postpone the surgery. Despite that, aortic root dilatation progressed to 33 mm (z-score +9.3). To avoid aortic rupture, a valve sparing aortic root replacement was performed at patient age of 8 months. The purpose of this report is to share our dilemmas and experience in the treatment of this child.

Identifiants

pubmed: 30315797
pii: S0003-4975(18)31448-6
doi: 10.1016/j.athoracsur.2018.07.094
pii:
doi:

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

e321-e323

Informations de copyright

Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Auteurs

Konrad Wisniewski (K)

Division of Pediatric Cardiac Surgery, University Hospital Muenster, Muenster, Germany. Electronic address: konrad.wisniewski@ukmuenster.de.

Sybille Singer (S)

Department of Pediatric Cardiology, University Hospital Muenster, Muenster, Germany.

Hans-Gerd Kehl (HG)

Department of Pediatric Cardiology, University Hospital Muenster, Muenster, Germany.

Pawel Nawrocki (P)

Division of Pediatric Cardiac Surgery, University Hospital Muenster, Muenster, Germany.

Katarzyna Januszewska (K)

Division of Pediatric Cardiac Surgery, University Hospital Muenster, Muenster, Germany.

Edward Malec (E)

Division of Pediatric Cardiac Surgery, University Hospital Muenster, Muenster, Germany.

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