David V procedure and hemiarch replacement in a patient with Loeys-Dietz-Syndrome and beta thalassemia minor: a case report.

Beta thalassemia minor Cardiopulmonary bypass David procedure Hypothermia Loeys-Dietz-Syndrome Valve sparing root replacement

Journal

Journal of cardiothoracic surgery
ISSN: 1749-8090
Titre abrégé: J Cardiothorac Surg
Pays: England
ID NLM: 101265113

Informations de publication

Date de publication:
27 Aug 2023
Historique:
received: 26 11 2022
accepted: 09 08 2023
medline: 29 8 2023
pubmed: 28 8 2023
entrez: 27 8 2023
Statut: epublish

Résumé

We report the case of a 36-year-old European female patient presenting with a sinus valsalva aneurysm of 47 mm with moderate aortic regurgitation. Additionally, an aneurysm of the brachiocephalic trunk and multiple aneurysms of the right internal mammary artery were identified. Previous medical history included Loeys-Dietz syndrome (LDS) Type RII due to a TGF-beta receptor mutation, and beta thalassemia minor with a baseline hemoglobin of 9,3 g/dL on admission.Reconstruction of the aortic root and hemiarch replacement was performed in circulatory arrest under moderate hypothermia. During surgery, hypothermia was required as part of the cerebral protection strategy. We aim to highlight special considerations and discuss the effects of cooling, rewarming and the use of cardiopulmonary bypass (CPB) during extensive surgery in a patient with LDS and beta thalassemia minor.

Identifiants

pubmed: 37635220
doi: 10.1186/s13019-023-02347-6
pii: 10.1186/s13019-023-02347-6
pmc: PMC10464094
doi:

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

253

Informations de copyright

© 2023. BioMed Central Ltd., part of Springer Nature.

Références

Interact Cardiovasc Thorac Surg. 2009 Jan;8(1):134-47
pubmed: 18940830
Ann Thorac Surg. 2012 Aug;94(2):587-90; discussion 590-1
pubmed: 22748646
Ann Thorac Surg. 2007 Feb;83(2):S757-63; discussion S785-90
pubmed: 17257922
Ann Thorac Surg. 2013 Feb;95(2):579-85
pubmed: 23103004
Catheter Cardiovasc Interv. 2010 Aug 1;76(2):E43-86
pubmed: 20687249

Auteurs

Frieda-Maria Kainz (FM)

Department of Cardiac Surgery, University Hospital of St. Poelten, St. Pölten, Austria. frieda-maria.kainz@stpoelten.lknoe.at.

Kathrin Freystaetter (K)

Department of Cardiac Surgery, University Hospital of St. Poelten, St. Pölten, Austria.

Bruno K Podesser (BK)

Department of Cardiac Surgery, University Hospital of St. Poelten, St. Pölten, Austria.
Center for Biomedical Research and Translational Surgery, Medical University of Vienna, Vienna, Austria.

Christoph Holzinger (C)

Department of Cardiac Surgery, University Hospital of St. Poelten, St. Pölten, Austria.

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