Article ; Online: David V procedure and hemiarch replacement in a patient with Loeys-Dietz-Syndrome and beta thalassemia minor: a case report.
Journal of cardiothoracic surgery
2023 Volume 18, Issue 1, Page(s) 253
Abstract: 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 ...
Abstract | 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. |
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MeSH term(s) | Humans ; Female ; Adult ; Loeys-Dietz Syndrome/complications ; Loeys-Dietz Syndrome/surgery ; beta-Thalassemia/complications ; Hypothermia ; Aorta ; Aortic Valve Insufficiency |
Language | English |
Publishing date | 2023-08-27 |
Publishing country | England |
Document type | Case Reports ; Journal Article |
ZDB-ID | 2227224-0 |
ISSN | 1749-8090 ; 1749-8090 |
ISSN (online) | 1749-8090 |
ISSN | 1749-8090 |
DOI | 10.1186/s13019-023-02347-6 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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