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.
J Cardiothorac Surg. 2023 Aug 27;18(1):253. doi: 10.1186/s13019-023-02347-6.
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.
我们报告了一例 36 岁的欧洲女性患者，其患有 47mm 的窦房结瓦氏窦动脉瘤，伴有中度主动脉瓣反流。此外，还发现头臂干动脉瘤和右内乳动脉多发动脉瘤。既往病史包括 Loeys-Dietz 综合征（LDS）RII 型，因 TGF-β受体突变，以及β地中海贫血伴入院时基础血红蛋白 9.3g/dL。在中度低温体外循环下进行主动脉根部重建和半弓置换。在手术过程中，需要低温作为脑保护策略的一部分。我们旨在强调特殊注意事项，并讨论在患有 LDS 和β地中海贫血的患者中进行广泛手术时冷却、复温以及使用体外循环（CPB）的影响。
