Institutional report - Cardiac general Cardiac surgery in type-1-myotonic muscular dystrophy (Steinert syndrome) associated to Barlow disease
Institutional report - Cardiac general Cardiac surgery in type-1-myotonic muscular dystrophy (Steinert syndrome) associated to Barlow disease
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机构报告 - 心脏普通心脏手术治疗与巴洛病相关的 1 型强直性肌营养不良症（斯坦纳特综合征）
DOI：
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发表时间：
2008
期刊：
影响因子：
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通讯作者：
G. Gensini
中科院分区：
文献类型：
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作者：
S. Gelsomino;R. Lorusso;G. Billè;G. Cicco;U. Broi;C. Rostagno;G. Gensini
英语文献中没有关于Barlow病合并Steinert综合征患者的资料，对这些患者使用低温体外循环（CPB）和高钾血症性心脏截瘫的了解也很少。我们介绍了我们的经验与六个患者的黏液瘤变性相关的斯坦纳特病进行复杂的二尖瓣修复。在所有患者中，我们采用了轻度低温CPB（31.8℃），在整个队列中，心肌保护是通过使用血高钾冷心脏截瘫来实现的。所有患者的术后过程都很顺利，没有观察到寒颤或全身肌肉收缩。此外，所有患者在门诊基础上保持良好。低温CPB和高钾性心脏停搏可以安全地用于需要复杂心脏手术的Steinert综合征患者。需要进一步的大型研究来证实我们的发现。2008年由欧洲心胸外科协会出版。版权所有。
No data exist in the English-language literature about patients with Barlow disease associated to Steinert syndrome and little is known about the employment of hypothermic cardiopulmonary bypass (CPB) and hyperkalemic cardioplegia in these patients. We present our experience with six patients affected by myxomatous degeneration associated to Steinert disease undergoing complex mitral valve repair. In all patients we employed mild hypothermic CPB (31 8C) and myocardial protection was achieved, in the entire cohort, by the use of blood hyperkalemic cold cardioplegia. The postoperative course was uneventful in all patients and neither shivering nor generalized muscle contraction were observed. Furthermore, all patients have remained well on an outpatient basis. Hypothermic CPB and hyperkalemic cardioplegia can be safely employed in patients with Steinert syndrome requiring complex cardiac surgery. Further large studies are necessary to confirm our findings. 2008 Published by European Association for Cardio-Thoracic Surgery. All rights reserved.