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特纳综合征中的左心发育不全:移植的主要指征?

Hypoplastic left heart in Turner's syndrome: a primary indication for transplant?

作者信息

Philip Joseph, Gupta Dipankar, Bleiweis Mark S, Pietra Biagio A, Vyas Himesh V

机构信息

Congenital Heart Center,UF Health Shands Children's Hospital,University of Florida,Gainesville,Florida,United States of America.

出版信息

Cardiol Young. 2018 Mar;28(3):458-460. doi: 10.1017/S1047951117001962. Epub 2017 Dec 13.

DOI:10.1017/S1047951117001962
PMID:29233199
Abstract

Survival for hypoplastic left heart syndrome patients following the Norwood procedure is 71-90%. Mortality in patients with Turner's syndrome and hypoplastic left heart syndrome after conventional palliation (Norwood operation) has been reported as high as 80%. This questions the approach of traditional staged palliation. Here, we report a patient with hypoplastic left heart syndrome and Turner's syndrome bridged to orthotopic heart transplantation following a hybrid procedure.

摘要

诺伍德手术后,左心发育不全综合征患者的生存率为71%-90%。据报道,患有特纳综合征和左心发育不全综合征的患者在进行传统姑息治疗(诺伍德手术)后的死亡率高达80%。这对传统分期姑息治疗方法提出了质疑。在此,我们报告一例患有左心发育不全综合征和特纳综合征的患者,在接受杂交手术后成功过渡到原位心脏移植。

相似文献

1
Hypoplastic left heart in Turner's syndrome: a primary indication for transplant?特纳综合征中的左心发育不全:移植的主要指征?
Cardiol Young. 2018 Mar;28(3):458-460. doi: 10.1017/S1047951117001962. Epub 2017 Dec 13.
2
Stage 1 hybrid palliation for hypoplastic left heart syndrome--assessment of contemporary patterns of use: an analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.阶段 1 杂交姑息治疗左心发育不良综合征——当代应用模式评估:对胸外科医生学会先天性心脏外科学数据库的分析。
J Thorac Cardiovasc Surg. 2015 Jan;149(1):195-201, 202.e1. doi: 10.1016/j.jtcvs.2014.08.020. Epub 2014 Aug 14.
3
Results of heart transplantation following failed staged palliation of hypoplastic left heart syndrome and related single ventricle anomalies.左心发育不全综合征及相关单心室畸形分期姑息治疗失败后的心脏移植结果。
Eur J Cardiothorac Surg. 2015 Nov;48(5):792-8; discussion 798-9. doi: 10.1093/ejcts/ezu547. Epub 2015 Jan 18.
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Comparison of in-hospital and longer-term outcomes of hybrid and Norwood stage 1 palliation of hypoplastic left heart syndrome.比较左心发育不全综合征的杂交术与 Norwood Ⅰ期心脏姑息术的院内和长期结局。
J Thorac Cardiovasc Surg. 2015 Sep;150(3):474-80.e2. doi: 10.1016/j.jtcvs.2015.06.071. Epub 2015 Jul 4.
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Hybrid versus Norwood procedure for hypoplastic left heart syndrome: contemporary series from a single center.左心发育不良综合征的杂交手术与 Norwood 手术比较:单中心的当代系列研究。
J Thorac Cardiovasc Surg. 2014 Jun;147(6):1777-82. doi: 10.1016/j.jtcvs.2014.02.066. Epub 2014 Feb 27.
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Outcomes of heart transplantation in children with hypoplastic left heart syndrome previously palliated with the Norwood procedure.曾接受诺伍德手术姑息治疗的左心发育不全综合征儿童心脏移植的结果。
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Surgical palliation strategy does not affect interstage ventricular dysfunction or atrioventricular valve regurgitation in children with hypoplastic left heart syndrome and variants.对于左心发育不全综合征及变异型患儿,外科姑息治疗策略并不影响中胚层室功能障碍或房室瓣反流。
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Fifteen-year single-center experience with the Norwood operation for complex lesions with single-ventricle physiology compared with hypoplastic left heart syndrome.15 年单中心经验:比较单一心室生理与左心发育不全综合征,行 Norwood 手术治疗复杂病变。
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Application of the hybrid Stage 1 palliation concept to patients without hypoplastic left heart syndrome as a bridge to heart transplant.将一期混合姑息治疗概念应用于无左心发育不全综合征的患者作为心脏移植的过渡。
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Early Outcomes of Hypoplastic Left Heart Syndrome Infants: Meta-Analysis of Studies Comparing the Hybrid and Norwood Procedures.左心发育不全综合征婴儿的早期结局:比较杂交手术和诺伍德手术的研究的荟萃分析
World J Pediatr Congenit Heart Surg. 2018 Mar;9(2):224-233. doi: 10.1177/2150135117752896.

引用本文的文献

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Postoperative Morbidity and Interstage Hemodynamics Following Stage I Palliation in Patients with Turner Syndrome and Hypoplastic Left Heart Syndrome.Turner 综合征合并左心发育不良综合征患者一期姑息术后的并发症和中期间歇性血液动力学
Pediatr Cardiol. 2024 Feb;45(2):221-227. doi: 10.1007/s00246-023-03395-4. Epub 2023 Dec 28.
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Ventricular assist device support in neonates and infants with a failing functionally univentricular circulation.患有功能单心室循环衰竭的新生儿和婴儿的心室辅助装置支持。
JTCVS Tech. 2021 Oct 19;13:194-204. doi: 10.1016/j.xjtc.2021.09.056. eCollection 2022 Jun.