Current state of the art in hypoplastic left heart syndrome.
Current state of the art in hypoplastic left heart syndrome.
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DOI：
10.3389/fcvm.2022.878266
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发表时间：
2022
影响因子：
3.6
通讯作者：
中科院分区：
文献类型：
--
作者：
左心发育不良综合征 (HLHS) 是一种复杂的先天性心脏病，新生儿出生时左心室和相关结构发育不全。如果没有姑息性干预措施，HLHS 是致命的。治疗通常包括出生时的医疗管理，以保持动脉导管的通畅，然后进行三种姑息手术：最常见的诺伍德手术、双向腔肺分流术和 Fontan 手术。随着 HLHS 患者手术治疗的最新进展，三级治疗中心现在获得了高生存率，尽管不良的神经发育结果仍然是一个临床挑战。虽然手术治疗仍然是 HLHS 患者的护理标准，但创新的治疗策略仍在不断发展。了解 HLHS 患者的遗传基础对于制定针对 HLHS 患者的新策略非常重要。为 HLHS 患者开发的另一项研究策略是在右心室心肌内注射干细胞。组织工程和再生医学的最新创新有望提供重要的工具，以了解 HLHS 的根本基础并提供新的治疗策略。在这篇综述文章中，我们概述了 HLHS，从历史描述开始，逐步讨论遗传学、手术管理、术后结果、干细胞治疗、血流动力学和组织工程方法。
Hypoplastic left heart syndrome (HLHS) is a complex congenital heart condition in which a neonate is born with an underdeveloped left ventricle and associated structures. Without palliative interventions, HLHS is fatal. Treatment typically includes medical management at the time of birth to maintain patency of the ductus arteriosus, followed by three palliative procedures: most commonly the Norwood procedure, bidirectional cavopulmonary shunt, and Fontan procedures. With recent advances in surgical management of HLHS patients, high survival rates are now obtained at tertiary treatment centers, though adverse neurodevelopmental outcomes remain a clinical challenge. While surgical management remains the standard of care for HLHS patients, innovative treatment strategies continue to be developing. Important for the development of new strategies for HLHS patients is an understanding of the genetic basis of this condition. Another investigational strategy being developed for HLHS patients is the injection of stem cells within the myocardium of the right ventricle. Recent innovations in tissue engineering and regenerative medicine promise to provide important tools to both understand the underlying basis of HLHS as well as provide new therapeutic strategies. In this review article, we provide an overview of HLHS, starting with a historical description and progressing through a discussion of the genetics, surgical management, post-surgical outcomes, stem cell therapy, hemodynamics and tissue engineering approaches.
影响因子：
3.7
作者：
Ricci M;Xu Y;Hammond HL;Willoughby DA;Nathanson L;Rodriguez MM;Vatta M;Lipshultz SE;Lincoln J
通讯作者：
Lincoln J
DOI：
10.1016/j.mayocpiqo.2017.07.002
发表时间：
2017-09
期刊：
Mayo Clinic proceedings. Innovations, quality & outcomes
影响因子：
--
作者：
Qureshi MY;Cabalka AK;Khan SP;Hagler DJ;Haile DT;Cannon BC;Olson TM;Cantero-Peral S;Dietz AB;Radel DJ;Taggart NW;Kelle AM;Rodriguez V;Dearani JA;O'Leary PW;Wanek Program Regenerative Medicine Pipeline
通讯作者：
Wanek Program Regenerative Medicine Pipeline