Center for Cardiovascular Research, Abigail Wexner Research Institute, Nationwide Children's Hospital, Columbus, OH, 43215, USA.
The Heart Center, Nationwide Children's Hospital, Columbus, OH, 43215, USA.
Curr Cardiol Rep. 2024 Nov;26(11):1221-1229. doi: 10.1007/s11886-024-02122-6. Epub 2024 Sep 28.
Hypoplastic left heart syndrome (HLHS) is a critical congenital heart defect characterized by the underdevelopment of left-sided heart structures, leading to significant circulatory challenges, and necessitating multiple surgeries for survival. Despite advancements in surgical interventions, long-term outcomes often involve heart failure, highlighting the need for a deeper understanding of HLHS pathogenesis. Current in vivo and in vitro models aim to recapitulate HLHS anatomy and physiology, yet they face limitations in accuracy and complexity.
In vivo models, including those in chick, lamb, and mouse, provide insights into hemodynamic and genetic factors influencing HLHS. In vitro models using human induced pluripotent stem cells offer valuable platforms for studying genetic mutations and cellular mechanisms. This review evaluates these models' utility and limitations, and proposes future directions for developing more sophisticated models to enhance our understanding and treatment of HLHS.
左心发育不全综合征(HLHS)是一种严重的先天性心脏缺陷,其特征为左心结构发育不全,导致显著的循环挑战,需要多次手术才能存活。尽管手术干预取得了进展,但长期结果往往涉及心力衰竭,这凸显了深入了解 HLHS 发病机制的必要性。目前的体内和体外模型旨在重现 HLHS 的解剖结构和生理学,但它们在准确性和复杂性方面存在局限性。
体内模型,包括鸡、羊和鼠的模型,提供了对影响 HLHS 的血流动力学和遗传因素的深入了解。使用人诱导多能干细胞的体外模型为研究遗传突变和细胞机制提供了有价值的平台。本综述评估了这些模型的实用性和局限性,并提出了未来开发更复杂模型的方向,以增强我们对 HLHS 的理解和治疗。