Bokhari Syed Faqeer Hussain, Faizan Sattar Syed M, Mehboob Umair, Umais Muhammad, Ahmad Muhammad, Malik Ahmad, Bakht Danyal, Iqbal Asma, Dost Wahidullah
Department of Medicine and Surgery, King Edward Medical University, Lahore 54000, Punjab, Pakistan.
Curative Medicine, Kabul University of Medical Sciences, Kabul 10001, Kābul, Afghanistan.
World J Cardiol. 2025 Mar 26;17(3):103668. doi: 10.4330/wjc.v17.i3.103668.
Hypoplastic left heart syndrome is a severe congenital defect involving underdeveloped left-sided cardiac structures, leading to significant mortality and morbidity. Prenatal diagnosis using fetal ultrasound and echocardiography enables early detection, family counseling, and improved clinical decision-making. Advanced prenatal interventions, such as fetal aortic valvuloplasty and atrial septostomy, show promise but require careful patient selection. A multidisciplinary approach involving obstetricians, neonatologists, and pediatric cardiologists is vital for effective management. Future directions include refining imaging techniques, such as three-dimensional ultrasound, cardiovascular magnetic resonance imaging, and exploring bioengineering solutions, stem cell therapies, and genetic research. These advancements aim to improve therapeutic options and address current limitations, including transplant scarcity and postoperative complications. Although surgical innovations have improved survival rates, challenges remain, including neurological risks and long-term hemodynamic issues. Ongoing research and technological advancements are essential to enhance outcomes and quality of life for hypoplastic left heart syndrome patients.
左心发育不全综合征是一种严重的先天性缺陷，涉及左侧心脏结构发育不全，导致显著的死亡率和发病率。使用胎儿超声心动图进行产前诊断能够实现早期检测、为家庭提供咨询并改善临床决策。先进的产前干预措施，如胎儿主动脉瓣成形术和房间隔造口术，显示出前景，但需要仔细选择患者。产科医生、新生儿科医生和儿科心脏病专家参与的多学科方法对于有效管理至关重要。未来的方向包括改进成像技术，如三维超声、心血管磁共振成像，以及探索生物工程解决方案、干细胞疗法和基因研究。这些进展旨在改善治疗选择并解决当前的局限性，包括移植稀缺和术后并发症。尽管手术创新提高了生存率，但挑战依然存在，包括神经风险和长期血流动力学问题。持续的研究和技术进步对于提高左心发育不全综合征患者的治疗效果和生活质量至关重要。
