Department of Pediatric Cardiology, Besat Hospital, Kurdistan University of Medical Sciences, Sanandaj, Iran.
Arch Iran Med. 2024 Sep 1;27(9):527-529. doi: 10.34172/aim.31185.
Hypoplastic left heart syndrome (HLHS) is a relatively prevalent fetal echocardiography finding in complex congenital heart diseases. Current studies indicate that intrinsic and extrinsic mechanisms could be involved in the development of left heart hypoplasia. Left ventricular inflow or outflow disorders may cause left heart hypoplasia. Prenatal aortic valvuloplasty has become more common as a therapeutic strategy. Our case presentation provides an opportunity for a new vision toward the etiology, prevention, and treatment of HLHS. In our patient, prenatal progressive left heart hypoplasia associated with restrictive foramen oval (FO) suggested the likelihood of a flow-mediated mechanism. Additionally, postnatal improvement of the hypoplastic left heart in the presence of a functional perimembranous ventricular septal defect (PM-VSD) reinforced the suspicion of an extrinsic mechanism. Pre- or postnatal interventional creation of an atrial septal defect (ASD) or VSD is our proposed method for HLHS in selected patients.
左心发育不良综合征（HLHS）是复杂先天性心脏病中较为常见的胎儿超声心动图表现。目前的研究表明，内在和外在机制可能参与左心发育不良的发生。左心室流入或流出障碍可能导致左心发育不良。作为一种治疗策略，产前主动脉瓣成形术越来越常见。我们的病例报告为 HLHS 的病因、预防和治疗提供了新的视角。在我们的患者中，与限制性卵圆孔（FO）相关的进行性左心发育不良提示可能存在血流介导的机制。此外，在存在膜周室间隔缺损（PM-VSD）的功能性的情况下，左心发育不良的改善进一步提示可能存在外在机制。我们建议在某些患者中，通过介入方法预先或产后创建房间隔缺损（ASD）或室间隔缺损（VSD）来治疗 HLHS。
