Subramanian Anand, Bharath A P, Jayaranganath M
Department of Pediatric Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bengaluru, Karnataka, India.
Ann Pediatr Cardiol. 2019 May-Aug;12(2):153-155. doi: 10.4103/apc.APC_124_18.
An unguarded atrioventricular orifice is an extremely rare congenital anomaly characterized by the absence of the atrioventricular valve in varying proportions. While atresia of the mitral or aortic valves are usually described as causes for hypoplastic left heart, our case highlights the role of free atrioventricular valve regurgitation and consequent volume loss of the left heart, giving rise to a small left ventricle. There was an associated double-outlet right ventricle and Type B aortic interruption. While we have attempted to discuss the complex management options in this scenario, the parents decided to withdraw further care.
无保护的房室口是一种极其罕见的先天性异常，其特征是房室瓣不同程度缺失。虽然二尖瓣或主动脉瓣闭锁通常被认为是左心发育不全的原因，但我们的病例突出了房室瓣游离反流以及随之而来的左心容量损失所起的作用，导致左心室变小。同时还伴有右心室双出口和B型主动脉中断。虽然我们试图讨论这种情况下的复杂管理方案，但患儿父母决定停止进一步治疗。
