Overman David M, Baffa Jeanne M, Cohen Meryl S, Mertens Luc, Gremmels David B, Jegatheeswaran Anusha, McCrindle Brian W, Blackstone Eugene H, Morell Victor O, Caldarone Christopher, Williams William G, Pizarro Christian
Division of Pediatric Cardiac Surgery, The Children's Heart Clinic, Children's Hospitals and Clinics of Minnesota, MN, USA.
World J Pediatr Congenit Heart Surg. 2010 Apr;1(1):91-6. doi: 10.1177/2150135110363024.
Unbalanced atrioventricular septal defect is an uncommon lesion with widely varying anatomic manifestations. When unbalance is severe, diagnosis and treatment is straightforward, directed toward single-ventricle palliation. Milder forms, however, pose a challenge to current diagnostic and therapeutic approaches. The transition from anatomies that are capable of sustaining biventricular physiology to those that cannot is obscure, resulting in uneven application of surgical strategy and excess mortality. Imprecise assessments of ventricular competence have dominated clinical decision making in this regard. Malalignment of the atrioventricular junction and its attendant derangement of inflow physiology is a critical factor in determining the feasibility of biventricular repair in the setting of unbalanced atrioventricular septal defect. The atrioventricular valve index accurately identifies unbalanced atrioventricular septal defect and also brings into focus a zone of transition from anatomies that can support a biventricular end state and those that cannot.
不平衡型房室间隔缺损是一种少见的病变，其解剖表现差异很大。当不平衡严重时，诊断和治疗很直接，针对单心室姑息治疗。然而，较轻的形式对当前的诊断和治疗方法构成挑战。从能够维持双心室生理功能的解剖结构向不能维持的解剖结构的转变是模糊不清的，导致手术策略应用不均衡和死亡率过高。在这方面，对心室功能的不精确评估主导了临床决策。房室连接不对位及其伴随的流入道生理紊乱是决定不平衡型房室间隔缺损情况下双心室修复可行性的关键因素。房室瓣指数能准确识别不平衡型房室间隔缺损，还聚焦了从能够支持双心室终末状态的解剖结构向不能支持的解剖结构的过渡区域。
