Washington University in St. Louis School of Medicine/St. Louis Children's Hospital, St. Louis, Missouri.
Washington University in St. Louis School of Medicine/St. Louis Children's Hospital, St. Louis, Missouri.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2022;25:38-47. doi: 10.1053/j.pcsu.2022.04.001.
Management strategies for congenitally corrected transposition of the great arteries (ccTGA) historically consisted of a physiologic repair, resulting in the morphologic right ventricle (mRV) supporting systemic circulation. This strategy persisted despite the development of heart failure by middle age because of the reasonable short-term outcomes, and the natural history of some patients with favorable anatomy (felt to demonstrate the mRV's ability to function in the long-term), and due to the less-than-optimal outcomes associated with anatomical repair. As outcomes with anatomical repair improved, and the long-term risk of systemic mRV dysfunction became apparent, more have begun to realize its advantages. In addition to the decision on whether or not to pursue anatomical repair, and the optimal timing, studies demonstrating the nuance to morphologic left ventricle retraining have demonstrated its feasibility. Further considerations in ccTGA have begun to be better understood, including: the management of a poorly functioning mRV, systemic tricuspid valve regurgitation, the utility of morphologic left ventricle outflow tract obstruction (native or surgically created) and pacing strategies. While some considerations are apparent: biventricular pacing is superior to univentricular, tricuspid regurgitation must be managed early with either progression towards anatomical repair (pulmonary artery banding if needed for retraining) or tricuspid replacement (not repair) based on the patient's age; others remain to be completely elucidated. Overall, the heterogeneity of ccTGA, as well as the unique presentation with each patient regarding ventricular and valvular function and center-to-center variability in management strategies has made the interpretation of published data difficult. That said, more recent long-term outcomes favor anatomical repair in most situations.
先心病矫正型大动脉转位（ccTGA）的管理策略历来包括生理性修复，从而使形态右心室（mRV）支持体循环。尽管在中年时会出现心力衰竭，但由于短期结果合理，一些具有良好解剖结构的患者（认为mRV 具有长期功能的能力）的自然病史，以及解剖修复相关的不太理想的结果，这种策略仍然存在。随着解剖修复结果的改善，以及 mRV 系统功能长期受损的风险变得明显，越来越多的人开始认识到其优势。除了是否进行解剖修复以及最佳时机的决策外，还研究了形态左心室再训练的细微差别，证明了其可行性。ccTGA 中的进一步考虑因素开始得到更好的理解，包括：功能不良的 mRV 的管理、系统三尖瓣反流、形态左心室流出道梗阻（固有或手术创建）的实用性和起搏策略。虽然一些考虑因素显而易见：双心室起搏优于单心室起搏，三尖瓣反流必须早期管理，无论是通过解剖修复进展（如果需要再训练则进行肺动脉带缩术）还是三尖瓣置换（而非修复），具体取决于患者的年龄；其他方面仍有待完全阐明。总体而言，ccTGA 的异质性以及每个患者在心室和瓣膜功能以及管理策略中心到中心变异性方面的独特表现，使得对已发表数据的解释变得困难。尽管如此，最近的长期结果在大多数情况下都倾向于解剖修复。
