Division of Pediatric Cardiac Surgical Centre, Chinese Academy of Medical Sciences and Peking Union Medical College, Fuwai Hospital, Beijing, China.
Division of Pediatric Echo Centre, Chinese Academy of Medical Sciences and Peking Union Medical College, Fuwai Hospital, Beijing, China.
Ann Thorac Surg. 2021 Dec;112(6):2029-2037. doi: 10.1016/j.athoracsur.2020.10.025. Epub 2020 Nov 12.
The outcomes, therapeutic strategies, and risk factors of congenital corrected transposition of great arteries (ccTGA) unoperated before adulthood are unclear.
From October 2009 to January 2018, 117 adult ccTGA patients, classified into ccTGA with intact ventricular septum, ventricular septum defect, and pulmonary valve or subpulmonary outflow tract stenosis (PS) groups, were reviewed. Statistical analysis was performed with SPSS 19.0 (IBM, Armonk, NY).
At the first visit, no patients suffered operation. The PS group had the least systemic atrioventricular valve regurgitation and the greatest systemic ventricular ejection fraction. All 49 patients underwent surgery. From the first visit to last follow-up, systemic ventricular ejection fraction of unoperated ccTGA decreased significantly. In the intact ventricular septum group, patients receiving systemic atrioventricular valve replacement/valvuloplasty had a significantly increased systemic ventricular ejection fraction and statistically more freedom from death and transplant than unoperated. In the ventricular septum defect group the late systemic ventricular ejection fraction of operated patients was not statistically different from their basic data at first visit. In the PS group patients receiving physiologic repair had significantly decreased systemic ventricular ejection fractions. Severe systemic atrioventricular valve regurgitation, physiologic repair, and systemic ventricular dysfunction (ejection fraction <40%) were risk factors for mortality, transplant, and congestive heart failure.
PS protects against systemic atrioventricular valve regurgitation and ventricular dysfunction. Systemic atrioventricular valve replacement/valvuloplasty improved systemic ventricular function for ccTGA with an intact ventricular septum. Physiologic repair was not ideal for ccTGA with PS. Severe systemic atrioventricular valve regurgitation and systemic ventricular dysfunction were associated with suboptimal outcomes.
成人前未经手术的先天性矫正型大动脉转位（ccTGA）的结局、治疗策略和危险因素尚不清楚。
从 2009 年 10 月至 2018 年 1 月，回顾了 117 例成人 ccTGA 患者，分为 ccTGA 伴完整室间隔、室间隔缺损和肺动脉瓣或亚肺动脉流出道狭窄（PS）组。使用 SPSS 19.0（IBM，Armonk，NY）进行统计分析。
首次就诊时，无患者接受手术。PS 组的系统性房室瓣反流最少，系统性心室射血分数最大。所有 49 例患者均接受手术。从首次就诊到最后一次随访，未经手术的 ccTGA 患者的系统性心室射血分数显著下降。在完整室间隔组中，接受系统性房室瓣置换/成形术的患者系统性心室射血分数显著增加，且在未手术的患者中，死亡和移植的自由度显著更高。在室间隔缺损组中，接受手术的患者的晚期系统性心室射血分数与首次就诊时的基本数据无统计学差异。在 PS 组中，接受生理性修复的患者的系统性心室射血分数显著下降。严重的系统性房室瓣反流、生理性修复和系统性心室功能障碍（射血分数<40%）是死亡、移植和充血性心力衰竭的危险因素。
PS 可防止系统性房室瓣反流和心室功能障碍。对于完整室间隔的 ccTGA，系统性房室瓣置换/成形术可改善系统性心室功能。对于 PS 的 ccTGA，生理性修复不理想。严重的系统性房室瓣反流和系统性心室功能障碍与预后不佳相关。
