Preda Alberto, Testoni Alessio, Baroni Matteo, Mazzone Patrizio, Gigli Lorenzo
Cardio-Thoraco-Vascular Department, Electrophysiology Unit ASST Grande Ospedale Metropolitano Niguarda Milan Italy.
Clin Case Rep. 2024 Apr 23;12(4):e8745. doi: 10.1002/ccr3.8745. eCollection 2024 Apr.
A 46-year-old woman with congenitally corrected transposition of the great arteries (ccTGA) associated with dextrocardia, situs viscerus inversus, and left superior vena cava persistence presented with an incessant supraventricular tachycardia. Electrophysiological study was not conclusive in differential diagnosis of atrial tachycardia versus atypical atrioventricular (AV) nodal reentrant tachycardia, also due to the unconventional anatomy of the coronary sinus. By a comprehensive mapping of cardiac chambers, a double side slow-pathway was localized in both atrial chambers and subsequently ablated by radiofrequency delivery without tachycardia changes. Aortic root and cusps were devoid of electrical activity. The muscular part of the sub-pulmonary ventricle at the level of interatrial septum showed an earliest activation signal of -90 ms and ablation of this site was effective in abolish the tachycardia. This is the first case to report technical concerns of septal atrial tachycardia ablation in ccTGA associated with multiple anatomical malformations. Moreover, some peculiarities have been reported for the first time including the presence of double-side AV nodal slow-pathway and atypical localization of the tachycardia origin into the muscular part of the sub-pulmonary ventricle instead of posterior pulmonary cusp.
一名46岁女性,患有先天性矫正型大动脉转位(ccTGA),合并右位心、内脏反位和左上腔静脉持续存在,出现持续性室上性心动过速。由于冠状窦解剖结构异常,电生理研究在鉴别诊断房性心动过速与非典型房室(AV)结折返性心动过速时未得出明确结论。通过对心脏腔室进行全面标测,在两个心房腔室中均定位到双侧慢径路,随后通过射频消融治疗,心动过速未发生改变。主动脉根部和瓣膜无电活动。房间隔水平的肺下心室肌部显示最早激动信号为-90毫秒,消融该部位可有效消除心动过速。这是首例报告ccTGA合并多种解剖畸形时室间隔房性心动过速消融技术问题的病例。此外,首次报告了一些特殊情况,包括双侧AV结慢径路的存在以及心动过速起源于肺下心室肌部而非后肺动脉瓣叶的非典型定位。