Miyamoto Masakazu, Nishii Nobuhiro, Morita Hiroshi, Ito Hiroshi
Department of Cardiovascular Medicine, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
Department of Cardiovascular Therapeutics, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
Eur Heart J Case Rep. 2020 Sep 7;4(5):1-6. doi: 10.1093/ehjcr/ytaa264. eCollection 2020 Oct.
The incidence of ventricular tachycardia (VT) in patients following Fontan operation is reported as 3.5%. Furthermore, in patients with repaired double outlet right ventricle (DORV), scar-related VT and outflow tract VT have been reported; however, Purkinje-related VT has not previously been reported. In this report, we present the case of idiopathic left VT (ILVT) in a patient with DORV who underwent Fontan operation.
A 31-year-old man was diagnosed as having DORV with complete atrioventricular defect at birth. When he was 17 years old, he underwent surgical repair, including extracardiac Fontan operation and common atrioventricular valve replacement. Five years later, VT was detected. Since some medications were ineffective in suppressing VT, he was referred to our hospital for definitive treatment. Ventricular tachycardia was induced by atrial and ventricular programmed electrical stimulations. The mechanism of the VT was determined to be re-entry. The earliest activation site was located at the mid-inferior septum of the hypoplastic left ventricle, in which Purkinje potentials were observed before the local ventricular electrogram. Radiofrequency catheter ablation (RFCA) was performed at this site to eliminate VT.
Most VTs originate from surgical scars in patients with congenital heart disease. Catheter ablation was feasible in scar-related VT. To the best of our knowledge, this is the first report of ILVT treated successfully with RFCA in a DORV patient who had undergone Fontan operation.
据报道，接受Fontan手术的患者室性心动过速（VT）的发生率为3.5%。此外，在修复的右心室双出口（DORV）患者中，已报道有瘢痕相关室性心动过速和流出道室性心动过速；然而，此前尚未报道过浦肯野相关室性心动过速。在本报告中，我们介绍了1例接受Fontan手术的DORV患者发生特发性左室室性心动过速（ILVT）的病例。
一名31岁男性出生时被诊断为患有DORV并伴有完全性房室缺损。17岁时，他接受了手术修复，包括心外Fontan手术和共同房室瓣置换术。5年后，检测到室性心动过速。由于一些药物在抑制室性心动过速方面无效，他被转诊至我院进行确定性治疗。通过心房和心室程序性电刺激诱发室性心动过速。确定室性心动过速的机制为折返。最早激动部位位于发育不良的左心室中下间隔，在局部心室电图之前观察到浦肯野电位。在该部位进行了射频导管消融（RFCA）以消除室性心动过速。
大多数室性心动过速起源于先天性心脏病患者的手术瘢痕。导管消融在瘢痕相关室性心动过速中是可行的。据我们所知，这是首例在接受Fontan手术的DORV患者中用RFCA成功治疗ILVT的报告。
