Yasuhara Jun, Kumamoto Takashi, Kojima Takuro, Shimizu Hiroyuki, Yoshiba Shigeki, Kobayashi Toshiki, Oyanagi Takayuki, Yamagishi Hiroyuki, Sumitomo Naokata
Department of Pediatric Cardiology, Saitama Medical University International Medical Center, Saitama, Japan.
Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan.
J Cardiol Cases. 2018 Sep 14;19(1):1-4. doi: 10.1016/j.jccase.2018.08.005. eCollection 2019 Jan.
A 1-year-old infant with asplenia syndrome and congenital heart disease consisting of common atrium, common inlet left ventricle with a common atrio-ventricular (AV) valve, pulmonary atresia, and total anomalous pulmonary venous connection was admitted to our hospital for radiofrequency catheter ablation (RFCA) of supraventricular tachycardia (SVT) before total cavo-pulmonary connection. After antiarrhythmic medications were discontinued for RFCA, she suffered from SVT that resulted in the rapid deterioration of hemodynamic status. Antiarrhythmic medications and cardioversion were not effective in terminating SVT. The baseline electrocardiogram confirmed the existence of twin AV nodes; however, this SVT was revealed to be focal atrial tachycardia (AT) with enhanced automaticity. The origin of AT was not related to surgical scar. Emergent RFCA for AT was successful in our case of asplenia syndrome. AT is a life-threatening complication in a single ventricle and delayed treatment can be fatal. It is important to perform RFCA promptly when drug treatment is not effective. We suggest that the AV node is not always the target site for ablation in patients with asplenia syndrome and twin AV nodes. < In the case of supraventricular tachycardia with asplenia and twin atrio-ventricular (AV) nodes, atrial tachycardia (AT) as well as AV reentrant tachycardia could occur. AT is a life-threatening complication in infants with single ventricle. If drug therapy is not effective, emergent catheter ablation should be performed. AV node is not always the target site for ablation in patients with asplenia and twin AV nodes.>.
一名1岁患有无脾综合征和先天性心脏病的婴儿，其先天性心脏病包括共同心房、具有共同房室（AV）瓣的共同流入道左心室、肺动脉闭锁和完全性肺静脉异位连接，在进行全腔静脉-肺动脉连接术前因室上性心动过速（SVT）入住我院接受射频导管消融术（RFCA）。在停用抗心律失常药物以进行RFCA后，她出现了SVT，导致血流动力学状态迅速恶化。抗心律失常药物和心脏复律均无法有效终止SVT。基线心电图证实存在双房室结；然而，该SVT被发现是具有增强自律性的局灶性房性心动过速（AT）。AT的起源与手术瘢痕无关。在我们的无脾综合征病例中，紧急进行的AT射频导管消融术取得了成功。AT在单心室中是一种危及生命的并发症，延迟治疗可能致命。当药物治疗无效时，及时进行RFCA很重要。我们建议，在无脾综合征和双房室结患者中，房室结并不总是消融的目标部位。<在患有无脾和双房室结的室上性心动过速病例中，可能会发生房性心动过速（AT）以及房室折返性心动过速。AT在单心室婴儿中是一种危及生命的并发症。如果药物治疗无效，应紧急进行导管消融。在无脾和双房室结患者中，房室结并不总是消融的目标部位。>
