Zhang Lei, Liu Hongyang, Wan Qilin, Han Xinqiang
Department of Cardiology, Huaihe Hospital, Henan University College of Medicine, Kaifeng, Henan 475000, China.
Cardiovascular Division, Reid Health, Indiana University School of Medicine, 1100 Reid Parkway, Richmond, IN 47374, USA.
Eur Heart J Case Rep. 2019 Oct 30;3(4):1-6. doi: 10.1093/ehjcr/ytz199. eCollection 2019 Dec.
Congenitally corrected transposition of the great arteries (CCTGA) is a rare form of congenital heart disease which may present with sudden death from malignant arrhythmias including complete heart block and ventricular tachyarrhythmias as late complications. Only few cases about ventricular tachyarrhythmias, usually in those with markedly depressed systemic ventricular function, have been reported.
A 26-year-old woman with a known history of CCTGA presented to the emergency department with palpitations and breathlessness for 3-4 weeks and worsening symptoms for 8 h. She had a history of ventricular septal defect repair 14 years ago. Her initial presentation electrocardiogram demonstrated high degree atrioventricular block with a ventricular rate of 44 b.p.m. She had two episodes of complete syncope during this hospitalization, both required external defibrillation due to documented bradycardia-dependent ventricular fibrillations. Her two-dimensional echocardiography study confirmed the diagnosis of CCTGA with preserved systolic ventricular function. She underwent urgent temporal pacing wire placement with a paced ventricular rate at 90 b.p.m. Having thoroughly reviewed the arrhythmia events and discussed with the patient about the option of defibrillator vs. pacemaker therapy a decision was made upon her request for dual-chamber pacemaker implantation. She was discharged home uneventfully 3 days after hospital presentation and has been physically active at 3-, 6-, and 9-month follow-ups.
Our case illustrates the individualized clinical decision making in choosing device therapy for a rare congenital heart disease presented with malignant arrhythmia. Careful history taking, open communication, and closely planned long-term follow-up will be essential in caring for such patients.
先天性矫正型大动脉转位（CCTGA）是一种罕见的先天性心脏病，可能会出现恶性心律失常导致的猝死，包括完全性心脏传导阻滞和室性快速心律失常等晚期并发症。仅有少数关于室性快速心律失常的病例报道，通常发生在体循环心室功能明显降低的患者中。
一名26岁有CCTGA病史的女性因心悸和气促3 - 4周，症状在8小时内加重而就诊于急诊科。她14年前有室间隔缺损修复史。她最初的心电图显示高度房室传导阻滞，心室率为44次/分钟。此次住院期间她发生了两次完全性晕厥，均因记录到的心动过缓依赖性心室颤动而需要体外除颤。她的二维超声心动图检查确诊为CCTGA，体循环心室收缩功能保留。她接受了紧急临时起搏电极置入，起搏心室率为90次/分钟。在全面回顾心律失常事件并与患者讨论了除颤器与起搏器治疗的选择后，根据她对双腔起搏器植入的要求做出了决定。住院3天后她顺利出院，在3个月、6个月和9个月的随访中身体状况良好。
我们的病例说明了对于罕见的伴有恶性心律失常的先天性心脏病在选择器械治疗时的个体化临床决策。仔细的病史采集、开放的沟通以及精心规划的长期随访对于护理此类患者至关重要。
