Touray Mariama, Ladouceur Magalie, Bouchardy Judith, Schwerzmann Markus, Greutmann Matthias, Tobler Daniel, Engel Reto, Gabriel Harald, Pruvot Etienne, Blanche Coralie, Sekarski Nicole, Rutz Tobias
Service of Cardiology, Heart and Vessel Department, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland.
Adult Congenital Heart Disease Unit, Department of Cardiology, Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Centre de référence des Malformations Cardiaques Congénitales Complexes, M3C, Paris, France.
CJC Pediatr Congenit Heart Dis. 2022 Sep 8;1(6):263-269. doi: 10.1016/j.cjcpc.2022.08.003. eCollection 2022 Dec.
The long-term outcome of adults with repaired total anomalous pulmonary venous connection (TAPVC) is poorly documented. Therefore, the present study aims to provide current clinical data on adult survivors with repaired TAPVC focusing on arrhythmia.
Clinical and imaging data (prevalence and type of arrhythmias, symptoms, surgical and medical treatment, echocardiographic and cardiac magnetic resonance haemodynamic parameters) were retrospectively collected from 8 European centres and compared between patients with and without arrhythmias.
Fifty-seven patients were included (age 20 [16-67] years [female 28, 49%]). At the last follow-up, that is, 21 (8-51) years after surgery, 79% and 93% of patients were free of symptoms and cardiac medication, respectively. The prevalence of late arrhythmias was 21%; 9 (16%) patients showed intra-atrial re-entrant tachycardia (IART) and 2 (4%) ventricular arrhythmias. Patients with IART were older ( = 0.018) and 4 (7%) required antiarrhythmic medication. Three patients (5%) underwent an electrophysiological study, and another 3 (5%) underwent pacemaker implantation within 36 months after surgical correction, which were removed in 2 patients after 7 years. Early postoperative arrhythmias ( = 0.005), right ventricular dilatation ( = 0.003), and valvulopathy ( = 0.009) were more often present in patients with late IART.
Adult survivors after isolated-TAPVC repair presented a high prevalence of arrhythmias. Age, right ventricular dilatation, early arrhythmias, and valvular lesions are risk factors for IART. Long-term follow-up is important as some of these currently asymptomatic patients will probably develop arrhythmias in the future.
关于接受完全性肺静脉异位连接（TAPVC）修复术的成人患者的长期预后，相关记录较少。因此，本研究旨在提供有关接受TAPVC修复术的成年幸存者的当前临床数据，重点关注心律失常。
回顾性收集了8个欧洲中心的临床和影像学数据（心律失常的患病率和类型、症状、手术和药物治疗、超声心动图和心脏磁共振血流动力学参数），并对有心律失常和无心律失常的患者进行了比较。
纳入了57例患者（年龄20[16 - 67]岁[女性28例，占49%]）。在最后一次随访时，即术后21（8 - 51）年，分别有79%和93%的患者无症状且未服用心脏药物。晚期心律失常的患病率为21%；9例（16%）患者出现房内折返性心动过速（IART），2例（4%）出现室性心律失常。发生IART的患者年龄较大（P = 0.018），4例（7%）需要抗心律失常药物治疗。3例患者（5%）接受了电生理检查，另外3例（5%）在手术矫正后36个月内接受了起搏器植入，其中2例患者在7年后取出了起搏器。晚期IART患者更常出现早期术后心律失常（P = 0.005）、右心室扩张（P = 0.003）和瓣膜病变（P = 0.009）。
孤立性TAPVC修复术后的成年幸存者心律失常患病率较高。年龄、右心室扩张、早期心律失常和瓣膜病变是IART的危险因素。长期随访很重要，因为这些目前无症状的患者中一些将来可能会发生心律失常。
