Wallet Justin, Kimura Yoshitaka, Blom Nico A, Jongbloed Monique R M, Bertels Robin A, Hazekamp Mark G, Zeppenfeld Katja
Department of Cardiology, Heart Lung Centre, Leiden University Medical Centre, Leiden, the Netherlands; Willem Einthoven Centre of Arrhythmia Research and Management (WECAM), Leiden, the Netherlands and Aarhus, Denmark; Center for Congenital Heart Disease Amsterdam-Leiden (CAHAL), Utrecht, the Netherlands.
Center for Congenital Heart Disease Amsterdam-Leiden (CAHAL), Utrecht, the Netherlands; Department of Pediatric Cardiology, Leiden University Medical Centre, Leiden, the Netherlands.
JACC Clin Electrophysiol. 2024 Dec;10(12):2613-2624. doi: 10.1016/j.jacep.2024.07.016. Epub 2024 Sep 25.
Patients with repaired tetralogy of Fallot (rTOF) have a time-dependent increased risk of ventricular tachycardia (VT). Slow conducting anatomical isthmuses (SCAIs) are the dominant VT substrates in adults with rTOF. It is unknown if they are present at younger age.
This study aimed to characterize VT substrates in patients with rTOF <30 years of age.
Data of consecutive patients with rTOF aged <30 years who underwent electroanatomical mapping and programmed electrical stimulation between 2005 and 2022 were analyzed.
Fifty-five patients were included (median age: 15.8 years, IQR: 13.8-21.8 years; 15 repaired via ventriculotomy; 13 complex TOF variants). Twelve patients had right ventricle-to-pulmonary artery conduits inserted during initial repair or had early pulmonary valve replacement (PVR) (<1 year after repair). Indications for electroanatomical mapping and programmed electrical stimulation were spontaneous VT, before PVR, and risk stratification in 5, 40, and 10 patients, respectively. In 16 patients (29%), SCAI 3 was identified; no other SCAI was present. Monomorphic VT was inducible in 8 and related to SCAI 3 in 7 patients. Identified VT substrates were targeted by ablation. Right ventricle-to-pulmonary artery conduit/early PVR, ventriculotomy, and complex TOF were associated with SCAI 3 in univariable analysis. During a median follow-up of 5.3 years, VT recurred in 2 patients. No patients died.
In young patients with rTOF, SCAI 3 is the dominant substrate for VT. Complex TOF and interrelated type and timing of (re-)operation may contribute to the development of SCAI 3 already at a young age.
法洛四联症修复术后（rTOF）患者发生室性心动过速（VT）的风险随时间增加。缓慢传导的解剖峡部（SCAIs）是成年rTOF患者VT的主要基质。目前尚不清楚它们在较年轻患者中是否存在。
本研究旨在描述年龄<30岁的rTOF患者的VT基质特征。
分析了2005年至2022年间连续接受电解剖标测和程控电刺激的年龄<30岁的rTOF患者的数据。
纳入55例患者（中位年龄：15.8岁，IQR：13.8 - 21.8岁；15例经心室切开术修复；13例为复杂型法洛四联症变异型）。12例患者在初次修复期间插入了右心室至肺动脉导管或早期进行了肺动脉瓣置换术（PVR）（修复后<1年）。电解剖标测和程控电刺激的指征分别为5例患者的自发性VT、40例患者在PVR前以及10例患者的风险分层。16例患者（29%）被识别出SCAI 3；未发现其他SCAI。8例患者可诱发单形性VT，其中7例与SCAI 3相关。识别出的VT基质通过消融进行靶向治疗。在单因素分析中，右心室至肺动脉导管/早期PVR、心室切开术和复杂型法洛四联症与SCAI 3相关。中位随访5.3年期间，2例患者VT复发。无患者死亡。
在年轻的rTOF患者中，SCAI 3是VT的主要基质。复杂型法洛四联症以及（再）手术的相关类型和时机可能导致在年轻时就出现SCAI 3。
