Vătășescu Radu Gabriel, Deaconu Silvia, Iorgulescu Corneliu Nicolae, Marascu Gabriela, Oprita Bogdan, Deaconu Alexandru
Faculty of Medicine, Carol Davila University of Medicine and Pharmacy, 050474 Bucharest, Romania.
Cardiology Department, Clinical Emergency Hospital, 014461 Bucharest, Romania.
J Clin Med. 2024 Jan 30;13(3):804. doi: 10.3390/jcm13030804.
Long QT syndrome (LQT) and WPW syndrome are causes of sudden cardiac death (SCD) in the young, and their association has been rarely reported. A 26-year-old woman presented with recurrent syncope. Her ECG showed a short PR interval, wide QRS (150 ms) due to a delta wave, and QT prolongation (QT 580 ms, QTc 648 ms). ECG monitoring documented recurrent salvos of a self-terminating wide QRS tachycardia, generally slightly polymorphic, sometimes with "torsade des pointes" (TdP) appearance, which were linked to the syncopal/presyncope episodes. Electrophysiologic monitoring diagnosed a right para-hisian accessory pathway with a very short ERP (240 ms baseline, <200 ms after isoproterenol). The pathway was ablated successfully. Despite QRS narrowing (80 ms), QT prolongation persisted after ablation (QT 620 ms, QTc 654 ms), with short runs of TdP, despite beta-blocker treatment, which was increased to the maximal dosage. A dual-chamber implantable cardioverter defibrillator (ICD) was implanted. To our knowledge, this is the first case report of an association between LQT and WPW syndrome in which both conditions are associated with an increased risk of SCD.
长QT综合征（LQT）和预激综合征（WPW）是年轻人心脏性猝死（SCD）的病因，二者关联鲜有报道。一名26岁女性反复出现晕厥。其心电图显示PR间期缩短、因δ波导致QRS波增宽（150毫秒）以及QT间期延长（QT 580毫秒，QTc 648毫秒）。心电图监测记录到反复发作的自限性宽QRS心动过速，通常稍有形态变化，有时呈“尖端扭转型室速”（TdP）表现，这些发作与晕厥/先兆晕厥发作相关。电生理监测诊断为右希氏束旁旁路，有效不应期（ERP）极短（基础值240毫秒，异丙肾上腺素给药后<200毫秒）。该旁路成功消融。尽管QRS波变窄（80毫秒），但消融后QT间期延长仍持续存在（QT 620毫秒，QTc 654毫秒），尽管使用了β受体阻滞剂治疗且剂量增至最大，但仍有短阵TdP发作。遂植入双腔植入式心脏复律除颤器（ICD）。据我们所知，这是首例LQT与WPW综合征相关联且二者均与SCD风险增加有关的病例报告。
