Dalili Mohammad, Dastafshan Marieh, Mahdavi Mohammad, Shahzadi Hossein, Mortaz Hejri Golnar, Rashidighader Fariba, Brugada Pedro
Rajaie Cardiovascular, Medical, and Research Center, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.
Cardiovascular Division, Free University of Brussels (UZ Brussel) VUB, Brussels, Belgium.
Pediatr Cardiol. 2024 Oct 21. doi: 10.1007/s00246-024-03684-6.
Children diagnosed with dilated cardiomyopathy (DCM) are at an increased risk of developing arrhythmias, which can significantly affect their overall prognosis. However, the implantation and effectiveness of implantable cardioverter-defibrillators (ICDs) pose challenges in the pediatric population. Therefore, it is crucial to determine the prevalence of arrhythmia-related death in pediatric patients with DCM. This study investigated the correlation between arrhythmias and mortality in pediatric patients with DCM. This retrospective cohort study examined children with idiopathic DCM referred to Rajaie Cardiovascular, Medical, and Research Institute in Tehran, Iran, from 2017 to 2023. All patients underwent a standard 12-lead electrocardiogram, 24-h Holter monitoring, cardiac magnetic resonance imaging, and echocardiography. Patient outcomes, including death, heart transplantation, and survival, were evaluated within one year of Holter monitoring. A total of 178 children (79 males) with a mean age of 83.06 ± 56.41 months were included in the study. Overall, 72 deaths (40.4%) and 46 heart transplants (25.8%) were recorded and 60 patients (33.7%) survived. There was no significant association between patients' outcomes with ventricular arrhythmias, late gadolinium enhancement, corrected QT interval, and QRS interval prolongation; however, a significantly higher proportion of dead patients had abnormal heart rate variability (P = 0.034). Heart failure was the leading cause of death, with ventricular arrhythmia accounting for two out of 72 deaths. Both cases of ventricular arrhythmia were associated with severe cardiac dysfunction. Arrhythmia-related mortality is rare in children with idiopathic DCM, with severe heart failure being the primary cause of death in this population. Life-threatening arrhythmias primarily occur in DCM children with more compromised cardiac function.
被诊断为扩张型心肌病（DCM）的儿童发生心律失常的风险增加，这可能会显著影响其总体预后。然而，植入式心脏复律除颤器（ICD）的植入和有效性在儿科人群中存在挑战。因此，确定DCM儿科患者心律失常相关死亡的发生率至关重要。本研究调查了DCM儿科患者心律失常与死亡率之间的相关性。这项回顾性队列研究检查了2017年至2023年转诊至伊朗德黑兰拉贾心血管医学和研究所的特发性DCM儿童。所有患者均接受了标准12导联心电图、24小时动态心电图监测、心脏磁共振成像和超声心动图检查。在动态心电图监测的一年内评估患者的结局，包括死亡、心脏移植和存活情况。共有178名儿童（79名男性）纳入研究，平均年龄为83.06±56.41个月。总体而言，记录到72例死亡（40.4%）和46例心脏移植（25.8%），60例患者（33.7%）存活。患者结局与室性心律失常、延迟钆增强、校正QT间期和QRS间期延长之间无显著关联；然而，死亡患者中心率变异性异常的比例显著更高（P = 0.034）。心力衰竭是主要死因，72例死亡中有2例是室性心律失常所致。这两例室性心律失常均与严重的心功能不全有关。在特发性DCM儿童中，心律失常相关死亡率罕见，严重心力衰竭是该人群的主要死因。危及生命的心律失常主要发生在心脏功能受损更严重的DCM儿童中。
