Integrated Management of Malignant Arrhythmias in Pediatric LMNA-Related Cardiomyopathy Using Active-Fixation CRT-D
Yujia Wang1, Huafeng Wang1, Haiyan Xu1
1Department of Cardiology, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Children and Adolescents' Health and Diseases, Hangzhou, China.
Insights
This study presents a novel dual-track strategy for pediatric LMNA-related dilated cardiomyopathy (DCM). Combining cardiac resynchronization therapy with defibrillator (CRT-D) and epicardial ablation effectively manages life-threatening arrhythmias in young patients.
Area of Science:
- Cardiology
- Pediatric Electrophysiology
- Genetic Cardiology
Background:
- Pediatric LMNA-related dilated cardiomyopathy (DCM) presents a high risk of sudden cardiac death due to malignant arrhythmias.
- Standard cardiac resynchronization therapy with defibrillator (CRT-D) implantation is challenging in children due to anatomical limitations affecting lead stability.
Background:
Pediatric LMNA-related dilated cardiomyopathy (DCM) carries extreme risks of malignant arrhythmias and sudden cardiac death. While cardiac resynchronization therapy with defibrillator (CRT-D) is life-saving, small body size and coronary anatomy make lead stability technically challenging.
Case Summary:
An 11-year-old boy with LMNA-related DCM (c.1622G>A, p.R541H) experienced ventricular fibrillation arrest requiring extracorporeal membrane oxygenation support. A CRT-D was implanted using an active-fixation quadripolar left ventricular (LV) lead in the coronary vein. Subsequent refractory ventricular tachycardia was managed with epicardial radiofrequency ablation. Postoperatively, LV ejection fraction improved from 16% to 35.9%, and QRS duration narrowed from 150 to 120 ms. No arrhythmia recurrence was observed at the 3-month follow-up.
Discussion:
Adapting adult active-fixation technology for the pediatric anatomy ensures lead stability. An integrated "dual-track" strategy-combining resynchronization with epicardial ablation-effectively manages the malignant electromechanical phenotype in high-risk LMNA carriers.
Take-Home Message:
Active-fixation LV leads, integrated with epicardial ablation, provide a stable and effective "dual-track" strategy for managing malignant arrhythmias in pediatric LMNA-mutation carriers.
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