Work

Integrated Management of Malignant Arrhythmias in Pediatric LMNA-Related Cardiomyopathy Using Active-Fixation CRT-D

Year: 2026

Type: article

Abstract: 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.

Source: JACC Case Reports

DOI: 10.1016/j.jaccas.2026.107762

Authors: Yujia Wang, Huafeng Wang, Haiyan Xu, Fangqi Gong, Ying Zhou, Wei Wang

Institutions: Children's Hospital of Zhejiang University, Zhejiang University, Zhejiang Provincial People's Hospital

Language: English

Topic: Cardiac pacing and defibrillation studies

Subfield: Cardiology and Cardiovascular Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $850