JACC Case Rep. 2026 Apr 2:107762. doi: 10.1016/j.jaccas.2026.107762. Online ahead of print.
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.
PMID:41931098 | DOI:10.1016/j.jaccas.2026.107762
