ArticleJACC. Case reports2026
Integrated Management of Malignant Arrhythmias in Pediatric LMNA-Related Cardiomyopathy Using Active-Fixation CRT-D.
Article in JACC. Case reports, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundPediatric 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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