Key result
ICD with LBBP fails to prevent electrical storm in LMNA cardiomyopathy despite initial QRS narrowing.
Why the study?
When patients present with atrial fibrillation, cardiomegaly, heart failure, and subsequent atrioventricular block, genetic factors must be considered to aid prognosis and treatment strategies.
Case Report (n=1)
No
In patients presenting with atrial fibrillation, heart enlargement, heart failure, and subsequent atrioventricular block, genetic testing for LMNA mutations is crucial for accurate diagnosis and guiding management.
Pursue LMNA testing in AF, enlarged heart, HF and AV block; challenges LBBP efficacy in preventing electrical storm.
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Xie et al. (2026) conducted a case report in 61-year-old male patient with atrial fibrillation, complete atrioventricular block, dilated cardiomyopathy with NYHA Class III heart failure, and recurrent ventricular tachycardia typical of LMNA cardiomyopathy (n=1). Dual chamber implantable cardioverter defibrillator (ICD) with left bundle branch area pacing and amiodarone (later discontinued), propranolol, anti-heart failure drugs was evaluated on Reduction/prevention of malignant arrhythmias and heart failure progression after ICD implantation and pharmacotherapy. Dual chamber ICD implantation with left bundle branch pacing initially reduced QRS duration by 10 ms but did not prevent progression to electrical storm in a patient with LMNA cardiomyopathy.
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