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.
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.
hen patients initially present with atrial fibrillation along with an enlarged heart and heart failure, followed by atrioventricular block, it's essential to consider genetic factors. 1enetic testing can offer crucial diagnostic evidence, aiding in prognosis assessment and the adoption of appropriate treatment strategies.A 61-year-old male patient was admitted to our hospital on December 18, 2023, complaining of "sudden palpitations over the past 12 years, worsened by chest tightness for the last 9 days".In 2011, he sought treatment at our hospital for sudden palpitations accompanied by left limb weakness, and was diagnosed with "atrial flutter" during an outpatient visit.He was also diagnosed with "cerebral infarction" and was prescribed oral warfarin for anticoagulation and antiarrhythmic drugs to control fast ventricular rate.By August 2020, his cardiac symptoms had worsened, with fatigue and decreased endurance during physical activity.He experienced shortness of breath after walking just 600 meters.An electrocardiogram (ECG) performed at our hospital revealed atrial fibrillation, complete atrioventricular block, and an escape rhythm with a widened QRS complex (Figure 1).Transthoracic echocardiography (TTE) showed overall enlargement of the heart, widening of the pulmonary artery, severely reduced left ventricular function with an estimated ejection fraction of 30%, moderate to severe leakage of the tricuspid valve, mild to moderate leakage of the pulmonary valve, and moderately elevated pressure in the pulmonary artery.On August 19, 2019, he underwent permanent implantation of an dual chamber implantable cardioverter defibrillator (ICD) (Medtronic DDBC3D4).An ventricular electrode was placed in the region of the left bundle branch, with significant fibrosis observed during implantation, and a
Xie et al. (Thu,) 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.