ArticleEuropean heart journal. Case reports2022
A case report of successful physiological pacing in a patient with lamin A/C cardiomyopathy.
Article in European heart journal. Case reports, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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3 authors at 1 institution in 1 country.
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Abstract
Background: Lamin A/C (LMNA) mutations account for 5-8% of familial dilated cardiomyopathies, and can manifest with conduction abnormalities and ventricular arrhythmias in 78% of patients. Therefore, when suspected, it is important to implant the correct type of device. Case summary: A 52-year-old gentleman with a family history of cardiomyopathy, presented with asymptomatic atrial fibrillation and complete atrioventricular block associated with a narrow QRS interval. Investigations confirmed dilated and severely impaired left ventricular systolic function. He underwent successful conduction system pacing in combination with a primary prevention defibrillator. Genetic screening confirmed LMNA cardiomyopathy. During 3 years follow up, his left ventricular function remained unchanged with stable conduction system capture and he received appropriate therapy from his device for ventricular tachycardia. Discussion: His-bundle pacing promotes rapid and synchronous activation of the ventricles via the intrinsic conduction system of the heart. In selected individuals with LMNA cardiomyopathy, conduction system pacing is viable alternative to conventional cardiac resynchronization therapy using coronary sinus tributaries.
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