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A case report of successful physiological pacing in a patient with lamin A/C cardiomyopathy.


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 unch

SUBMITTER: Chandra Mohan N 

PROVIDER: S-EPMC9426304 | biostudies-literature | 2022 Aug

REPOSITORIES: biostudies-literature