Key result
ICD successfully terminates symptomatic VT within one month in Lamin C cardiomyopathy with preserved LVEF.
Why the study?
Lamin A/C gene-related cardiomyopathy is linked to progressive heart failure and malignant arrhythmias, but implantable defibrillator use is guided by severe left ventricular dysfunction evidence.
Case Report (n=1)
Supports ICD for symptomatic VT in Lamin C deficiency with preserved EF; leaves open primary prevention role.
Lamin A/C gene-related cardiomyopathy is associated with progressive heart failure and malignant arrhythmias. Current guidelines advise the use of implantable defibrillators to prevent arrhythmogenic sudden cardiac death only in situations where there is evidence of severe left ventricular dysfunction. We describe a case of a woman with genetically confirmed Lamin C deficiency with preserved left ventricular function in whom an implantable defibrillator was inserted and within a month of implantation was used to terminate symptomatic ventricular tachycardia.
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Kevin Kit Ng (2013) conducted a case report in Lamin C deficiency with preserved left ventricular function (n=1). Implantable defibrillator was evaluated on Termination of symptomatic ventricular tachycardia. An implantable defibrillator inserted in a woman with Lamin C deficiency and preserved systolic function successfully terminated symptomatic ventricular tachycardia within one month.
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