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Familial dilated cardiomyopathy and spontaneous ventricular arrhythmias
L Jordaens1, M de Pauw, F Caes
1Department of Cardiology, University Hospital Ghent, Belgium.
Insights
Dilated cardiomyopathy patients with ventricular arrhythmias may require advanced therapies beyond implantable cardioverter-defibrillators (ICDs). Beta-blockers like bisoprolol can help manage symptoms and prevent shocks, but individual responses vary, sometimes necessitating heart transplantation.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Autosomal dominant dilated cardiomyopathy (DCM) can present with symptomatic ventricular tachyarrhythmias.
- Implantable cardioverter-defibrillators (ICDs) are a standard treatment for preventing sudden cardiac death in such patients.
Observation:
- Two family members with DCM and ventricular arrhythmias were treated with ICDs.
- One patient experienced amiodarone-induced torsades de pointes with ICD failure and recurrent cardiac arrest.
- The second patient experienced syncope due to non-sustained ventricular tachycardia, with rapid functional decline.
Findings:
- Bisoprolol improved functional status and reduced ICD shocks in the first patient, delaying the need for transplantation.
- Bisoprolol was not tolerated by the second patient, who underwent transplantation 16 months post-ICD implantation despite no shocks delivered by the device.
Implications:
- ICD therapy may have limitations in managing specific ventricular arrhythmias in DCM.
- Beta-blocker therapy, such as bisoprolol, can be a valuable adjunct but requires careful patient selection and monitoring.
- Genetic factors in DCM may influence arrhythmia characteristics and treatment response, highlighting the need for personalized management strategies.
Abstract:
Two members of a family with (autosomal dominant) dilated cardiomyopathy and symptomatic short-lasting ventricular tachyarrhythmias were each treated with an ICD in the course of their disease. One patient had an episode of torsades de pointes induced by amiodarone, and the ICD failed to recognize some events. Cardiac arrest recurred in this setting. Treatment with bisoprolol was helpful in maintaining an acceptable functional status and in preventing multiple shocks until transplantation became mandatory. Bisoprolol was not tolerated by the second patient, who had several episodes of syncope because of nonsustained ventricular tachycardia. His functional course went downhill fast, and he received a heart transplantation 16 months after implantation of an ICD, which had not delivered any shocks, in spite of one symptomatic short ventricular tachycardia.