The Clinical Management of Relatives of Sudden Unexplained Death Victims; Implantable Defibrillators Are Rarely Indicated

Study Questions:

What are the outcomes of relatives of young patients with sudden death followed in inherited cardiac condition (ICC) clinics?

Methods:

An observational study of 193 patients (108 families) referred to a regional ICC clinic was performed. All individuals underwent assessment including history, electrocardiogram, and echocardiography. Additional studies such as exercise electrocardiography, ajmaline provocation, and genetic testing were performed in select patients. Patients underwent placement of an implantable cardioverter-defibrillator (ICD) according to national guidelines.

Results:

Of the 193 patients enrolled at the clinic, 45 (23%) were diagnosed with an inheritable cause of sudden death. Eighteen of these patients were started on a new medication as a result of their diagnosis, and four underwent ICD placement. Two patients had ICDs removed after negative testing for familial RYR2 mutations.

Conclusions:

Diagnosis of an inheritable cause of sudden death was relatively common in patients referred to the ICC clinic. The clinic had a major role in the reassurance in patients without disease.

Perspective:

This demonstrates the role of an ICC clinic in evaluating relatives of young sudden unexplained death victims. The clinics appear to streamline the diagnostic approach to these patients and can serve a role in diagnosing disease as well as reassuring those without disease. Importantly, no deaths occurred in patients followed by the clinic, although the follow-up period was relatively short (mean 16.5 months, range 0.1-61 months).

Clinical Topics: Arrhythmias and Clinical EP, Dyslipidemia, Noninvasive Imaging, Implantable Devices, Genetic Arrhythmic Conditions, SCD/Ventricular Arrhythmias, Lipid Metabolism, Echocardiography/Ultrasound

Keywords: Defibrillators, Follow-Up Studies, Genetic Testing, Electrocardiography, Heart Diseases, Mutation, Ryanodine Receptor Calcium Release Channel, Death, Cardiology, Echocardiography, Exercise Test


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