Outcome After Implantation of Cardioverter-Defibrillator in Patients With Brugada Syndrome: A Multicenter Study – Part 2

Study Questions:

What are the rates of appropriate and inappropriate therapy in Brugada Syndrome (BrS) patients enrolled in a multicenter registry?

Methods:

Seventy-eight patients (310 male, ages 46 ± 13 years) with a type 1 BrS electrocardiogram (ECG) pattern implanted with an implantable cardioverter-defibrillator (ICD) (31 for aborted sudden cardiac arrest [SCA], 181 for syncope, and 166 asymptomatic) were included.

Results:

Fifteen patients (4%) were lost to follow-up. During a mean follow-up of 77 ± 42 months, seven patients (2%) died (one due to inappropriate shock [IS]) and 46 patients (12%) had appropriate device therapy (5 ± 5 shocks/patient). Appropriate device therapy rates at 10 years were 48% for patients whose ICD indication was aborted SCA, 19% for those whose indication was syncope, and 12% for the patients who were asymptomatic at implantation. At 10 years, rates of IS and lead failure are 37% and 29%, respectively. IS occurred in 91 patients (24%, 4 ± 4 shocks/patient) because of lead failure (n = 38), supraventricular tachycardia (n = 20), T-wave oversensing (n = 14), or sinus tachycardia (n = 12). Importantly, introduction of remote monitoring as well as programming a high single ventricular fibrillation zone (>210/220 bpm) and a long detection time were associated with a reduced risk of IS.

Conclusions:

Appropriate therapies are more prevalent in symptomatic BrS patients, but are not insignificant in asymptomatic patients (1%/year). Optimal ICD programming and follow-up dramatically reduce IS. However, lead failure remains a major problem in this population.

Perspective:

Asymptomatic patients with BrS experience a relatively low, but not insignificant risk of appropriate ICD therapy. A robust risk stratification scheme for asymptomatic BrS patients is lacking. In the entire cohort of BrS, patients are twice as likely to receive an inappropriate than appropriate shock, mostly due to high risk of lead failure in this young and active population. Optimal ICD programming reduces the inappropriate shock rate.

Clinical Topics: Arrhythmias and Clinical EP, Implantable Devices, Genetic Arrhythmic Conditions, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias

Keywords: Brugada Syndrome, Defibrillators, Death, Shock, Syncope, Tachycardia


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