ICDs life-saving—but risky—for children with Brugada syndrome

Roughly 1 in 4 children with Brugada syndrome treated with implantable cardioverter-defibrillators (ICDs) received life-saving shock therapy in a single-center study of 35 patients published in the Journal of the American College of Cardiology. However, 20 percent experienced inappropriate shocks and another 14 percent had device-related complications, highlighting the challenging clinical decision-making in this high-risk group.

“These 2 undesirable events occur despite very careful device implantation and programming in experienced hands,” wrote lead researcher M. Cecilia Gonzalez Corcia, MD, and colleagues. “It is thus extremely important to evaluate every patient on an individual basis and discuss with the patient and the family the benefits and potential risks associated with this therapy. Long-term follow-up studies are needed to assess the value of diagnostic electrophysiological testing and of specific genetic mutations in this population.”

Brugada syndrome was first described 25 years ago. The inherited condition is typically asymptomatic until adulthood but severe cases are evident during childhood and can lead to life-threatening ventricular arrhythmias and sudden cardiac death.

Due to its relative newness and rarity—it affects roughly five in 10,000 people worldwide—there has been little research on clinical outcomes, particularly for children.

Gonzalez Corcia et al. studied 35 consecutive patients at their center in Brussels, Belgium, who underwent implantation of an ICD before the age of 20. The mean age at diagnosis was 10.1 years and the average age at ICD implantation was 13.9 years.

During a mean follow-up period of 88 months after implantation:

  • ICDs treated sustained ventricular arrhythmias in nine patients (26 percent), including eight with shock therapy and one with antitachycardia pacing.
  • Three patients (9 percent) died in an electrical storm, defined as at least three sustained episodes of ventricular arrhythmias with or without appropriate ICD interventions during a 24-hour period.
  • Seven patients (20 percent) experienced inappropriate shocks, and five patients (14 percent) had device-related complications, including three related to a fracture of the ventricular electrode.

In an accompanying editorial, Peter F. Aziz, MD, with the division of pediatric cardiology at the Cleveland Clinic, urged his colleagues to be judicious in implanting ICDs in young patients with Brugada syndrome. He pointed out the Gonzalez Corcia et al.’s hospital only placed ICDs in 16 percent of pediatric patients diagnosed with the condition during the 25-year study.

“As pediatric electrophysiologists, it is important to keep these proportions under consideration when we reflect on the treatment of our own BrS patients,” Aziz wrote. “If one is substantially exceeding the 16 percent implant proportion in this article in their own BrS population, prompt reconsideration of institutional risk stratification algorithms should ensue.”

The researchers said there was a trend toward fewer device complications after 2010, likely as a result of improvements in device technology and programming algorithms. Even so, Aziz said it is “inappropriate and unethical to implant a device without detailing the potential for adverse outcomes.”

“Despite the fact that ICD therapy is the only proven intervention to prevent sudden cardiac death, overzealous implantation should be avoided at all costs,” he wrote. “In the current state of device technology, even in carefully selected at risk patients as described in this study, the dark side of implantation is unavoidable.

“The decision to implant an ICD in a pediatric patient is a team sport, consisting of the implanting physician/surgeon, the pediatric patient, and the family. Only programs that are equipped to provide pediatric-specific social support and mental health care are appropriate to provide this subspecialized treatment modality.”