This article is more than 5 years old. Information may no longer be current.
Persistent testing warranted for young relatives of patients with Brugada syndrome
Click Here to Manage Email Alerts
Repeated testing for Brugada syndrome via ajmaline challenge may be necessary for asymptomatic individuals with a first-degree relative with Brugada syndrome, even among those considered at low risk due to young age, according to recent results.
Researchers evaluated 53 asymptomatic children with a first-degree relative with Brugada syndrome between 1992 and 2010 at the University Hospital of Brussels. The condition is a hereditary primary arrhythmia syndrome associated with risk for sudden death that can present as early as within the first months of life, but typically emerges within the fourth or fifth decade, the researchers wrote.
All participants had a previous negative ajmaline challenge conducted before age 16 years. Participants underwent annual diagnostic type 1 ECG and were slated to repeat ajmaline challenge after the onset of puberty.
Of the 53 children, nine were younger than 16 years and one presented spontaneously with Brugada syndrome on ECG at age 16 years. Repeat challenge was performed in the remaining 43 children between 2008 and 2013 — at least 3 years after the initial test — with follow-up for those with positive results on repeat testing through February 2014. The mean patient age at the time of repeat testing was comparable between those with positive (21.2 years) and negative (19.3 years) results (P=.15).
Four patients exhibited symptoms before repeat testing, including three cases of syncope and one of nocturnal agonal respiration. The mean age at symptom onset among these patients was 22.5 years. Researchers observed a significantly higher prevalence of positive results on repeat testing among those who developed symptoms compared with those who did not (75% of patients vs. 18%; P=.03).
After follow-up (mean, 42 months; range, 11-67 months), no sudden deaths occurred among patients who tested positive on repeat analysis.
According to the researchers, a patient’s response to ajmaline appears to change after puberty due to hormonal, autonomic or genetic factors.
“Screening of first-degree relatives of patients with Brugada syndrome is advisable, although the ideal timing is unknown,” they wrote. “Relatives developing symptoms should always be investigated with ajmaline challenge, even if they had a negative drug test performed before puberty. These findings support the need for repeat monitoring of family members of patients with Brugada syndrome, including those initially considered at low risk because of young age.”
Disclosure: One researcher reported receiving research grants from Biosense Webster, Biotronik, Medtronic, Sorin-Ela Medical and St Jude Medical, and serving as a teaching consultant to Biotronik, Boston Scientific and Medtronic.
Click Here to Manage Email Alerts