Diagnosis and management of pediatric brugada syndrome: a survey of pediatric electrophysiologists.
BACKGROUND
Brugada syndrome (BrS) can be difficult to diagnose and treat, especially in the young patient. As there is currently no consensus on the evaluation and treatment of BrS in the pediatric population, we sought to describe the current practice for the diagnosis and treatment of BrS among pediatric electrophysiologists.


METHODS
A web-based survey was distributed to 204 physician members (MDs) of The Pediatric and Adult Congenital Electrophysiology Society (PACES). Practice characteristics, BrS patient attributes, and diagnostic and therapeutic preferences were collected.


RESULTS
Responses were obtained from 83 pediatric electrophysiologists. The most common initial presentation was family history. There is a large variation in testing, particularly in the use of electrophysiology (EP) studies, drug challenge testing, and genetic testing. Despite limited treatment options, there is only consensus in the therapeutic approach to the pediatric patient with symptomatic BrS with 97% of physicians recommending an implantable cardioverter defibrillator (ICD). In the asymptomatic patient, a wide variation in therapy was seen with only 27% of physicians recommending an ICD CONCLUSIONS: Significant practice variation exists among pediatric electrophysiologists with deviation from accepted diagnostic and therapeutic practices for adult BrS patients. Further studies are necessary to establish best practice guidelines for BrS in the pediatric EP community.
Division of Pediatric Cardiology, Lucile Packard Children's Hospital at Stanford, Palo Alto, California.
Bronwyn U
BU
Harris
Christina Y
CY
Miyake
Kara S
KS
Motonaga
Anne M
AM
Dubin
D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use*; D053840:Brugada Syndrome / Q000175:diagnosis* / Q000628:therapy*; D054849:Cardiac Electrophysiology / Q000706:statistics & numerical data*; D002648:Child; D017147:Defibrillators, Implantable / Q000706:statistics & numerical data*; D022062:Electrophysiologic Techniques, Cardiac / Q000706:statistics & numerical data*; D005260:Female; D019538:Health Care Surveys; D006801:Humans; D038622:Internationality; D008297:Male; D010372:Pediatrics / Q000706:statistics & numerical data*; D010818:Practice Patterns, Physicians' / Q000706:statistics & numerical data
D000889:Anti-Arrhythmia Agents
Brugada syndrome; arrhythmia; diagnosis; pediatrics; survey; treatment