The Brugada syndrome. Its relevance to paediatric practice.
The diagnostic and therapeutic aspects of Brugada syndrome, one of the important genetic arrhythmias which causes sudden cardiac death in young people, and the relevance of this diagnostic entity to the paediatric population are briefly summarised. The role of diagnostic testing (genetic, pharmacologic and invasive electrophysiologic) for establishing the diagnosis and for risk stratification are discussed. Finally, while the implantable defibrillator is the only therapy of proven effectiveness in preventing sudden cardiac death, alternative therapies which are being considered (pharmacologic, hormonal and catheter ablation) are overviewed. The aim of this manuscript is to raise the awareness among doctors caring for young patients to the possibility of Brugada syndrome in patients presenting with potentially life-threatening symptoms of syncope or near-miss sudden death, and in index patients with a similar family history.
Sreeram
N
N
Universitätsklinikum Köln, Köln, Germany.
Simmers
T
T
Brockmeier
K
K
D000293:Adolescent; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D001145:Arrhythmias, Cardiac / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D002037:Bundle-Branch Block / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D004562:Electrocardiography; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008133:Long QT Syndrome / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D013398:Sudden Infant Death / Q000209:etiology; D013577:Syndrome; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000235:genetics / Q000628:therapy
D000889:Anti-Arrhythmia Agents