Bruuske syncope in een pashokje

Abstract

Sudden syncope in a fitting room A 15-year-old girl experienced a sudden syncope. The cardiac evaluation demonstrated the diagnosis of Brugada syndrome (BrS). The differential diagnosis of a syncope is broad. In BrS, ventricular arrhythmia may be the cause of the transient loss of consciousness. BrS was first described in 1992 in patients with cardiac arrest due to ventricular arrhythmia and with characteristic ST segment elevation in the right precordial leads. The diagnosis of BrS is made on a spontaneous type 1 electrocardiogram in the absence of underlying cardiac disease or on the onset of a type 1 electrocardiogram after provocative testing or fever and in the presence of documented polymorphic ventricular tachycardia or ventricular fibrillation, an arrhythmogenic syncope or a suggestive family history. The disease belongs to the channelopathies, in which mutations in the SCN5A gene are found in 1/3 of the cases. Its treatment is aimed at avoiding ventricular arrhythmias and sudden cardiac arrests. An implantable cardioverter-defibrillator is used in patients with a high risk for sudden cardiac arrests. In addition, drug therapy and/or an ablation may be considered. BrS is a genetic disorder in which an important role is attributed to the cardiac sodium channel. Making the right diagnosis and performing a correct risk stratification remain the main challenges.