Current clinical and experimental data demonstrate that the electrocardiographic J wave plays a critical role in the pathogenesis of ventricular fibrillation (VF) in patients with Brugada syndrome (BS) and early repolarization (ER) syndrome (ERS). This has generated renewed interest in the presence of J waves and ERS in the general population, yet the identification of high-risk ECG markers and the risk stratification of subjects with ERS remain to be established. More recently, this concept has been expanded to VF mechanisms in patients with structural heart diseases. Some of the fatal arrhythmias in the setting of acute myocardial ischemia or infarction may share a similar, J wave-related electrophysiologic process. In canine arterially perfused wedge preparations, the occurrence of J waverelated arrhythmias is mediated by phase 2 reentry. The stability of the action potential (AP) dome in the ventricular epicardium is dependent on the prominence of the AP phase 1 notch. The ability to maintain the AP dome depends on a delicate balance between inward and outward ionic currents during depolarization and the early phase of repolarization. Outward shifts of the balance and inability to maintain the AP dome result in marked dispersion of repolarization and vulnerability to VF. This review describes the electrocardiographic and clinical features of the J waves in idiopathic VF and other structural heart diseases. (Circ J 2012; 76: 2723 -
2724NAM GB cardioverter defibrillator (ICD) implantation at the Asan Medical Center, Korea. Structural diseases were present in 209 (68%) of 309 ICD recipients. Coronary artery disease or myocardial infarction, and hypertrophic cardiomyopathy were underlying cardiac diseases in approximately 40% of all ICD recipients. Other structural cardiac abnormalities (dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, sarcoidosis, valvular or congenital heart diseases) were present in 28% of these cases. Among the 100 patients without structural heart diseases, long QT syndrome (LQTS), BS and ERS were identified in 10 (3.2%), 29 (9.4%) and 16 (5.2%) of the patients, respectively. After extensive search for an underlying etiology, the diagnosis remained idiopathic in 45 patients (14.5%). Interestingly, after careful review of all the ECGs taken during peri-event periods, provocation, and at the outpatient clinic, a significant proportion (19/45, 42.2%) of the patients whose diagnosis was presumed to be idiopathic showed a J wave fluctuation, which did not satisfy the diagnostic criteria of either BS or ERS. These patients are described later in the section about J wave-related VF syndrome in the absence of structural heart disease (IVF-J). In 26 patients, abnormal findings were not identified in any of the ECGs, and these cases were termed true "idiopathic VF (IVF)".
BSBS is the prototype of cardiac ion-channel diseases, and has opened a new era in translational electrophysiology. It is characterized by unique ECG changes of coved-type J/ST/T waves, and a high risk of ...