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Review
. 2017 Apr 1;19(4):665-694.
doi: 10.1093/europace/euw235.

J-Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge

Charles Antzelevitch  1 , Gan-Xin Yan  2 , Michael J Ackerman  3 , Martin Borggrefe  4 , Domenico Corrado  5 , Jihong Guo  6 , Ihor Gussak  7 , Can Hasdemir  8 , Minoru Horie  9 , Heikki Huikuri  10 , Changsheng Ma  11 , Hiroshi Morita  12 , Gi-Byoung Nam  13 , Frederic Sacher  14 , Wataru Shimizu  15 , Sami Viskin  16 , Arthur A M Wilde  17
Affiliations
Review

J-Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge

Charles Antzelevitch et al. Europace. .
No abstract available

Keywords: Brugada syndrome; Cardiac arrhythmia; Early repolarization syndrome; Inherited cardiac arrhythmia syndrome; J wave; Sudden cardiac death; Ventricular fibrillation.

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Figures

Figure 1
Figure 1
Three types of ST-segment elevation associated with Brugada syndrome. Only type 1 is diagnostic of Brugada syndrome.
Figure 2
Figure 2
Schematic showing overlap syndromes resulting from genetic defects resulting in loss of function of sodium channel current (INa) or gain of function in Late INa. In the absence of prominent Ito or IK-ATP, loss-of-function mutations in the inward currents result in various manifestations of conduction disease. In the presence of prominent Ito or IK-ATP, loss-of-function mutations in inward currents cause conduction disease as well as the J-wave syndromes (Brugada and early repolarization syndromes). Early repolarization syndrome is believed to be caused by loss-of-function mutations of inward current in the presence of prominent Ito in certain regions of the left ventricle, particularly the inferior wall of the left ventricle. The genetic defects that contribute to Brugada syndrome and early repolarization syndrome can also contribute to the development of long QT and conduction system disease, in some cases causing multiple expressions of these overlap syndromes. In some cases, structural defects contribute to the phenotype. PVT = polymorphic ventricular tachycardia; VF = ventricular fibrillation.
Figure 3
Figure 3
Different manifestations of early repolarization. A: The J wave may be distinct or appear as a slur. In the latter case, part of the J wave is buried inside the QRS, resulting in an elevation of Jo. Patients with a distinct J wave have a worse prognosis than do patients with a slurred J wave. B: The ST segment may be upsloping, horizontal, or descending. Horizontal and descending ST segments are associated with a worse prognosis.
Figure 4
Figure 4
Prevalence and arrhythmic risk associated with the appearance of ECG J waves and clinical manifestations of Brugada and early repolarization syndromes. Yellow highlighted region estimates the prevalence of the J-wave syndromes. J waves in the lateral ECG leads have a high prevalence but are associated with a very low arrhythmic risk in a relatively small fraction of the cohort of individuals displaying J waves. On the other extreme, J waves appearing globally in the ECG have a very low prevalence but are associated with a very high level of arrhythmic risk in a large fraction of the cohort presenting with J waves. Likewise, individuals displaying rapidly ascending ST-segment elevation have a high prevalence but low risk, whereas subjects resuscitated from cardiac arrest have a very low prevalence but the highest level of arrhythmic risk.
Figure 5
Figure 5
Global early repolarization (type 3 early repolarization). J waves are apparent in the inferior, lateral, and anterior (right precordial) leads.
Figure 6
Figure 6
Indications for therapy of patients with Brugada syndrome. Recommendations with class designations are taken from Priori SG, Wilde AA, Horie M, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm 2013;10:1932–1963, and Priori SG, Blomstrom-Lundqvist C, Mazzanti A, et al. 2015 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC). Eur Heart J 2015;36:2757–9. Recommendations without class designations are derived from unanimous consensus of the authors. ES = extrastimuli at right ventricular apex; ICD = implantable cardioverter-defibrillator; ILR = implantable loop recorder; NAR = nocturnal agonal respiration; RVOT = right ventricular outflow tract; VF = ventricular fibrillation; VT = ventricular tachycardia.
Figure 7
Figure 7
Indications for therapy of patients with early repolarization syndrome. Recommendations with Class designations are taken from Priori SG, Wilde AA, Horie M, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm 2013;10:1932–1963, and Priori SG, Blomstrom-Lundqvist C, Mazzanti A, et al. 2015 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC). Eur Heart J 2015;36: 2757–9. Recommendations without Class designations are derived from unanimous consensus of the authors. ER = early repolarization; ICD = implantable cardioverter-defibrillator; ILR = implantable loop recorder; NAR = nocturnal agonal respiration; VT = ventricular tachycardia.
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