1
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! Chief Medical Officer and Vice President, NewCardio, Inc.
Clinical Professor of Medicine, UMDNJ-RWJ Medical School, New Jersey, USA.
• ECG phenomena of Early Ventricular Repolarization (EVR) have often been
misdiagnosed, misinterpreted, or undermined.
This happened mainly
because of prevailing opinion of the “benign” or “misleading” nature of various
EVR phenomena:
– Early repolarization changes consistent with Brugada syndrome have
been interpreted “innocent” and overlooked for decades - until 1992
– “Early repolarization syndrome” has been regarded as “normal”,
“normal variant”, “benign early repolarization” - until 2000
•
Clinical interest in EVR has been rekindled recently mainly because of:
– “Pre-clinical” concern about “universally and unequivocally benign”
nature of ERS (2000)
– Clinically established association with between ERS (in otherwise
healthy individuals with no (or minimal) structural diseases of the
heart) and fatal cardiac arrhythmias (2008)
• J-point:
– point at which there is abrupt transition from the QRS complex to the
ST-segment
• J-deflection:
– deviation of the J-point from the isoelectric line; common ECG feature of
ERS, acute myocardial ischemia, hyperkalemia, and various IVCD
• QRS-notching:
– J-deflection inscribed on either the downsloping limb of the QRS
complex or S wave
2
•
•
QRS-slurring:
– smooth and prolonged transition from the QRS segment to the ST
segment.
J-wave (Hypothermic, Non-hypothermic, and Idiopathic):
– Increased amplitude and duration of the J-deflection that takes the
shape of a “dome or a hump”
1. Different ECG phenomena of the EVR often share similar ECG presentations,
yet their mechanisms and arrhythmogenic potentials - different
– ECG similarities between different ECG phenomena of EVR raise
certain concerns for their misdiagnosis, misinterpretation, and clinical
relevance
– Standard 12L ECG is very valuable diagnostic tool to identify ERS
subjects, yet its clinical utility in the risk stratification is of limited
intrinsic value
2. ERS should not be regarded as either benign or malignant, unless otherwise
proven
– Clinical judgment based on clinical presentation, family history or
syncope/SCD, potential use of cardio-active drugs is the most essential
in the risk stratification in ERS
– Additional diagnostic work-up, such as tilt-test, signal-averaging ECG,
and EPS with or without drug testing, should be considered on the
case-by-case basis upon physician’s discretion
– Genetic screening of ERS subjects at risk could be one of the most
important diagnostic tools to identify and/or confirm the diagnosis of
primary electrical abnormalities/diseases of the EVR and risk
stratification
3. ERS subjects might be predisposed to the drug-induced ventricular
arrhythmias
4. New ECG/VCG technologies should be developed and clinically validated to
help to identify, differentiate ERS from other forms of EVR phenomena, and
stratify this risk
3
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ĥ11Ħ
Director of Cardiac Hospitalization at the Sourasky Tel-Aviv Medical center, Israel.
Associate Professor of Cardiology, Sackler School of Medicine, Tel Aviv University.
It is now 20 years since we published the first review on idiopathic ventricular
fibrillation. Many of the things we know about idiopathic VF were already described
in that review (Viskin & Belhassen. Idiopathic Ventricular fibrillation. Am Heart J
1990). These include the patients' demographic characteristics, their tendency to
develop arrhythmic storms and the excellent therapeutic profile of quinidine.
However, great advances in our understanding of this disease occurred since then: 1)
In 1996 we described the characteristic mode of onset of ventricular fibrillation in
these patients, always triggered by a ventricular extrasystoles with very short
coupling interval (ultimately associated with His-Purkinje extrasystoles by
Haissaguerre many years later); 2) by the year 2000 we realized that 21% of the
patients we had been calling "idiopathic VF" had in fact what we now know as
Brugada syndrome; 3) in 2004 we proposed that some of the patients with idiopathic
VF have , in fact, a congenital short QT syndrome "with not so short" QT intervals.
Finally, the association between the early repolarization pattern with J-point
elevation and idiopathic VF was established in 2008.
12