Some Controversies about Early Repolarization: The Haïssaguerre Syndrome

Abstract

Controversy has followed the groundbreaking and cornerstone paper of Haïssaguerre et al. Much of this controversy has been due to the use of the term "early repolarization pattern" and possible waveform morphologies on the standard 12-lead ECG ( it is 10 second strip) that could predict who will manifest the malignant arrhythmogenic syndrome described by Haïssaguerre et al. The standard ECG definition of early repolarization pattern (ERP) or early repolarization variant (ERV) since then has changed its clinical meaning for a surface electrocardiographic waveform from benign to malignant. The new definition of ERP/ERV contains only J wave but ST-segment elevation is no more obligatory. In the old definition, early repolarization pattern (ERP) or early repolarization variant (ERV) 3 is a well-recognized idiopathic electrocardiographic phenomenon considered to be present when at least two adjacent precordial leads show elevation of the ST segment, with values equal or higher than 1 mm. In the new electrocardiographic ERP concept, the ST segment may or may not be elevated and can be up-sloping, horizontal or down-sloping while in the old ERP/ERV concept it must be elevated at least 1 mm in at least two adjacent leads and the variant is characterized by a diffuse elevation of the ST segment of upper concavity, ending in a positive T wave of V2 to V4 or V5 and prominent J wave and ST-segment elevation predominantly in left precordial leads. The phenomenon constitutes a normal variant; it is almost a rule in athletes (present in 89% of the cases in this universe).

Primary Electrical Heart Disease—Principles of Pathophysiology and Genetics

“…It is characterized by J-point elevation, often seen as terminal QRS slurring or notching, coupled with ST-segment elevation displaying an upper concavity, along with prominent T-waves in at least two contiguous leads [143]. However, there is ongoing debate and modifications regarding its definition due to inconsistencies in describing ST segment and J-waves, leading to varying interpretations and controversies over the appropriateness of the use of the term "early repolarization" [144,191,192]. Some undermine the use of the term "ST-elevation", highlighting that it might not adequately capture the diversity of ST segment presentations, proposing a focus on detailed J-point changes or expanding the definition to specify early repolarization with ST-segment elevation or terminal slur/notch [144].…”

Diretriz da Sociedade Brasileira de Cardiologia sobre a Análise e Emissão de Laudos Eletrocardiográficos – 2022

“…124 A presença de onda J (espessamento ou entalhe da porção final do QRS) com aspecto retificado do ST em derivações inferiores (isoladamente ou em associação às derivações laterais) pode ser marcador de risco elétrico para o desenvolvimento de taquiarritmias ventriculares. [125][126][127][128][129] Nas últimas décadas, grandes avanços ocorreram relacionados à repolarização ventricular. Dentre eles temos a dispersão da repolarização ventricular como marcador da recuperação não uniforme da excitabilidade miocárdica e o reconhecimento da macro ou da microalternância cíclica da onda T. Deve-se considerar como alterações da repolarização ventricular as modificações significativas na polaridade, na duração, na morfologia dos fenômenos elétricos acima descritos.…”

ST Segment Elevation

“…The slow recovery kinetics of I t0 channel enables its opening during bradycardia while preventing it during tachycardia and thereby explains the dependency of the J wave on the heart rate. Out of congenital J wave syndromes, J wave is sometimes seen in hypertrophic cardiomyopathy and in arrhythmogenic right ventricular cardiomyopathy [123], as well as in a few other conditions denominated together as acquired J wave syndromes: hypothermia, hypercalcaemia, myocardial ischaemia, subarachnoid haemorrhage [123] and myocarditis [22].…”

“…On the other hand, central temperature below 32 °C slows the sinus rate, lengthens PR and QT intervals, widens the QRS complex, and promotes atrial premature ectopic activity (and sometimes atrial fibrillation, too). J wave (=Osborn wave, first detected by Tomaszewski in 1938 in a frozen man [28,131], while discovered by Osborn only in 1953 in dogs shivering with cold [11,121,123]) sees daylight amidst the inferior leads, then spreads towards the lateral ones, to finally fill up the precordial leads, too, and unfolds positive in most of the leads (except aVR and V1, where negative) [123]. The lower the temperature, the higher and more split the J wave from the QRS complex (resembling an iceberg falling magnificently apart from the ice sheet), likely following the enhancement of the subepicardial I t0 current secondary to bradycardia, as well as its activation delay by cold [119].…”

“…The lower the temperature, the higher and more split the J wave from the QRS complex (resembling an iceberg falling magnificently apart from the ice sheet), likely following the enhancement of the subepicardial I t0 current secondary to bradycardia, as well as its activation delay by cold [119]. The J wave enlargement elevates the initial part of the ST segment, whereas the remaining part of the ST segment features an ascending/ horizontal/descending slope, being like the early repolarization pattern [123]. When central temperature falls below 30 °C, the elephantine widening of the QRS complex fosters ventricular fibrillation.…”