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).

Keywords: Haïssaguerre syndrome; J-wave syndrome; early repolarization; idiopathic ventricular fibrillation.

Figure 1

(A) A 12‐lead ECG of a patient with electrical storm. Diffuse and large J wave, positive in all leads, except aVR and V₁ (negative one) followed by ST‐segment depression and deep negative T wave. (B) ECG before episode of ventricular fibrillation. Large J wave, described above in A. (Thanks to courtesy and permission of Dr. Shogo Ito, Department of Internal Medicine, Division of Cardiovascular Medicine, Kurume University School of Medicine, Japan).

Figure 2

(A) ECG from a patient with IVF (permission from Elsevier, Journal of Electrocardiology). (B) ECG from a patient with hypothermia (permission from Japanese Circulation Society, Circulation Journal). Arrows show a pronounced J wave in all 12‐leads of ECG; a negative J wave in leads aVR and V₁ and positive J wave in rest ECG leads.

Figure 3

(A–C) Different morphology of J wave (black arrows). ECG tracings (lead V₆), from patients with hypothermia. (A) A small J wave, (B) notch >2 mm, (C) large J wave. (D–F) The different morphology of ST segment pattern (in leads V₅ and V₆) in hypothermia: (D) upsloping, (E) horizontal, and (F) downsloping.

Figure 4

(A) ECG from a patient with idiopathic ventricular fibrillation and Haïssaguerre pattern. Dynamicity of a J wave and augmentation of its amplitude after a sudden cycle length change due to sinus pause. A secondary ST segment changes from upsloping or horizontal before the pauses to downsloping after it and T wave changes polarity from a positive before to a negative after the pauses (permission from Elsevier, Journal of Electrocardiology). (B) ECG from a patient with vasospastic angina, with dynamic J waves changes. ECG tracing in turn: (1) before chest pain, (2) onset of pain, (3) immediately before VF episode, (4) after DC shock, (5) 2 days after VF. Black arrows show a dynamicity of J wave amplitude and ST segment and T wave changes. Courtesy of Dr. Mitsunori Maruyama.

Figure 5

Dynamicity of a J wave in a patient with hypothermia. At body temperature (BT) 26°C, the large J wave and marked ST‐segment depression; at BT 28°C, the large J wave and ST‐segment depression; at BT, 28.5°C notch >2 mm J wave and horizontal ST segment; at BT 29°C, slurr‐like J wave and horizontal/ascending ST segment (permission from BMJ Publishing Group Ltd.).

Figure 6

The peculiar J wave—ST segment–T wave pattern—called “a lambda wave”: (A) in a patient with IVF (permission from Elsevier, Journal of Electrocardiology), (B) in a patient with vasospastic angina (permission from Oxford University Press, Europace), and (C) in a patient with electrical storm and ST segment elevation myocardial infarction.

Figure 7

(A) ECG of a patient with HCM and ER pattern. (B) ECG of a patient with genetically confirmed LQTS type 2 and ER pattern.