Genetic, molecular and cellular mechanisms underlying the J wave syndromes.

摘要: An early repolarization (ER) pattern in the ECG, distinguished by J-point elevation, slurring of terminal part QRS and ST-segment elevation has long been recognized considered to be a benign electrocardiographic manifestation. Experimental studies conducted over decade ago suggested that some cases ER may associated with malignant arrhythmias. Validation this hypothesis was provided recent demonstrating an inferior or inferolateral leads is increased risk for life-threatening arrhythmias, termed syndrome (ERS). Because accentuated J waves characterize both Brugada (BS) ERS, these syndromes have grouped under term "J wave syndromes". ERS BS share similar ECG characteristics, clinical outcomes factors, as well common arrhythmic platform related amplification Ito-mediated waves. Although differ respect magnitude lead location abnormal manifestation, they can represent continuous spectrum phenotypic expression. most subjects exhibiting are at minimal no risk, mounting evidence suggests careful attention should paid "high risk" ER. The challenge ahead able identify those sudden cardiac death. Here I review genetic aspects cellular molecular mechanisms underlying syndromes. (Circ 2012; 76: 1054-1065)