Vox clamantis in deserto. We spoke but nobody was listening: echocardiography can help risk stratification of the long-QT syndrome

This editorial refers to ‘Electromechanical window negativity in genotyped long-QT syndrome patients: relationship to arrhythmia risk’[†][1], by R.M.A. ter Bekk et al ., on page 179 Over 20 years ago we reported for the first time the presence of mechanical abnormalities in the contraction pattern of patients with the long QT syndrome (LQTS), showing the presence both of rapid early contraction and of an extended ‘plateau’ phase clearly visible at M-mode Doppler before rapid relaxation.1 These abnormalities were almost absent in controls and more prevalent among symptomatic than asymptomatic patients [77% vs. 19%, relative risk (RR) 2.75], suggesting their potential value for diagnosis and risk stratification of LQTS patients. This report and our subsequent evidence that these abnormalities were abolished by calcium blockers2 and could be the mechanical counterparts of early afterdepolarizations (EADs), thus being markers of arrhythmic propensity, were received with scepticism and essentially ignored. Eventually, an abnormal contraction pattern was confirmed by others, mostly using tissue Doppler imaging,3–5 thus ending the era in which LQTS was considered a pure electrical disease.6 Once set in motion, the ball keeps rolling. Now ter Bekk et al. 7 describe the presence of a negative ‘electromechanical window’ (EMW) in LQTS patients and report a strong association between a markedly negative value and arrhythmic events. One strength lies in the size of the population, almost 250 genotype-positive LQTS patients and 74 controls, much larger than previous studies.1–5 This allowed the authors … [1]: #fn-2