Prolonged QT interval and sudden infant death - report of two cases

In the two cases where infants died suddenly and unexpectedly the electroca rdiogram (ECG) of a younger sibling (case 1) and of a living twin (case 2) led to the suspicion that the two infants could have died from long QT synd rome (LQTS). In case 1, a His bundle (HB) dispersion and a pronounced hypop lasia of the right external nucleus arcuatus were detected. In case 2, a se vere interstitial pneumonia and an accompanying mild myocarditis were found by histology. Molecular genetic investigations of the coding regions of the genes, HERG, KVLQT1 and SCN5A gave no indication for the mutations, thus, affecting rela ted myocardial ion channels as possible sources of inhomogeneity of repolar isation. Since a molecular genetic deviation could not yet be elaborated the possibl e role of related disturbance remains unknown. (C) 2001 Elsevier Science Ir eland Ltd. All rights reserved.