[Syncopal attacks and sudden death due to ventricular fibrillation -- a variant of the Jervell or Lange-Nielsen syndrome (author's transl)].

Syncopal attacks due to ventricular fibrillation or -- flutter with elongation of QT in the ECG and deafness are signs of a congenital condition described by Jervell and Lange-Nielsen 18 years ago. Since then we have learned that the range of variation of symptoms is very wide. There are differences genetically and in age-groups. A boy aged 15 years with supposedly cerebral fits is reported on. Since the right diagnosis was missed he had been treated with anticonvulsants for years. But these were syncopal attacks, Effort produced a tendency to bigeminus. -- A knee-bending exercise repeated 13 times lead to his death from ventricular fibrillation. Post-mortem examination showed no pathologic findings except mild left hypertrophy of the ventricle. His sister also had similar attacks without spasms after excitement or mild physical effort. She too had no extension of the QT interval. Their mother and grandmother also had syncopal attacks and died at the ages of 31 and 24 years. A girl-cousin died suddenly at the age of 15. In addition we have seen a neonate with a mild cardiac murmur on whom an ECG was done on the 16th day. The QT interval was significantly prolonged. There were numerous extrasystoles. He died suddenly 2 days later. Post mortem showed no cardiac pathology. One should now be able to explain some cases of unexpected natural death. But ignorance of the pathogenesis does not yet permit definition of the clinical pictures.