[Sympathetic ganglionitis in the hereditary syndrome of QT prolongation (Romano-Ward syndrome); intracardiac registration of after-potentials (author's transl)].
A 20-year-old woman, admitted to an intensive care unit in ventricular fibrillation, had marked QT prolongation in the ECG after successful defibrillation. Family study revealed ECG signs of QT prolongation, as seen in the Romano-Ward syndrome, in four generations. Syncopal attacks only occurred in the third generation, affecting four siblings. In the described patient the intraventricular ECG revealed additional depolarizations (after-potentials) during and following repolarization (ST-T) in the surface ECG. The patient died later as a result of irreversible hypoxic cerebral damage. Invasive ECG studies in the mother also revealed after-potentials. In the patient, morbid-anatomical examination revealed, in addition to fibrosis and lipomatosis around the sinus and AV nodes, focal round-cell ganglionitis in both sympathetic trunks, but predominantly on the right. These infiltrations have not previously been described in this disease and could be of significance in the pathogenesis of the prolonged QT syndrome.
D000328:Adult; D001145:Arrhythmias, Cardiac / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D001262:Atenolol / Q000627:therapeutic use; D001342:Autonomic Nervous System Diseases / Q000473:pathology*; D001344:Autopsy; D004562:Electrocardiography; D005260:Female; D005728:Ganglia, Sympathetic / Q000473:pathology*; D006801:Humans; D010375:Pedigree; D013577:Syndrome; D013997:Time Factors; D014693:Ventricular Fibrillation / Q000473:pathology
D001262:Atenolol
Grenzstrang-Ganglionitis bei erblichem Syndrom der QT-Verlängerung (Romano-Ward-Syndrom). Intrakardiale Aufzeichnung von "Nachpotentialen".