Circulation, Vol 57, 1221-1231, Copyright © 1978 by American Heart Association
TN James, P Froggatt, WJ Atkinson Jr, PR Lurie, DG McNamara, WW Miller, GT Schloss, JF Carroll and RL North
Eight patients (different families) with syncopal attacks and a long QT
interval in the ECG died suddenly. Five heard normally and three were born
deaf. At postmortem examination of all eight hearts the single consistent
abnormality was focal neuritis and neural degeneration within the sinus
node, A-V node, His bundle and ventricular myocardium. Although the
etiology of this intracardiac neural disease is uncertain, a chronic viral
infection or some noninfectious degenerative process are among the
plausible causes discussed. If intracardiac neuritis and neural
degeneration prove to be a prevalent finding among other victims dying from
the long QT syndromes, further consideration should be given to whether
there is any genuine hereditary component in the pathogenesis. Because of
the asymmetrical and focal distribution of the cardioneural lesions, the
response to present forms of medical or surgical treatment of the lung QT
syndromes may vary from benefit to harm. Until more is known of the true
etiology of the neural disease, treatment will probably remain empirical in
nature and should be conducted with cautious clinical observation.
ARTICLES
De subitaneis mortibus. XXX. Observations on the pathophysiology of the long QT syndromes with special reference to the neuropathology of the heart
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