Key result
Invasive electrophysiological study in two siblings with congenital long QT syndrome revealed intra-Hisian conduction delay, documenting an association with functional conduction system disease.
Case Report (n=2)
This report documents the association of functional conduction system disease with congenital QT prolongation through invasive electrophysiological findings.
No immediate practice change in congenital LQTS; leaves open conduction disease prevalence and causality.
This report describes the cardiac conduction abnormalities, detected by invasive electrophysiological study, in two identical siblings with symptomatic congenital long QT syndrome. Both patients had evidence of intra-Hisian conduction delay in response to programmed atrial stimulation and pacing induced infranodal block was seen in one of the two patients. The response of the observed conduction delay to autonomic interventions is described. The observed electrophysiologic abnormalities are consistent with previously reported pathological findings and document the association of functional conduction system disease with congenital QT prolongation.
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Greenspon et al. (1989) conducted a case report in Symptomatic congenital long QT syndrome (n=2). Invasive electrophysiological study was evaluated on Cardiac conduction abnormalities. Invasive electrophysiological study in two siblings with congenital long QT syndrome revealed intra-Hisian conduction delay, documenting an association with functional conduction system disease.
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