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Hereditary Long QT Syndrome Associated with Cardiac Conduction System Disease
Author(s) -
GREENSPON ARNOLD J.,
KIDWELL GREGORY A.,
BARRASSE LINDA D.,
HESSEN SCOTT E.,
GIUDICI MICHAEL
Publication year - 1989
Publication title -
pacing and clinical electrophysiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.686
H-Index - 101
eISSN - 1540-8159
pISSN - 0147-8389
DOI - 10.1111/j.1540-8159.1989.tb02685.x
Subject(s) - medicine , long qt syndrome , conduction abnormalities , short qt syndrome , cardiology , electrical conduction system of the heart , disease , electrocardiography , qt interval
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.