title: Familial occurrence of sinus bradycardia, short PR interval, intraventricular conduction  defects, recurrent supraventricular tachycardia, and cardiomegaly.
abstract: Four members of a family presenting with sinus bradycardia, a short P-R interval, intraventricular conduction defects, recurrent supraventricular tachycardia (SVT), syncope, and cardiomegaly had His bundle studies and were found to have markedly shortened A-H intervals (30 to 55 msec.) with normal H-V times (35 to 50 msec.). Right atrial pacing at rates as high as 170 to 215 per minute failed to increase the A-H or H-V intervals significantly. The data are compatible with the presence of an A-V nodal bypass tract (James bundle) or even complete absence of an A-V node. Ventricular pacing and spontaneous ventricular premature beats resulted in a short ventriculoatrial conduction time (110 msec.) suggesting that if A-V nodal bypass tracts exist, they are utilized in an antegrade and retrograde fashion. None of the features of WPW syndrome was present. The mechanism of syncope in the mother and daughter was intermittent third-degree heart block. Both went on to develop permanent complete heart block despite electrophysiologic studies demonstrating 1:1 A-V conduction at extremely rapid atrial pacing rates and both required implantation of permanent pacemakers. The mechanism of syncope in the two brothers was possibly marked sinus bradycardia, but transient complete heart block has not been ruled out. Permanent pacemaker therapy was recommended for both. The nature of the cardiomegaly, which was mild in three patients, is not known. Although not well documented, several maternal relatives have had enlarged hearts, SVT, complete heart block, and syncope.
mesh_terms: D000293:Adolescent; D000328:Adult; D001146:Arrhythmia, Sinus / Q000235:genetics / Q000628:therapy; D001919:Bradycardia / Q000235:genetics*; D002036:Bundle of His / Q000503:physiopathology; D002037:Bundle-Branch Block / Q000235:genetics; D006332:Cardiomegaly / Q000235:genetics*; D002648:Child; D002675:Child, Preschool; D004562:Electrocardiography; D005260:Female; D006327:Heart Block / Q000235:genetics* / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D010138:Pacemaker, Artificial; D013575:Syncope / Q000235:genetics; D013610:Tachycardia / Q000235:genetics*