Two:One atrioventricular block in infants with congenital long QT syndrome

William A. Scott, Macdonald Dick

Research output: Contribution to journalArticlepeer-review

51 Scopus citations


Congenital prolongation of the QT interval is a rare but life-threatening disorder of the cardiac conduction system.1-5 The disorder usually presents as syncope or sudden death due to complex polymorphic ventricular tachycardia (torsade de pointes) or ventricular fibrillation.6 The major hypothesis explaining the disorder is an imbalance of sympathetic discharge between the right and left stellate ganglia resulting in inhomogeneity of ventricular refractoriness and a substrate for reentrant ventricular arrhythmias.4-6 Although sinus bradycardia may be associated with the syndrome, abnormalities in atrioventricular (AV) conduction are rare.4 In this report we present 3 infants with the congenital long QT syndrome and 2:1 AV block and provide support for the role of the long effective refractory period in producing the block.

Original languageEnglish (US)
Pages (from-to)1409-1410
Number of pages2
JournalThe American journal of cardiology
Issue number16
StatePublished - Dec 1 1987

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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