Familial atrial dysrhythmia with A V block. Intracellular microelectrode, clinical electrophysiologic, and morphologic observations

Y. F. Amat Leon, A. J. Racki, P. Denes

Research output: Contribution to journalArticlepeer-review

15 Scopus citations

Abstract

This study is of a family with a syndrome of atrial fibrillation or flutter with advanced or complete atrioventricular (A V) block, involving four members in two generations. Less serious arrhythmias were documented in another 15 members (three generations). Inheritance was by autosomal dominance with varying degrees of expression. An atrial biopsy was obtained in one of the members, a 40 yr old female who had atrial flutter with advanced A V block proximal to the His bundle (H V interval of 39 msec). Intracellular action potential (IAP) recordings from this tissue revealed partially depolarized cells with depressed IAP amplitude, phase IV diastolic depolarization with spontaneous firing, diminished excitability and responsiveness, and decremental conduction with local block and reentry. Histological findings revealed vacuolar degeneration, hypertrophy, and early necrosis of atrial cells. In conclusion, the multiple IAP and pathologic abnormalities provide a basis for atrial dysrhythmia in this family. The etiology of the disease is unknown.

Original languageEnglish (US)
Pages (from-to)1097-1104
Number of pages8
JournalCirculation
Volume50
Issue number6
DOIs
StatePublished - 1974

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Fingerprint Dive into the research topics of 'Familial atrial dysrhythmia with A V block. Intracellular microelectrode, clinical electrophysiologic, and morphologic observations'. Together they form a unique fingerprint.

Cite this