Lev's Syndrome: A rare case of progressive cardiac conduction disorder presenting to the emergency department

Am J Emerg Med. 2019 May;37(5):1006.e1-1006.e4. doi: 10.1016/j.ajem.2019.01.054. Epub 2019 Jan 31.

Abstract

Lev's Syndrome is a rare, progressive cardiac conduction defect (PCCD) due to myocardial fibrosis first described by Maurice Lev in 1964. This condition, proposed to start in the fourth decade of life, involves a sclerotic fibro-fatty degeneration of the Bundle of His and Purkinje fibers, which Lev proposed caused increasing AV delay with age. With the prevalence of electrocardiogram (ECG) use in the emergency department (ED) for cardiac- and non-cardiac complaints, dysrhythmias can be incidentally found and confuse diagnosis and disposition. We highlight the case of an 84-year-old male who presented to the ED for acute onset of diffuse facial paresthesias with elevated blood pressure at home and was found to be significantly bradycardic on initial evaluation. On serial ECGs, the conduction rhythm changed from an initial new first-degree atrioventricular (AV) block with left bundle branch block (LBBB), to a later first-degree AV block without LBBB. Cardiology was consulted. Serial ECGs demonstrated an evolving conduction block arrhythmia consistent with Lev's Syndrome. Here we describe a case of symptomatic bradycardia found to be consistent with Lev's Syndrome.

Keywords: Cardiac dysfunction; Dysrhythmia; Lev's Syndrome.

Publication types

  • Case Reports

MeSH terms

  • Aged, 80 and over
  • Bradycardia / diagnosis*
  • Bradycardia / etiology
  • Bradycardia / therapy
  • Cardiac Conduction System Disease / complications
  • Cardiac Conduction System Disease / diagnosis*
  • Cardiac Conduction System Disease / therapy
  • Cardiac Pacing, Artificial
  • Disease Progression
  • Electrocardiography
  • Emergency Service, Hospital
  • Fibrosis
  • Heart Block / complications
  • Heart Block / diagnosis*
  • Heart Block / therapy
  • Humans
  • Male
  • Myocardium / pathology*
  • Severity of Illness Index

Supplementary concepts

  • Hereditary bundle branch system defect