Familial atrial fibrillation is a genetically heterogeneous disorder

J Am Coll Cardiol. 2003 Jun 18;41(12):2185-92. doi: 10.1016/s0735-1097(03)00465-0.

Abstract

Objectives: The aims of this study were to identify and characterize familial cases of atrial fibrillation (AF) in our clinical practice and to determine whether AF is genetically heterogeneous.

Background: Atrial fibrillation is not generally regarded as a heritable disorder, yet a genetic locus for familial AF was previously mapped to chromosome 10.

Methods: Of 2,610 patients seen in our arrhythmia clinic during an 18-month study period, 914 (35%) were diagnosed with AF. Familial cases were identified by history and medical records review. Four multi-generation families with autosomal dominant AF (FAF 1 to 4) were tested for linkage to the chromosome 10 AF locus.

Results: Fifty probands (5% of all AF patients; 15% of lone AF patients) were identified with lone AF (age 41 +/- 9 years) and a positive family history (1 to 9 additional relatives affected). In FAF 1 to 3, AF was associated with rapid ventricular response. In contrast, AF in FAF-4 was associated with a slow ventricular response and, with progression of the disease, junctional rhythm and cardiomyopathy. Genotyping of FAF 1 to 4 with deoxyribonucleic acid markers spanning the chromosome 10q22-q24 region excluded linkage of AF to this locus. In FAF-4, linkage was also excluded to the chromosome 3p22-p25 and lamin A/C loci associated with familial AF, conduction system disease, and dilated cardiomyopathy.

Conclusions: Familial AF is more common than previously recognized, highlighting the importance of genetics in disease pathogenesis. In four families with AF, we have excluded linkage to chromosome 10q22-q24, establishing that at least two disease genes are responsible for this disorder.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Atrial Fibrillation / diagnostic imaging
  • Atrial Fibrillation / genetics*
  • Atrial Fibrillation / physiopathology
  • Chromosome Mapping
  • Chromosomes, Human, Pair 10 / genetics*
  • Echocardiography
  • Electrocardiography
  • Female
  • Genetic Heterogeneity*
  • Genetic Linkage / genetics
  • Genotype
  • Humans
  • Male
  • Middle Aged
  • Pedigree