title: Incidence of cardiac arrhythmias in asymptomatic hereditary hemochromatosis subjects with C282Y homozygosity.
abstract: It is not well known whether systemic iron overload per se in hereditary hemochromatosis (HH) is associated with cardiac arrhythmias before other signs and symptoms of cardiovascular disease occur. In the present study, we examined the incidence of cardiac arrhythmia in cardiac asymptomatic subjects with HH (New York Heart Association functional class I) and compared it to that in age- and gender-matched normal volunteers. The 42 subjects with HH and the 19 normal control subjects were recruited through the National Heart, Lung, and Blood Institute-sponsored "Heart Study of Hemochromatosis." They completed 48-hour Holter electrocardiography ambulatory monitoring at the baseline evaluation. The subjects with HH were classified as newly diagnosed (group A) and chronically treated (group B) subjects. All subjects with HH had C282Y homozygosity, and the normal volunteers lacked any HFE gene mutations known to cause HH. Although statistically insignificant, the incidence of ventricular and supraventricular ectopy tended to be greater in the combined HH groups than in the controls. Supraventricular ectopy was more frequently noted in group B compared to in the controls (ectopy rate per hour 11.1 ± 29.9 vs 1.5 ± 3.5, p < 0.05, using the Kruskal-Wallis test). No examples of heart block, other than first-degree atrioventricular node block, were seen in any of the subjects. The incidence of cardiac arrhythmias was not significantly reduced after 6 months of intensive iron removal therapy in the group A subjects. No life-threatening arrhythmias were observed in our subjects with HH. In conclusion, our data suggest that the incidence of cardiac arrhythmias is, at most, marginally increased in asymptomatic subjects with HH. A larger clinical study is warranted to further clarify our observation.
authors.affiliation: Cardiopulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland, USA.
authors.forename: Yukitaka
authors.initials: Y
authors.lastname: Shizukuda
authors.forename: Dorothy J
authors.initials: DJ
authors.lastname: Tripodi
authors.forename: Gloria
authors.initials: G
authors.lastname: Zalos
authors.forename: Charles D
authors.initials: CD
authors.lastname: Bolan
authors.forename: Yu-Ying
authors.initials: YY
authors.lastname: Yau
authors.forename: Susan F
authors.initials: SF
authors.lastname: Leitman
authors.forename: Myron A
authors.initials: MA
authors.lastname: Waclawiw
authors.forename: Douglas R
authors.initials: DR
authors.lastname: Rosing
mesh_terms: D001145:Arrhythmias, Cardiac / Q000453:epidemiology* / Q000209:etiology / Q000503:physiopathology; D004247:DNA / Q000235:genetics*; D004252:DNA Mutational Analysis; D015716:Electrocardiography, Ambulatory; D005260:Female; D005293:Ferritins / Q000097:blood; D005500:Follow-Up Studies; D006432:Hemochromatosis / Q000097:blood / Q000150:complications* / Q000235:genetics; D000071020:Hemochromatosis Protein; D015395:Histocompatibility Antigens Class I / Q000097:blood / Q000235:genetics*; D006720:Homozygote; D006801:Humans; D015994:Incidence; D007501:Iron / Q000097:blood; D008297:Male; D008565:Membrane Proteins / Q000097:blood / Q000235:genetics*; D008875:Middle Aged; D009154:Mutation; D011379:Prognosis; D012189:Retrospective Studies; D014168:Transferrin / Q000378:metabolism; D014481:United States / Q000453:epidemiology
chemical_list: C103673:HFE protein, human; D000071020:Hemochromatosis Protein; D015395:Histocompatibility Antigens Class I; D008565:Membrane Proteins; D014168:Transferrin; D004247:DNA; D005293:Ferritins; D007501:Iron