title: Abnormal action potential duration restitution property in the right ventricular outflow tract in Brugada syndrome.
abstract: BACKGROUND
  Although patients with Brugada syndrome (BS) are at risk of ventricular fibrillation (VF) and ensuing death, the action potential duration (APD) restitution properties of the right ventricular outflow tract (RVOT) in patients with BS remain undetermined.
  
  
  RESULTS
  Endocardial monophasic action potentials (MAPs) were obtained from 16 patients with BS and 17 control patients. MAPs were recorded from the RVOT in all patients. The MAP duration at 90% repolarization (MAPD(90)), effective refractory period (ERP), and maximum slope of the APD restitution curve were obtained. VF was induced with up to 3 extrastimuli from the RV apex or RVOT. There was no difference in MAPD(90) between the 2 groups, but the ERP was significantly shorter in patients with BS than in control patients (210.7+/-10.5 vs 223.8+/-13.4 ms, P=0.008). MAPD at the shortest diastolic interval was significantly shorter in patients with BS than in control patients (149.9+/-19.9 vs 179.8+/-13.7 ms, P<0.001). The maximum slope of the APD restitution curve was steeper in patients with BS than in control patients (2.90+/-1.29 vs 1.38+/-0.41, P<0.001).
  
  
  CONCLUSIONS
  The shorter ERP, shorter MAPD at the shortest diastolic interval and steeply sloped APD restitution curve in the RVOT appear to be related to the inducibility of VF in patients with BS.
authors.affiliation: Division of Cardiology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan.
authors.forename: Sonoko
authors.initials: S
authors.lastname: Ashino
authors.forename: Ichiro
authors.initials: I
authors.lastname: Watanabe
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Kofune
authors.forename: Koichi
authors.initials: K
authors.lastname: Nagashima
authors.forename: Kimie
authors.initials: K
authors.lastname: Ohkubo
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Okumura
authors.forename: Toshiko
authors.initials: T
authors.lastname: Nakai
authors.forename: Yuji
authors.initials: Y
authors.lastname: Kasamaki
authors.forename: Atsushi
authors.initials: A
authors.lastname: Hirayama
mesh_terms: D000200:Action Potentials / Q000502:physiology*; D000368:Aged; D053840:Brugada Syndrome / Q000150:complications / Q000503:physiopathology*; D016022:Case-Control Studies; D004562:Electrocardiography; D005260:Female; D006329:Heart Conduction System / Q000502:physiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D012032:Refractory Period, Electrophysiological / Q000502:physiology; D012307:Risk Factors; D013997:Time Factors; D018497:Ventricular Dysfunction, Right / Q000503:physiopathology*; D014693:Ventricular Fibrillation / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology