title: Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) Associated With Ryanodine Receptor (RyR2) Gene Mutations　- Long-Term Prognosis After Initiation of Medical Treatment.
abstract: BACKGROUND
  The long-term prognosis of cardiac ryanodine receptor (RyR2) positive catecholaminergic polymorphic ventricular tachycardia (CPVT) patients after initiation of medical therapy has not been well investigated. This study aimed to assess the recurrence of fatal cardiac event after initiation of medical therapy inRyR2-positive CPVT patients.
  
  
  RESULTS
  Thirty-fourRyR2-positive CPVT patients with a history of cardiac events were enrolled. All patients had medical treatment initiated after the first symptom or diagnosis. Exercise stress tests (ESTs) were performed to evaluate the efficacy of the medical therapy. Even after the initiation of medical therapy, high-risk ventricular arrhythmias (VAs), including premature ventricular contraction couplets, bigeminy, and ventricular tachycardia, were still induced in the majority of patients (80.6%). During 7.4 years of follow-up after the diagnosis, 7 of the 34 (20.6%) patients developed fatal cardiac events. Among those 7 patients, 6 (85.7%) were not compliant with either exercise restriction or medication therapy at the time of the events.
  
  
  CONCLUSIONS
  Even after initiation of medical treatment, high-risk VAs were induced during EST in mostRyR2-positive CPVT patients. Most fatal recurrent cardiac events occurred in patients who were noncompliant with exercise restriction and/or medical therapy. Medical management including strict exercise restriction should be emphasized to prevent recurrent cardiac event in mostRyR2-positive CPVT patients. (Circ J 2016; 80: 1907-1915).
authors.affiliation: Division of Arrhythmia and Electrophysiology, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center.
authors.forename: Hiro
authors.initials: H
authors.lastname: Kawata
authors.forename: Seiko
authors.initials: S
authors.lastname: Ohno
authors.forename: Takeshi
authors.initials: T
authors.lastname: Aiba
authors.forename: Heima
authors.initials: H
authors.lastname: Sakaguchi
authors.forename: Aya
authors.initials: A
authors.lastname: Miyazaki
authors.forename: Naokata
authors.initials: N
authors.lastname: Sumitomo
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Kamakura
authors.forename: Ikutaro
authors.initials: I
authors.lastname: Nakajima
authors.forename: Yuko Y
authors.initials: YY
authors.lastname: Inoue
authors.forename: Koji
authors.initials: K
authors.lastname: Miyamoto
authors.forename: Hideo
authors.initials: H
authors.lastname: Okamura
authors.forename: Takashi
authors.initials: T
authors.lastname: Noda
authors.forename: Kengo
authors.initials: K
authors.lastname: Kusano
authors.forename: Shiro
authors.initials: S
authors.lastname: Kamakura
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Miyamoto
authors.forename: Isao
authors.initials: I
authors.lastname: Shiraishi
authors.forename: Minoru
authors.initials: M
authors.lastname: Horie
authors.forename: Wataru
authors.initials: W
authors.lastname: Shimizu
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009154:Mutation; D019837:Ryanodine Receptor Calcium Release Channel / Q000235:genetics*; D015996:Survival Rate; D017180:Tachycardia, Ventricular / Q000235:genetics* / Q000401:mortality* / Q000628:therapy*; D000098850:Polymorphic Catecholaminergic Ventricular Tachycardia
chemical_list: D019837:Ryanodine Receptor Calcium Release Channel