title: Pulmonary valve replacement in adult patients with a severely dilated right ventricle and refractory arrhythmias after repair of tetralogy of fallot.
abstract: BACKGROUND
  Refractory arrhythmias caused by right ventricular (RV) volume overload resulting from pulmonary regurgitation are the main concern in adult patients after repair of tetralogy of Fallot (TOF). Early pulmonary valve replacement (PVR) may prevent irreversible RV dysfunction and refractory arrhythmias, so the present study evaluated the PVR outcomes in adult patients with a severely dilated RV (d-RV) and refractory arrhythmias after TOF repair.
  
  
  RESULTS
  Three TOF patients with a d-RV and tachyarrhythmias underwent PVR between the ages of 28 and 38 years. All had a d-RV (RV end-diastolic volume index (RVEDVI) >200 ml/m(2)) with a polymorphic nonsustained ventricular tachycardia (NSVT). Atrial tachycardia (AT) was identified in 2 patients and they underwent radiofrequency catheter ablation. The arrhythmias in all 3 were refractory to antiarrhythmic drugs. One year after PVR, the RVEDVI, left ventricular ejection fraction, right atrial pressure, cardiac index, cardiothoracic ratio, brain natriuretic peptide levels, and peak VO(2) improved in all, but without normalization. During the 2.6-3.8 year follow-up, all experienced tachyarrhythmias (NSVT or AT), which were controlled with medication.
  
  
  CONCLUSIONS
  PVR may be beneficial for refractory arrhythmias, even in TOF patients with a d-RV, but it is difficult to completely normalize the hemodynamics and resolve the arrhythmogenicity.
authors.affiliation: Department of Pediatric Cardiology, National Cardiovascular Center, Suita, Japan. ayamiya@hsp.ncvc.go.jp
authors.forename: Aya
authors.initials: A
authors.lastname: Miyazaki
authors.forename: Masaki
authors.initials: M
authors.lastname: Yamamoto
authors.forename: Heima
authors.initials: H
authors.lastname: Sakaguchi
authors.forename: Shinya
authors.initials: S
authors.lastname: Tsukano
authors.forename: Koji
authors.initials: K
authors.lastname: Kagisaki
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Suyama
authors.forename: Hideo
authors.initials: H
authors.lastname: Ohuchi
authors.forename: Ken-ichi
authors.initials: K
authors.lastname: Kurosaki
authors.forename: Toshikatsu
authors.initials: T
authors.lastname: Yagihara
authors.forename: Osamu
authors.initials: O
authors.lastname: Yamada
mesh_terms: D000328:Adult; D001145:Arrhythmias, Cardiac / Q000209:etiology* / Q000503:physiopathology / Q000601:surgery; D001705:Bioprosthesis; D017115:Catheter Ablation; D004562:Electrocardiography; D005260:Female; D006350:Heart Valve Prosthesis; D006801:Humans; D017380:Hypertrophy, Right Ventricular / Q000209:etiology* / Q000503:physiopathology / Q000601:surgery*; D007564:Japan; D011183:Postoperative Complications / Q000209:etiology / Q000601:surgery; D011379:Prognosis; D011664:Pulmonary Valve / Q000601:surgery*; D011665:Pulmonary Valve Insufficiency / Q000209:etiology / Q000601:surgery; D013771:Tetralogy of Fallot / Q000601:surgery*; D013997:Time Factors