title: Quality of life in pediatric patients affected by electrophysiologic disease.
abstract: BACKGROUND
  Treatment of electrophysiologic (EP) disease in pediatric patients has improved; however, the effects on quality of life (QOL) are unknown.
  
  
  OBJECTIVE
  The purpose of this study was to compare QOL within EP disease groups and to other congenital heart diseases, to evaluate the effects of cardiac rhythm devices on QOL, and to identify drivers of QOL in EP disease.
  
  
  METHODS
  Cross-sectional study of patient/parent proxy-reported Pediatric Cardiac Quality of Life Inventory scores (Total, Disease Impact, Psychosocial Impact) in subjects aged 8 to 18 years from 11 centers with congenital complete heart block (CCHB), ventricular tachycardia (VT), supraventricular tachycardia (SVT), and long QT syndrome (LQTS). QOL was compared between EP disease groups and congenital heart disease groups [bicuspid aortic valve (BAV), tetralogy of Fallot (TOF), and Fontan]. General linear modeling was used to perform group comparisons and to identify predictors of QOL variation.
  
  
  RESULTS
  Among 288 patient-parent pairs, mean age was 12.8 ± 3.0 years. CCHB (μ = 83) showed higher patient Total QOL than other EP disease cohorts (P ≤ .02; LQTS μ = 73; SVT μ = 74). SVT (μ = 75) and LQTS (μ = 75) had lower patient Total scores than BAV (μ = 81; P ≤ .008). Patient/parent-proxy QOL scores for all EP disease groups were not different than TOF and higher than Fontan. The presence of a cardiac rhythm device was associated with lower QOL scores in LQTS (μ = 66 vs μ = 76; P < .01). Predictors of lower patient/parent-proxy QOL included EP disease type (P ≤ .03), increased medical care utilization (P ≤ .04), and no parental college degree (P ≤ .001).
  
  
  CONCLUSIONS
  Given the significant variation in QOL in EP disease type, stratification by EP disease type and increased medical care utilization may allow for targeted interventions to improve QOL.
authors.affiliation: The Heart Institute, Department of Pediatrics, Division of Pediatric Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address: Richard.czosek@cchmc.org.
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Czosek
authors.affiliation: Department of Biostatistics, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Amy E
authors.initials: AE
authors.lastname: Cassedy
authors.affiliation: Critical Care and Cardiorespiratory Division, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, United Kingdom.
authors.forename: Jo
authors.initials: J
authors.lastname: Wray
authors.affiliation: Miami Children's Hospital, Miami, Florida.
authors.forename: Gil
authors.initials: G
authors.lastname: Wernovsky
authors.affiliation: Department of Cardiology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts.
authors.forename: Jane W
authors.initials: JW
authors.lastname: Newburger
authors.affiliation: Herma Heart Center, Children's Hospital of Wisconsin, Milwaukee Wisconsin.
authors.forename: Kathleen A
authors.initials: KA
authors.lastname: Mussatto
authors.affiliation: Department of Pediatrics, University of Texas Southwestern Medical School, Dallas, Texas.
authors.forename: Lynn
authors.initials: L
authors.lastname: Mahony
authors.affiliation: Division of Pediatric Cardiology, UCSF Benioff Children's Hospital, and Department of Pediatrics, UCSF School of Medicine, San Francisco, California.
authors.forename: Ronn E
authors.initials: RE
authors.lastname: Tanel
authors.affiliation: Phoenix Children's Hospital and Arizona Pediatric Cardiology Consultants/Pediatrix, University of Arizona School of Medicine-Phoenix, Phoenix, Arizona.
authors.forename: Mitchell I
authors.initials: MI
authors.lastname: Cohen
authors.affiliation: Department of Paediatric Cardiology, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom.
authors.forename: Rodney C
authors.initials: RC
authors.lastname: Franklin
authors.affiliation: Critical Care and Cardiorespiratory Division, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, United Kingdom.
authors.forename: Kate L
authors.initials: KL
authors.lastname: Brown
authors.affiliation: Department of Pediatrics, Stanford University and Lucile Packard Children's Hospital, Palo Alto, California.
authors.forename: David
authors.initials: D
authors.lastname: Rosenthal
authors.affiliation: The Heart Institute, Department of Pediatrics, Division of Pediatric Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Dennis
authors.initials: D
authors.lastname: Drotar
authors.affiliation: The Heart Institute, Department of Pediatrics, Division of Pediatric Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Bradley S
authors.initials: BS
authors.lastname: Marino
mesh_terms: D000293:Adolescent; D001145:Arrhythmias, Cardiac / Q000151:congenital / Q000175:diagnosis / Q000523:psychology / Q000628:therapy; D002648:Child; D003430:Cross-Sectional Studies; D047548:Defibrillators / Q000523:psychology / Q000706:statistics & numerical data; D005260:Female; D006330:Heart Defects, Congenital / Q000523:psychology*; D006339:Heart Rate; D006801:Humans; D016014:Linear Models; D008297:Male; D010138:Pacemaker, Artificial / Q000523:psychology / Q000706:statistics & numerical data; D010372:Pediatrics / Q000379:methods; D011788:Quality of Life; D006113:United Kingdom; D014481:United States
keywords: Arrhythmia; Channelopathy; Electrophysiology; Pediatrics; Quality of life; cardiac rhythm devices