title: Cardiac resynchronisation therapy in paediatric and congenital heart disease: differential effects in various anatomical and functional substrates.
abstract: BACKGROUND
  Cardiac resynchronisation therapy (CRT) is increasingly used in children in a variety of anatomical and pathophysiological conditions, but published data are scarce.
  
  
  OBJECTIVE
  To record current practice and results of CRT in paediatric and congenital heart disease.
  
  
  METHODS
  Retrospective multicentre European survey.
  
  
  METHODS
  Paediatric cardiology and cardiac surgery centres.
  
  
  METHODS
  One hundred and nine patients aged 0.24-73.8 (median 16.9) years with structural congenital heart disease (n = 87), congenital atrioventricular block (n = 12) and dilated cardiomyopathy (n = 10) with systemic left (n = 69), right (n = 36) or single (n = 4) ventricular dysfunction and ventricular dyssynchrony during sinus rhythm (n = 25) or associated with pacing (n = 84).
  
  
  METHODS
  CRT for a median period of 7.5 months (concurrent cardiac surgery in 16/109).
  
  
  METHODS
  Functional improvement and echocardiographic change in systemic ventricular function.
  
  
  RESULTS
  The z score of the systemic ventricular end-diastolic dimension decreased by median 1.1 (p<0.001). Ejection fraction (EF) or fractional area of change increased by a mean (SD) of 11.5 (14.3)% (p<0.001) and New York Heart Association (NYHA) class improved by median 1.0 grade (p<0.001). Non-response to CRT (18.5%) was multivariably predicted by the presence of primary dilated cardiomyopathy (p = 0.002) and poor NYHA class (p = 0.003). Presence of a systemic left ventricle was the strongest multivariable predictor of improvement in EF/fractional area of change (p<0.001). Results were independent of the number of patients treated in each contributing centre.
  
  
  CONCLUSIONS
  Heart failure associated with ventricular pacing is the largest indication for CRT in paediatric and congenital heart disease. CRT efficacy varies widely with the underlying anatomical and pathophysiological substrate.
authors.affiliation: Department of Paediatric Cardiology, University of Leipzig, Heart Centre, Strümpellstrasse 39, 04289 Leipzig, Germany. jan.janousek@medizin.uni-leipzig.de
authors.forename: J
authors.initials: J
authors.lastname: Janousek
authors.forename: R A
authors.initials: RA
authors.lastname: Gebauer
authors.forename: H
authors.initials: H
authors.lastname: Abdul-Khaliq
authors.forename: M
authors.initials: M
authors.lastname: Turner
authors.forename: L
authors.initials: L
authors.lastname: Kornyei
authors.forename: O
authors.initials: O
authors.lastname: Grollmuss
authors.forename: E
authors.initials: E
authors.lastname: Rosenthal
authors.forename: E
authors.initials: E
authors.lastname: Villain
authors.forename: A
authors.initials: A
authors.lastname: Früh
authors.forename: T
authors.initials: T
authors.lastname: Paul
authors.forename: N A
authors.initials: NA
authors.lastname: Blom
authors.forename: J-M
authors.initials: JM
authors.lastname: Happonen
authors.forename: U
authors.initials: U
authors.lastname: Bauersfeld
authors.forename: J R
authors.initials: JR
authors.lastname: Jacobsen
authors.forename: F
authors.initials: F
authors.lastname: van den Heuvel
authors.forename: T
authors.initials: T
authors.lastname: Delhaas
authors.forename: J
authors.initials: J
authors.lastname: Papagiannis
authors.forename: C
authors.initials: C
authors.lastname: Trigo
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001145:Arrhythmias, Cardiac / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D002304:Cardiac Pacing, Artificial; D002311:Cardiomyopathy, Dilated / Q000150:complications* / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D006330:Heart Defects, Congenital / Q000150:complications* / Q000503:physiopathology; D016027:Heart Transplantation / Q000706:statistics & numerical data; D006801:Humans; D007223:Infant; D008875:Middle Aged; D008944:Mitral Valve Insufficiency / Q000209:etiology / Q000628:therapy; D010138:Pacemaker, Artificial; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome; D020257:Ventricular Remodeling; D055815:Young Adult