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Cardiac Resynchronization Therapy in Paediatric and Congenital Heart Disease: Differential Effects in Various Anatomic and Functional Substrates
  1. Jan Janousek (jan.janousek{at}medizin.uni-leipzig.de)
  1. Department of Paediatric Cardiology, Heart Centre, University of Leipzig, Germany
    1. Roman A Gebauer
    1. Kardiocentrum and Cardiovascular Research Center, University Hospital Motol, Prague, Czech Republic
      1. Hashim Abdul-Khaliq
      1. German Heart Center Berlin, Berlin, Germany
        1. Mark Turner
        1. Bristol Royal Infirmary and University of Bristol, Bristol, United Kingdom
          1. Laszlo Kornyei
          1. Hungarian Pediatric Heart Center, Budapest, Hungary
            1. Oswin Grollmuß
            1. Department of Paediatric Cardiology, Heart Centre, University of Leipzig, Germany
              1. Eric Rosenthal
              1. Evelina Children's Hospital, Guy's & St Thomas' Trust, London, United Kingdom
                1. Elisabeth Villain
                1. Département de Cardiologie Pédiatrique, Hôpital Necker, Paris, France
                  1. Andreas Früh
                  1. Pediatric Cardiology Unit, Rikshospitalet University Hospital, Oslo, Norway
                    1. Thomas Paul
                    1. Department of Pediatric Cardiology and Intensive Care, University Hospital, Göttingen, Germany
                      1. Nico A Blom
                      1. Department of Pediatric Cardiology, Leiden University Medical Center, Leiden, Netherlands
                        1. Juha-Matti Happonen
                        1. Division of Pediatric Cardiology, Department of Pediatrics, Helsinki University Central Hospital, He, Finland
                          1. Urs Bauersfeld
                          1. Division of Pediatric Cardiology, University Children's Hospital of Zurich, Zurich, Switzerland
                            1. Joes R Jacobsen
                            1. Department of Pediatrics, Rigshospitalet, Copenhagen, Denmark
                              1. Freek van den Heuvel
                              1. Beatrix Children's Hospital, Division of Pediatric Cardiology, University Medical Center Groningen, Netherlands
                                1. Tammo Delhaas
                                1. Division of Pediatric Cardiology, AZ Maastricht, Maastricht, Belgium
                                  1. John Papagiannis
                                  1. Department of Pediatric Cardiology, Onassis Cardiac Surgery Center, Athens, Greece
                                    1. Conceicao Trigo
                                    1. Servico de Cardiologia Pediatrica, Hospital de Santa Marta, Lisboa, Portugal

                                      Abstract

                                      Objective: Cardiac resynchronization therapy (CRT) is increasingly used in children in a variety of anatomic and pathophysiologic conditions, but published data are scarce. This study records current practice and results of CRT in paediatric and congenital heart disease.

                                      Design: Retrospective multi-centre European survey.

                                      Setting: Paediatric cardiology and cardiac surgery centres.

                                      Patients: One hundred nine patients aged 0.24 – 73.8 (median 16.9) years with structural congenital heart disease (N=87), congenital AV 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).

                                      Interventions: CRT for a median period of 7.5 months (concurrent cardiac surgery in 16/109).

                                      Main outcome measures: 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 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 (LV) was the strongest multivariable predictor of improvement in EF/fractional area of change (P<0.001). Results were independent of number of patients treated in each contributing centre.

                                      Conclusion: 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 anatomic and pathophysiologic substrate.

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