Cardiac resynchronisation therapy in paediatric and congenital heart disease: differential effects in various anatomical and functional substrates
OPEN ACCESS- J Janoušek1,
- R A Gebauer2,
- H Abdul-Khaliq3,
- M Turner4,
- L Kornyei5,
- O Grollmuß1,
- E Rosenthal6,
- E Villain7,
- A Früh8,
- T Paul9,
- N A Blom10,
- J-M Happonen11,
- U Bauersfeld12,
- J R Jacobsen13,
- F van den Heuvel14,
- T Delhaas15,
- J Papagiannis16,
- C Trigo17,
- for the Working Group for Cardiac Dysrhythmias and Electrophysiology of the Association for European Paediatric Cardiology
- 1Department of Paediatric Cardiology, University of Leipzig, Heart Centre, Leipzig, Germany
- 2Kardiocentrum and Cardiovascular Research Centre, University Hospital Motol, Prague, Czech Republic
- 3German Heart Centre Berlin, Berlin, Germany
- 4Bristol Royal Infirmary and University of Bristol, Bristol, UK
- 5Hungarian Paediatric Heart Centre, Budapest, Hungary
- 6Evelina Children’s Hospital, Guy’s and St Thomas’ Trust, London, UK
- 7Département de Cardiologie Pédiatrique, Hôpital Necker, Paris, France
- 8Paediatric Cardiology Unit, Rikshospitalet University Hospital, Oslo, Norway
- 9Department of Paediatric Cardiology and Intensive Care, University Hospital, Göttingen, Germany
- 10Department of Paediatric Cardiology, Leiden University Medical Centre, Leiden, The Netherlands
- 11Division of Paediatric Cardiology, Department of Paediatrics, Helsinki University Central Hospital, Helsinki, Finland
- 12Division of Paediatric Cardiology, University Children’s Hospital of Zurich, Zurich, Switzerland
- 13Department of Paediatrics, Rigshospitalet, Copenhagen, Denmark
- 14Beatrix Children’s Hospital, Division of Paediatric Cardiology, University Medical Centre Groningen, The Netherlands
- 15Division of Paediatric Cardiology, AZ Maastricht, Maastricht, The Netherlands
- 16Department of Paediatric Cardiology, Onassis Cardiac Surgery Centre, Athens, Greece
- 17Servico de Cardiologia Pediatrica, Hospital de Santa Marta, Lisboa, Portugal
- Professor J Janoušek, Department of Paediatric Cardiology, University of Leipzig, Heart Centre, Strümpellstrasse 39, 04289 Leipzig, Germany; jan.janousek{at}medizin.uni-leipzig.de
- Accepted 10 March 2009
- Published Online First 22 March 2009
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.
Design: Retrospective multicentre European survey.
Setting: Paediatric cardiology and cardiac surgery centres.
Patients: 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).
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 (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.
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 anatomical and pathophysiological substrate.
Footnotes
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Funding: Ministry of Health, Czech Republic (grant NR/9472-3 to RAG) and University Hospital Motol, Prague, Czech Republic (research project No 64203 to RAG).
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Competing interests: None.
