Original articles

Heart failure and cardiomyopathy

Cardiac resynchronisation therapy in paediatric and congenital heart disease: differential effects in various anatomical and functional substrates

J Janouek¹, 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

¹ Department of Paediatric Cardiology, University of Leipzig, Heart Centre, Leipzig, Germany
² Kardiocentrum and Cardiovascular Research Centre, University Hospital Motol, Prague, Czech Republic
³ German Heart Centre Berlin, Berlin, Germany
⁴ Bristol Royal Infirmary and University of Bristol, Bristol, UK
⁵ Hungarian Paediatric Heart Centre, Budapest, Hungary
⁶ Evelina Children’s Hospital, Guy’s and St Thomas’ Trust, London, UK
⁷ Département de Cardiologie Pédiatrique, Hôpital Necker, Paris, France
⁸ Paediatric Cardiology Unit, Rikshospitalet University Hospital, Oslo, Norway
⁹ Department of Paediatric Cardiology and Intensive Care, University Hospital, Göttingen, Germany
¹⁰ Department of Paediatric Cardiology, Leiden University Medical Centre, Leiden, The Netherlands
¹¹ Division of Paediatric Cardiology, Department of Paediatrics, Helsinki University Central Hospital, Helsinki, Finland
¹² Division of Paediatric Cardiology, University Children’s Hospital of Zurich, Zurich, Switzerland
¹³ Department of Paediatrics, Rigshospitalet, Copenhagen, Denmark
¹⁴ Beatrix Children’s Hospital, Division of Paediatric Cardiology, University Medical Centre Groningen, The Netherlands
¹⁵ Division of Paediatric Cardiology, AZ Maastricht, Maastricht, The Netherlands
¹⁶ Department of Paediatric Cardiology, Onassis Cardiac Surgery Centre, Athens, Greece
¹⁷ Servico de Cardiologia Pediatrica, Hospital de Santa Marta, Lisboa, Portugal

Professor J Janouek, Department of Paediatric Cardiology, University of Leipzig, Heart Centre, Strümpellstrasse 39, 04289 Leipzig, Germany; jan.janousek{at}medizin.uni-leipzig.de

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.

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?