Cardiac Resynchronization Therapy in Paediatric and Congenital Heart Disease: Differential Effects in Various Anatomic and Functional Substrates

Jan Janousek ^1*, Roman A Gebauer ², Hashim Abdul-Khaliq ³, Mark Turner ⁴, Laszlo Kornyei ⁵, Oswin Grollmuß ¹, Eric Rosenthal ⁶, Elisabeth Villain ⁷, Andreas Früh ⁸, Thomas Paul ⁹, Nico A Blom ¹⁰, Juha-Matti Happonen ¹¹, Urs Bauersfeld ¹², Joes R Jacobsen ¹³, Freek van den Heuvel ¹⁴, Tammo Delhaas ¹⁵, John Papagiannis ¹⁶ and Conceicao Trigo ¹⁷

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

^* To whom correspondence should be addressed. E-mail: jan.janousek{at}medizin.uni-leipzig.de.

Accepted 10 March 2009

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.