Eur J Pediatr (2009) 168:1081–1090 DOI 10.1007/s00431-008-0891-y ORIGINAL PAPER Prevalence of congenital heart defects in neuroblastoma patients: a cohort study and systematic review of literature Klaartje van Engelen & Johannes H. M. Merks & Jan Lam & Leontien C. M. Kremer & Manouk Backes & Marieke J. H. Baars & Heleen J. H. van der Pal & Alex V. Postma & Rogier Versteeg & Huib N. Caron & Barbara J. M. Mulder Received: 8 September 2008 / Accepted: 13 November 2008 / Published online: 17 December 2008 # The Author(s) 2008. This article is published with open access at Springerlink.com Abstract Data on the prevalence of congenital heart defects (CHD) in neuroblastoma patients are inconsistent. If CHD are more common in neuroblastoma patients than in the general population, cardiac screening might be warranted. In this study we used echocardiography to determine the prevalence of CHD in a single centre cohort of surviving neuroblastoma patients. In addition, we performed a systematic review of the literature. Echocardi- ography was performed in 119 of 133 patients (89.5%). Only two patients (1.7%) had CHD. The prevalence of CHD was not significantly different from a previously published control group of 192 leukaemia patients exam- ined by echocardiography (P= 0.49). Literature search revealed 17 studies, showing prevalence rates of CHD in neuroblastoma patients ranging from 0 to 20%. Prevalence was less than 3.6% in the majority of studies. Most studies K. van Engelen : M. Backes : B. J. M. Mulder Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands K. van Engelen (*) : M. J. H. Baars Department of Clinical Genetics, Academic Medical Centre, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands e-mail: k.vanengelen@amc.uva.nl J. H. M. Merks : H. N. Caron Department of Paediatric Oncology, Academic Medical Centre, Amsterdam, The Netherlands J. Lam Department of Paediatric Cardiology, Academic Medical Centre, Amsterdam, The Netherlands lacked information on validity. We conclude that current evidence does not support standard cardiac screening in all patients with neuroblastoma. Keywords Neuroblastoma . Congenital heart defects . Echocardiography . Association . Screening . Neural crest Introduction Neuroblastoma is an embryonal cancer of the postgangli- onic sympathetic nervous system, which mostly arises in the adrenal gland. It is the most common extracranial solid tumour in children, comprising 8% to 10% of all childhood cancers. The incidence is nearly one per 10,000 children under the age of 15 years [3]. L. C. M. Kremer : H. J. H. van der Pal Outpatient Clinic for the Late Effects of Treatment for Childhood Cancer, Academic Medical Centre, Amsterdam, The Netherlands L. C. M. Kremer Cochrane Childhood Cancer Group, Academic Medical Centre, Amsterdam, The Netherlands A. V. Postma Heart Failure Research Centre, Department of Anatomy and Embryology, Academic Medical Centre, Amsterdam, The Netherlands R. Versteeg Department of Human Genetics, Academic Medical Centre, Amsterdam, The Netherlands B. J. M. Mulder Department of Cardiology, University Medical Centre, Utrecht, The Netherlands DO00891; No of Pages