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Europace 2000 2(3):224-227; doi:10.1053/eupc.2000.0101
© 2000 by European Society of Cardiology
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CASE REPORT

Sudden death in a child with an unusual accessory connection

D. J. Heaven1, J. A. Till2 and S. Y. Ho2

1Department of Cardiology, Royal Brompton & Harefield NHS Trust and National Heart and Lung Institute, Imperial College School of Medicine London, U.K.; 2Department Paediatric Cardiology, Royal Brompton & Harefield NHS Trust and National Heart and Lung Institute, Imperial College School of Medicine London, U.K.

An 8-year-old boy with Wolff-Parkinson-White syndrome died suddenly whilst exercising. He had been well with no immediate preceding symptoms of an arrhythmia, and was receiving no drugs. As an infant he had suffered recurrent episodes of atrioventricular reciprocating tachycardia (AVRT) and had documented pre-excited atrial fibrillation. At electrophysiological (EP) study, the refractory periods of his two accessory pathways were less than 220 ms. Drugs were discontinued at 4 years of age when he remained asymptomatic. Autopsy confirmed a very unusual accessory pathway. It was a muscular connection between the base of the right atrial appendage and the right ventricle, distant from the annulus of the tricuspid valve.

Key Words: Wolff-Parkinson-White syndrome, sudden death, childhood, accessory connection

Correspondence: Jan A. Till, MD, Department of Paediatric Cardiology, Royal Brompton & Harefield NHS Trust, Sydney Street, London SW3 6NP, UK.


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