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Invasive electrophysiology in paediatric and congenital heart disease
  1. D J Abrams
  1. Department of Cardiac Electrophysiology, St Bartholomew’s Hospital, London, UK
  1. Correspondence to:
    Dr Dominic Abrams
    Department of Cardiac Electrophysiology, St Bartholomew’s Hospital, 60 Dominion Hose, Bartholomew Close, West Smithfield, London, EC1A 7BE, UK; d.abrams{at}qmul.ac.uk

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In the last decade invasive electrophysiological studies (EPS) and radiofrequency catheter ablation (RFCA) have become progressively common, driven predominantly by high success and low complication rates, coupled with the inefficacy and side effects of many antiarrhythmic agents. As our understanding of different arrhythmia mechanisms and successful ablative strategies has advanced, RFCA has been applied to increasingly complex substrates and broader clinical indications. Two such examples are young adults with native or surgically repaired congenital heart disease and children with structurally normal hearts. In such situations arrhythmia management should be performed on an individualised basis, considering the potential risks and benefits of different strategies.

In young children the natural history of the arrhythmia, the effects of medication and potential life-threatening complications must all be weighed against the risk of RFCA. In adults with congenital heart disease, arrhythmia management must be closely coordinated between electrophysiologist, cardiologist and cardiac surgeon to optimise haemodynamic performance, potentially performed in conjunction with surgery or other percutaneous interventions. This article aims to review the current indications for EPS and RFCA in both groups of patients, specifically focusing on areas of ongoing interest and debate.

CHILDREN WITH STRUCTURALLY NORMAL HEARTS

Arrhythmia mechanism and presentation

The vast majority of supraventricular tachycardias (SVT) seen in children without congenital cardiac anomalies are atrioventricular reentry tachycardia (AVRT) facilitated by an accessory pathway (AP), atrioventricular nodal reentry tachycardia (AVNRT) and focal atrial tachycardia (FAT). Presentation of AVRT is most common in the neonatal and infant groups, with the vast majority free from symptomatic arrhythmia by the end of the first year of life, although recurrence in later childhood or adolescence is well recognised. Spontaneous remission in those older than 5 years of age at presentation is much less common.1 Conversely AVNRT is rare in early childhood, becoming more frequent with increasing age, mirroring patterns seen in adult practice. Other less common …

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