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Shock to the heart: cardiac implantable devices’ bad name in adults with tetralogy of Fallot
  1. Jason F Deen1,2,
  2. Jordan M Prutkin2
  1. 1Division of Cardiology, Seattle Children’s Hospital, University of Washington, Seattle, Washington, USA
  2. 2Division of Cardiology, Department of Medicine, University of Washington, Seattle, Washington, USA
  1. Correspondence to Dr Jordan M Prutkin, Division of Cardiology, University of Washington, Seattle, WA 98195, USA; jprutkin{at}cardiology.washington.edu

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Because of the successes of paediatric cardiac care, there is a burgeoning population of adults with congenital heart disease with an ever-increasing complexity of underlying cardiac disease necessitating interventions to stave off mortality and morbidity.1 This is typified in patients with tetralogy of Fallot (TOF), the most common form of cyanotic congenital heart disease. While surgical palliation is usually completed in infancy, patients with TOF exhibit significant mortality and morbidity as they age, including incident myocardial dysfunction, need for pulmonary valve replacement and an elevated risk of arrhythmia and sudden cardiac death.2 These events are usually heralded by ventricular pressure and volume loading from significant pulmonary valve incompetence, a common haemodynamic derangement after pulmonary transannular patching used as a part of complete palliation for TOF.

The arrhythmia burden in adult patients with repaired TOF is substantial. In a large multicentre study, 43.3% of 556 patients aged 36.8±12.0 years had either a sustained arrhythmia or required intervention for arrhythmia.3 Ventricular arrhythmias occurred in 14.6%, usually associated with multiple surgeries, a widening QRS duration and left ventricular dysfunction. It was noted that there was an uptick in incident atrial fibrillation and ventricular arrhythmia …

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