Curative Ablation In A 12-Month-Old Baby With Severe Ventricular Systolic Dysfunction And Incessant Ventricular Tachycardia

Cardiomyopathies in the paediatric population portend a poor prognosis being characterized by a particularly accelerated and therapy refractory clinical course.1,2 Recognition and effective treatment of potential secondary causes may play a crucial role, leading to recovery in a not negligible proportion of cases. Left-ventricular dysfunction associated with wide QRS tachycardia is a quite common presentation, depicting two possible clinical pictures: ventricular tachycardia complicating idiopathic cardiomyopathy and primary tachycardiomyopathy. The distinction between the two might be pivotal, particularly in the paediatric population since, recognition of a tachycardiomyopathy must prompt an aggressive rhythm control. In this context, the role of the conduction system is being recognized in many incessant tachycardias with or without an underlying structural heart disease.3,4 The two most clinically relevant scenarios are fascicular tachycardia and junctional ectopic tachycardia (JET).5 The former is usually sustained by a reentry within a diseased Purkinjie network or involving the surrounding ventricular myocardium. The latter is thought to be caused by abnormal automaticity within the atrioventricular junction, and thus usually display a narrow QRS. Although JET is more frequently associated with a known structural injury such as myocarditis or post-operative status, still it should be kept in mind in the differential diagnosis, since isolated-idiopathic cases have been described as well.6 These arrhythmias, when displaying an incessant course may be responsible for systolic impairment, independent of their specific individual prognosis.7 We are describing a case of a 12-month-old baby that presented with a severe systolic dysfunction and almost incessant wide QRS tachycardia causing rapidly worsening heart failure despite full medical therapy. The arrhythmia was finally diagnosed as idiopathic left-ventricular fascicular tachycardia (ILVT) at the invasive electrophysiologic study, and transcatheter ablation through transseptal route was performed. The procedure turned out to be effective both in eliminating the …