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The author reports no conflicts of interest for the published content. Address correspondence to: George F. Van Hare, MD, Pediatric Cardiology, Campus Box 8116, Washington University School of Medicine, 1 Children‘s Place, St. Louis, MO 63110, USA. Email: vanhare@wustl.edu. In this issue of The Journal of Innovations in Cardiac Rhythm Management, Dr. Townsend and Dr. Aziz have provided a very good review1 of the field of primary-prevention implantable cardioverter-defibrillators (ICDs) in children, focusing specifically on those with inherited arrhythmias. I am senior enough to have observed a progression of attitudes concerning primary prevention ICDs in children and, in particular, those with an established diagnosis of long-QT syndrome (LQTS). Initially, when ICDs were only available epicardially, via sternotomy or thoracotomy, we did not recommend them as primary prevention. Even with the arrival of the first transvenous systems, we were still quite hesitant, as we were uncertain as to whether they would be effective, given the prospect of possible electrical storm following an appropriate shock. Subsequently, when it became clear that ICDs were indeed effective in patients with LQTS,2 we did implant a number of ICDs for primary prevention. We now know, based on careful clinical studies in genotyped patients, that most patients with LQTS (particularly those with LQT1) do very well with appropriate β-blocker therapy.3 However, questions remain for the less common and less-well-studied diagnoses such as LQT3, Timothy syndrome, short-QT syndrome, and other rare mutations, for which the effectiveness of β-blockers has not been well-established.

Implantable Cardioverter-defibrillators for Primary Prevention in Pediatric Patients: What’s Next?