Implantable defibrillators in primary prevention of genetic arrhythmias. A shocking choice?

Graphical Abstract Graphical Abstract This figure illustrates three different approaches to the management of cardiomyopathies and of channelopathies. The first approach, on the left, reflects a currently growing trend and relies on the semi-passive acceptance of algorithms originated by ‘electronic risk calculators’ and ‘risk scores’: in this situation a non-expert physician, instead of deciding on his/her own, turns to and fully relies on the algorithm and this automated response predominantly favours the choice of ICD implant over alternative therapies. The second approach, in the middle, represents the wise combination of an experienced cardiologist using also the data from an electronic risk calculator and thereby considering all possible reasonable choices. The third approach, on the right, is the traditional one, based on the personal choice by an expert clinician who first integrates the specific characteristics of each patient and then makes an unbiased choice among the different therapeutic options available. The second and third approaches are equally valid; we wish to underscore that the third one implies that the ‘true expert’ does not need electronic risk calculators. By contrast, we regard as potentially dangerous the situation in which a doctor without specific expertise in uncommon and life-threatening disorders decides essentially on the sole basis of the algorithm. In all three approaches the final decision must be shared with the patient. Finally, after installing the initial therapy, risk stratification for SCD should be re-assessed on a regular basis during follow-up because the risk of SCD may change over time either increasing, due to the disease progression, or decreasing when the therapies are effective. The panel illustrating “Cardiac sympathetic denervation” is reproduced with permission from Collura et al.147