We presented a case of sudden death in a 14 year old boy with two remarkable points, successful resuscitation at school using an AED and diagnosis of ARVC. Widespread introduction of AED has resulted in improved outcome from ventricular fibrillation. However, for a number of reasons, including the cost of these devices and unawareness of the importance of public access defibrillation also for children, AEDs are not found in most Spanish schools. According to current international guidelines [
5], a standard AED should be used in children over 8 years of age and a device with dose attenuator should be used in children between 1 and 8 years. If no such system is available, an unmodified adult AED may be used in children older than 1 year [
5,
6]. In our case, a BLS trained witness started CPR until a paramedic rapid response unit with a standard AED arrived at the scene. It is well known now that time to defibrillation is the major survival factor in out-of-hospital cardiac arrest due to a shockable rhythm. Also, AEDs are easy to use by minimally trained lay responders. Therefore, we consider that AEDs should be readily accessible at schools and teachers (especially physical education coaches) should be trained to use these devices. Although the absolute risk for young athletes remains low when compared to adult population, the risk excess when compared to general population in their age group suggest the need for systems able to respond to unexpected events [
7,
8]. The American Academy of Pediatrics, endorsed a standardized pre-participation athletic evaluation form that presents several useful questions for cardiovascular risk assessment [
9]. Also, Campbell and Berger [
10] developed a standardized cardiovascular risk-assessment form, which could be used by any provider, for any child, at any age, at any time. Recently, the American Heart Association published an update of recommendations and considerations related to screening for cardiovascular abnormalities in competitive athletes [
4].
Patients with ARVC usually have ventricular premature beats and non-sustained or sustained ventricular tachycardia demonstrating a left bundle branch block pattern. However, since ventricular tachycardia may also degenerate into ventricular fibrillation, sudden death may be the first manifestation of ARVC, as it was in our case. In recent years, ARVC has been more and more recognized as an important and frequent cause of ventricular tachyarrhythmias and sudden cardiac death, particularly in young patients and athletes, with apparently normal hearts [
3,
10]. ARVC is responsible for 3–5 % of sudden death for individuals younger than 65 years [
11]. The diagnosis is based on electrocardiographic abnormalities and the identification of regional or global right ventricular dysfunction and fibrolipomatosis [
12‐
14]. Electrocardiographic changes include inverted T waves in the right precordial leads beyond V1 in the absence of right bundle branch block. Right ventricular late potentials in the form of epsilon waves may be found on the routine 12 lead ECG [
13]. An implantable cardioverter-defibrillator is indicated in selected high-risk patients with ARVC as in our case. ARVC occurs in a familial fashion in 30–50% and appears to follow autosomal dominant inheritance [
11]. Therefore, it may be important to inform other family members about this fact and to instruct those members to also promote education in CPR and use of AEDs.