Abstract
Clinical presentation of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) usually is nonspecific in young, representing a challenge diagnostic. We present an 11-year-old male patient, with a syncope event and normal ECG in the first medical assessment. Eight months later, presented a new syncope event, but now with 1st degree block that evolved to complete AV block in 24 hrs in ECG. In Tertiary Medical Care Hospital showed sinus arrhythmia with nodal rescues. The echocardiographic assessment showed dilatation and global hypokinesia in the Right Ventricle. The MR showed in apex and lateral wall late Gadolinium enhancement indicating fibrosis greater than 20%. The electrophysiologic assessment showed a low-voltage zone in the RVOT, normal stimulation between sinus and AV nodes. Was implanted ICD which identified events of ventricular tachycardia (185 bpm), giving discharges between 21 to 41 Jules, which were not perceived for the patient. Currently continues under cardiology surveillance to eventually receive a heart transplant as definitive treatment
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