CPVT

First episode of syncope at 7-12 years, triggered by exercise / emotions.

1. Physiopathology

2. Treatment

A 14‑year‑old boy with exercise‑induced syncope is suspected to have catecholaminergic polymorphic ventricular tachycardia (CPVT). An exercise stress test cannot be performed due to orthopedic limitations. Which test may be considered to aid the diagnosis?

A. Adenosine challenge

B. Electrophysiological study with programmed stimulation

C. Epinephrine or isoproterenol challenge

D. Tilt‑table test

E. Standing ECG for QT variability

> ESC guidelines recommend non‑selective β‑blockers in CPVT and state that when exercise testing is not possible, an epinephrine or isoproterenol challenge may be considered for diagnosis.

The following ECG was recorded in a 16 y old male with recurrent syncope during exercise, with normal blood tests, no structural anomalies on cMRI and without a family history of SCD. What is the most adequate management strategy?

A) Start flecainide treatment.

B) Start nadolol treatment.

C) Start mexiletine treatment.

D) ICD implantation.

E) Schedule left cardiac sympathetic denervation.

Nadalol