EMedHome's Clinical Pearl
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic condition characterized by epinephrine-induced polymorphic VT leading to syncope and sudden death in children and adolescents. Epinephrine can be counterproductive and life-threatening when administered for polymorphic VT and ventricular fibrillation arrest caused by CPVT.
CPVT-associated cardiac arrest presents a unique challenge because it must be managed differently. Most cases are children between 4 and 14 years old. Common triggers include exercise or emotion. CPVT is an important differential diagnosis of any young person who has suffered an unexplained sudden cardiac arrest.
Features that should raise suspicion include that the patient was previously well; cardiac arrest occurred during a physical activity or with excitement; the ECG shows frequent ventricular ectopy (usually but not always polymorphic); ventricular ectopy becomes more frequent (or joins to form VT) with epinephrine; and bidirectional VT (where ventricular complex QRS axis alternates by 180 degrees) is virtually pathognomonic.
Once recognized, optimum therapies are IV opiates and general anesthetics. These work to reduce the catecholaminergic stimulation to break the cycle that triggers the VT. (Sources: Pediatr Crit Care Med. 2019;20:26; http://bit.ly/2V5Dt9I; Heart. 2017;103:889; Pediatr Emerg Care. 2017; 33:427.)
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