Key result
Propafenone completely suppresses recurrent syncope over 35-year follow-up in CPVT case report.
Why the study?
Does propafenone prevent syncope and ventricular arrhythmias in a patient with RyR2 mutation-associated CPVT?
Case Report (n=1)
No
Does propafenone prevent syncope and ventricular arrhythmias in a patient with RyR2 mutation-associated CPVT?
Propafenone may represent an effective, durable therapeutic option for patients with CPVT by providing combined sodium channel and beta blockade.
May support propafenone monotherapy in select CPVT cases; hypothesis-generating and requires prospective validation.
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inheritable cardiac channelopathy characterized by malignant polymorphic ventricular tachycardias (pVT) that are triggered by catecholaminergic stress.1,2 Affected patients typically suffer from syncope during physical exercise and/or (aborted) sudden cardiac death within the first 2 life decades.3 The most common genetic background of the disease is mutations in the gene encoding for the cardiac ryanodine receptor (RyR2).4
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Marx et al. (2018) conducted a case report in Catecholaminergic polymorphic ventricular tachycardia (CPVT) (n=1). Propafenone was evaluated on Recurrence of syncope and palpitations during exercise. Propafenone monotherapy completely suppressed recurrent syncope and mitigated palpitations over a 35-year follow-up in a patient with CPVT.
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