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March 21, 2026European Journal of Preventive Cardiology

Genetic sequencing diagnoses atypical CPVT via a likely pathogenic RYR2 variant following sudden cardiac arrest.

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Why the study?

Interpreting novel variants in CPVT is challenging due to the high polymorphism of RYR2, complicating diagnosis when clinical presentations do not match classic profiles.

Population

1 14-year-old male who suffered out-of-hospital cardiac arrest

Design

Case report

Key result

A 14-year-old male with sudden cardiac arrest and atypical clinical presentation was diagnosed with CPVT after genetic sequencing revealed a likely pathogenic RYR2 variant (c.12370A>G).

Authors

TKTomasz M. KsiążczykKOKlaudia ObsznajczykDCDorota Czapczak

Discussion

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Overview

Supports genetic testing for CPVT in SCA survivors with atypical arrhythmias; extends known phenotypic spectrum.

Key Points

  • This case aims to explore the diagnostic difficulties in identifying catecholaminergic polymorphic ventricular tachycardia (CPVT) in a young patient who experienced sudden cardiac arrest.
  • Case report of a 14-year-old male with sudden cardiac arrest during physical activity
  • Monitoring included ECG, echocardiography, and genetic sequencing
  • Patient's treatment involved defibrillation and medications (propranolol, flecainide)
  • Patient experienced ventricular fibrillation, treated successfully with defibrillation
  • Genetic sequencing identified a rare RYR2 variant associated with CPVT
  • No further arrhythmic events occurred after treatment, and family was referred for evaluation

Study Design

Type

Case Report (n=1)

Multicenter

No

Structured PICO

P
Population
14-year-old male who suffered out-of-hospital cardiac arrest due to ventricular fibrillation
I
Intervention
Genetic sequencing identifying RYR2 variant (c.12370A>G) and treatment with propranolol and flecainide

Genetic testing can guide the diagnosis of CPVT in sudden cardiac arrest survivors when clinical presentation does not fully match the classic profile.

Cite This Study

Książczyk et al. (2026) conducted a case report in Sudden cardiac arrest / Catecholaminergic Polymorphic Ventricular Tachycardia (n=1). Propranolol and flecainide was evaluated. A 14-year-old male with sudden cardiac arrest and atypical clinical presentation was diagnosed with CPVT after genetic sequencing revealed a likely pathogenic RYR2 variant (c.12370A>G).

synapsesocial.com/papers/69be36e36e48c4981c6762d0https://doi.org/10.1093/eurjpc/zwag115.052
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