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Distinct U wave changes in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT)
Yoshiyasu Aizawa1, Satoru Komura, Shinsuke Okada
1Division of Cardiology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
Insights
Catecholaminergic polymorphic ventricular tachycardia (CPVT) involves U-wave alterations, offering new insights into this condition. These ECG findings, including U-wave alternans, may be key to understanding CPVT pathogenesis.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder linked to fatal arrhythmias and sudden cardiac death.
- Understanding the electrocardiogram (ECG) characteristics of CPVT is crucial for diagnosis and management.
Observation:
- This study investigated U-wave alterations in seven patients from six families diagnosed with CPVT.
- Patients experienced bidirectional tachycardia and polymorphic ventricular tachycardia (VT) induced by exercise or isoproterenol.
- A novel mutation in the ryanodine receptor 2 (RyR2) gene was identified in two families.
Findings:
- U-wave alternans were observed in two patients, particularly in leads V3-V5, during ventricular pacing and post-exercise recovery.
- One patient exhibited a U-wave inversion that normalized after sinus arrest, accompanied by T-wave changes.
- These specific U-wave phenomena were not observed in other subjects undergoing similar provocative testing.
Implications:
- U-wave alterations may represent a significant, previously unrecognized ECG marker for CPVT.
- Further research is needed to elucidate the genesis and clinical significance of these U-wave changes in CPVT.
- Identifying novel genetic mutations (RyR2) and associated ECG findings advances the understanding of CPVT pathophysiology.
Abstract:
Although catecholaminergic polymorphic ventricular tachycardia (CPVT) is associated with fatal ventricular arrhythmias and sudden death, the ECG findings are not fully understood. In this paper, we report on alterations in the U-wave. Seven patients from 6 families with CPVT in which bidirectional tachycardia and polymorphic VT were induced by exercise or isoproterenol infusion visited our hospitals. VT was not inducible by programmed electrical stimulation. A novel gene mutation of the ryanodine receptor 2 (RyR2) was confirmed in 2 families. In one of these patients, U-wave alternans was observed following ventricular pacing at 160 beats/min. In the other patient, U-wave alternans was observed during the recovery phase after the exercise stress test, which was terminated because of polymorphic VT. In both cases, leads V3-V5 were the leads showing alternans most clearly. In the third patient, a negative U-wave became positive following a pause from sinus arrest and a change in T-wave was also noted. Since such findings were not found in the other subjects who underwent electrophysiologic study, isoproterenol infusion or exercise stress testing, the phenomenon seems to be relevant to the underlying pathogenesis of CPVT. The genesis and significance of U-wave alteration need to be determined.
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