Key result
Epinephrine testing and EP study unmask parahisian focal atrial tachycardia mimicking CPVT, successfully treated with flecainide.
Why the study?
Early symptoms of benign arrhythmias and life-threatening inherited arrhythmogenic syndromes can be similar, requiring thorough workup to avoid misdiagnosis or delayed diagnosis.
Case Report (n=1)
No
Focal atrial tachycardia with alternating bundle branch and fascicular aberrant conduction can closely mimic catecholaminergic polymorphic ventricular tachycardia, and an invasive electrophysiological study can safely differentiate aberrancy from life-threatening ventricular arrhythmias.
Answers are grounded in this paper and cite their sources.
Supports invasive EPS with epinephrine to identify treatable atrial tachycardia mimics in select suspected CPVT cases; leaves open broader diagnostic adoption.
Background: In childhood and adolescence, cardiac arrhythmias are often benign in the absence of congenital heart defects. Nevertheless, life-threatening inherited arrhythmogenic syndromes can become clinically manifest in early childhood. As early symptoms may be similar in both conditions, thorough workup is fundamental to avoid delayed diagnosis and misdiagnosis. Case summary: We present the case of a 26-year-old Caucasian female patient who presented with recurrent non-sustained polymorphic wide complex tachycardia. Structural heart disease was excluded by echocardiography as well as cardiac magnetic resonance imaging. Due to wide complex extrasystoles and couplets with alternating QRS axis occurring at low levels of physical exertion, catecholaminergic polymorphic ventricular tachycardia (CPVT) was suspected and further investigated. Epinephrine testing in combination with an electrophysiological (EP) study with placement of a coronary sinus catheter and subsequent programmed stimulation ruled out CPVT and unmasked wide complex tachycardia as varying aberrant conduction of focal atrial tachycardia (FAT). 3D-navigated mapping of FAT revealed a direct parahisian origin. Due to significantly increased risk of atrio-ventricular (AV) block during ablation, the patient refused ablation and preferred medical antiarrhythmic therapy. Discussion: Given the consequences of both, delayed diagnosis and misdiagnosis of CPVT, thorough workup is fundamental. In case of doubt regarding potential aberrant AV conduction in the context of wide complex tachycardia, an invasive EP study may easily and safely prove or rule out aberrancy.
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Seewald et al. (2023) conducted a case report in Focal atrial tachycardia mimicking catecholaminergic polymorphic ventricular tachycardia (n=1). Electrophysiological study with epinephrine testing and flecainide therapy was evaluated on Diagnosis of focal atrial tachycardia with aberrant conduction and freedom from symptoms. Invasive electrophysiological study with epinephrine testing ruled out CPVT and unmasked parahisian focal atrial tachycardia with varying aberrant conduction, which resolved with flecainide 50 mg twice daily.
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