Case Report (n=1)
No
This case highlights that atypical left dominant arrhythmogenic cardiomyopathy can present with sustained ventricular tachycardia and RBBB morphology, requiring cardiac MRI for reliable diagnosis.
Supports cardiac MRI for diagnosing atypical left-dominant arrhythmogenic cardiomyopathy in sustained VT; leaves open optimal long-term management.
A 56-year-old white man was admitted to hospital because of palpitations and dyspnoea. He had no family history of cardiomyopathy or sudden death, and the electrocardiograms (ECG) of his son and parents were normal. He had an history of palpitations, and in the emergency department ECG showed sustained ventricular tachycardia-flutter with inferior axis right bundle branch block (RBBB) in V1 and heart rate 250 beats per minute. His standard ECG showed a complete RBBB pattern with minor ST-T segment changes. 2D echo and coronary angiography were normal, while magnetic resonance imaging (MRI) revealed a reduction in left mid-lateral wall thickening with hypokinetic movement and mild reduction of ejection fraction. An Extensive sub-epicardial area of conspicuous left ventricular intra-myocardial fat and sub-epicardial left ventricular late enhancement in lateral wall, was also seen at MRI. No abnormalities of right ventricular size, function or tissue characterisation were detected. A diagnosis of atypical left dominant arrhythmogenic cardiomyopathy was made, based on clinical and images basis, as gene testing was not available.
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Guglielmi et al. (2016) conducted a case report in Left dominant arrhythmogenic cardiomyopathy (n=1). Bisoprolol was evaluated on Symptom recurrence and ventricular arrhythmias.
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