A rare case of spontaneous asymptomatic ventricular tachycardia due to arrhythmogenic right ventricular dysplasia (ARVD)

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Conference Proceeding

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Publication Title

J Am Coll Cardiol


Background Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited cardiomyopathy that can lead to heart failure and sudden cardiac death. This challenging diagnosis is based on clinical, electrocardiographic, and radiographic findings, along with suggestive genetic testing. Case A 72 year old man presented with stable ventricular tachycardia. 2D echo demonstrated an EF of 45%, grade I diastolic dysfunction, and LV hypokinesis. Left heart catheterization revealed non-obstructive coronary disease. Cardiac MRI revealed: dilated RV with basal RV wall akinesis; RV end-diastolic volume index 134 mL/m2, global LV hypokinesis, an area of mid-myocardial delayed gadolinium enhancement of the mid-inferoseptal LV myocardium at the RV insertion point, and no associated lymphadenopathy. Subsequent Invitae genetic testing revealed desmoplakin (DSP) gene mutation. The patient was managed with AICD placement and sotolol initiation. Decision-making In this rare case of asymptomatic stable VT, cardiac MRI findings met major criteria for ARVD. The differential diagnosis included cardiac sarcoidosis, however contrast enhanced CT imaging did not reveal hilar lymphadenopathy, and cardiac MRI was unimpressive for sarcoidosis. Furthermore, DSP gene mutation is associated with autosomal dominant ARVD. Conclusion The diagnosis of ARVD can be determined with cardiac imaging and genetic testing. It is important to rule out similar diagnoses by looking for differentiating features, such as hilar lymphadenopathy and myocardial granulomatous disease which is frequently seen in cardiac sarcoidosis, but not in ARVD.





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