Left Ventricular Involvement in Arrhythmogenic Right Ventricular Cardiomyopathy
A 55-year-old woman presented with an 18-month history of progressive dyspnoea, exertional chest tightness, and palpitations associated with light-headedness. Resting electrocardiogram showed sinus rhythm and incomplete right bundle branch block with nonsustained ventricular tachycardia of left bundle branch morphology on 24-hour recording. Thallium scintigraphy reported partial thickness infarction of the inferior wall and reversible ischemia in the anterior wall. Cardiac catheterization showed “disorganized” function of the left ventricle (LV) and severe hypokinesis of the inferior wall and mid-anterior wall. The left coronary system was normal, and the right coronary showed a 50% distal stenosis. Cardiovascular magnetic resonance (CMR) assessment confirmed an enlarged LV with severe hypokinesis of the inferior wall and hypokinesis and focal thinning of the mid anterior wall. The right ventricle (RV) was also abnormal with reduced systolic function, elevated end-systolic volume, dyscoordinate free wall contraction, and an area of prominent dyskinesis in the basal to mid inferolateral RV (Figure 1). T1-weighted spin-echo images showed fat deposits within the myocardium of the anterior and inferior LV walls and throughout the RV free wall with marked thinning in the areas of dyskinesis (Figure 2). There was no LV or RV myocardial infarction or macroscopic fibrosis on late enhancement imaging after gadolinium. These features are consistent with arrhythmogenic right ventricular cardiomyopathy with florid LV involvement. Fatty subepicardial deposits of the LV have been documented at autopsy in 64% of arrhythmogenic right ventricular cardiomyopathy cases but are rarely so clearly seen in life. The patient has been referred for electrophysiological testing and investigation of first-degree relatives.
Dr McCrohon is supported by the Marian Silcock Cardiac Fellowship, CORDA, UK
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke's Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
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