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Arrhythmogenic cardiomyopathy in a patient with Noonan syndrome
Eugenia Altamirano1, Ricardo Drut
1Department of Pathology, School of Medicine, Universidad Nacional de La Plata, La Plata, Argentina.
Abstract:
Arrhythmogenic ventricular cardiomyopathy (AVC) presents with fat replacement of the myocardium, most commonly of the right ventricle, and ventricular arrhythmias. We report an 11-year-old boy with Noonan syndrome, ventricular arrhythmias, and an ultrasound depicting hypertrophy of the ventricular septum with subaortic stenosis. A surgical resection of the left side of the ventricular septum revealed a thick fibroelastotic endocardium covering a broad band of mature adipose tissue focally containing myocardial cells, fibrosis and chronic inflammatory infiltrates. The two layers covered a band of hypertrophic myocardiocytes with mild interstitial fibrosis. Arrhythmogenic ventricular cardiomyopathy has not been previously reported in the Noonan syndrome.
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