title: Arrhythmogenic cardiomyopathy in a patient with Noonan syndrome.
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.
authors.affiliation: Department of Pathology, School of Medicine, Universidad Nacional de La Plata, La Plata, Argentina.
authors.forename: Eugenia
authors.initials: E
authors.lastname: Altamirano
authors.forename: Ricardo
authors.initials: R
authors.lastname: Drut
mesh_terms: D000273:Adipose Tissue / Q000473:pathology; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications / Q000473:pathology* / Q000601:surgery; D049108:Cell Enlargement; D002648:Child; D004452:Echocardiography; D004699:Endocardium / Q000473:pathology; D006801:Humans; D008297:Male; D032383:Myocytes, Cardiac / Q000473:pathology; D009634:Noonan Syndrome / Q000150:complications / Q000473:pathology* / Q000601:surgery; D054088:Ventricular Septum / Q000601:surgery