Atypical Presentation of Right Ventricular Cardiac Hamartoma in a Young Man

Abstract

Cardiac tumors in adults are exceedingly rare and usually benign. We describe a 29-year-old man with a previous diagnosis of interventricular septal hypertrophy who presented with increasing severity of dyspnea and fatigue. Work-up revealed a 4.9 × 3.7 cm mass at the base of the interventricular septum. Biopsy revealed a benign cardiac hamartoma atypically located in the right ventricle, and the mass was resected via right ventriculotomy.

Left Ventricular Outflow Tract Obstruction Due to Cardiac Hamartoma

“…Histologically, HMCM is composed of hypertrophic, disorganized mature cardiac myocytes with sarcoplasmic vacuolization. Frequently, it is associated with proliferated blood vessels [3]. Given the need to exclude malignancy and the similarity of HMCM to normal cardiac tissue without definitive histopathological findings, HMCM can be considered a diagnosis of exclusion.…”

“…Moreover, most cases are pediatric, as it is strongly associated with tuberous sclerosis [4]. In addition, HMCM, located in the septal or ventricular area, could mimic HCM as histological features are almost identical without good pathological distinction, causing misdiagnosis in some patients [3,5,6]. A family history of HCM and more diffuse myocardial involvement with septal localization may help to distinguish HCM from HMCM.…”