Primary cardiac tumors are uncommon clinical entities with an incidence of 0.0017% to 0.03% of all autopsies. Cystic tumor of the atrioventricular (AV) node, also known as mesothelioma of the AV node, is a benign congenital tumor that is located in the triangle of Koch in the AV nodal region of the atrial septum of the heart. It comprises of 2.7% of cardiac tumors and is the most common primary cardiac tumor causing sudden death. We herein review the etiology, presentation, differential diagnosis, diagnosis, management, surgical approaches and outcomes of this rare tumor.
Keywords: AV node mesothelioma; Cardiac tumor; cystic tumor of the atrioventricular (AV) node; pathology; sudden cardiac death.