2023 Volume 87 Issue 5 Pages 673-
An 84-year-old Japanese woman complaining of dyspnea on exertion for the previous 3 months presented to hospital. Transthoracic echocardiography showed an aortic valve with severe aortic stenosis (AS) (Figure A,B) and a 10-mm mobile high-echoic mass attached to near the edge of the anterior papillary muscle (APM) in the left ventricle (LV; Figure C). We performed a surgical aortic valve replacement and removal of the mass, which was a myxoma-like tumor, 10 mm in diameter (Figure D). Histological examination confirmed the diagnosis of cardiac papillary fibroelastoma (PFE; Figure E,F).
Transthoracic echocardiography showing significantly limited opening of the aortic valve (A,B) and a mass attached to anterior papillary muscle (APM) (A,C; arrow). Gross image of the mass on the APM (D; arrowhead). Microscopic appearance of the excised papillary fibroelastoma demonstrating avascular branches lined by endothelial cells (E,F). Ao, aorta; AV, aortic valve; LA, left atrium; LV, left ventricle; RV, right ventricle.
A meta-analysis of 725 histopathologically confirmed cases revealed PFEs on valves, especially the aortic valve (44%).1 The LV was the most common site of non-valvular occurrence (9%).1 The present case is the first combination of severe AS and a PFE on the APM. The most widely accepted theory of the histogenesis of PFE is microthrombus due to minor endothelial damage.1 It is speculated that accelerated blood flow due to the concentric LV hypertrophy associated with AS may have caused endothelial damage in the APN. In the era of transcatheter aortic valve implantation, careful preoperative observation of intracardiac tumors in patients with severe AS is vitally important to ensure procedures.
K.K. is a member of Circulation Journal’s Editorial Team.
