Abstract 12985: Extensive Valvular and Mural (Endocardial) Papillary Elastofibromatosis

Abstract

Introduction: Papillary fibroelastomas (PFEs) are benign, often isolated, valvular tumors of endocardial origin although they may originate from non-valvular tissue and can be multiple. We report a unique case of PFE that involved 2 cardiac valves and nearly entire left ventricular (LV) endocardial surface. Case: A 73-year-old white woman with a history of non-valvular atrial fibrillation was admitted with intermittent worsening headaches and loss of peripheral vision in the right eye. An occipital-parietal hematoma was detected on brain computed tomography. Transthoracic echocardiogram showed multiple mobile masses on the mitral valve and the subvalvular apparatus. Transesophageal echocardiography showed additional involvement of the aortic valve and LV endocardium and no otherpotential source of embolism. She experienced recurrent systemic embolism and underwent surgery for the removal of cardiac masses. At surgery, more than 40 loosely attached individual and clustered PFEs (some shown in Figures A and B) confirmed by histology (Figure C) covered both aortic (Figure D and E) and mitral (Figure F) valves and nearly the entire LV endocardial surface (including papillary muscles,Figure G). All LV masses were removed and aortic and mitral valves were replaced. Conclusion. Although cases of multiple valvular PFEs have been reported previously, we report a unique case with a large number of PFEs that involved 2 cardiac valves and the entire mural surface of LV. We have used the term papillary elastofibromatosis to describe such an extensive tumor growth.