Lung ventilation/perfusion scintigraphy shows the efficacy of anticoagulant therapy and surgical treatment for papillary fibroelastoma originating from the tricuspid valve

Papillary fibroelastoma (PFE) is a cardiac tumor that is mainly found on the heart valve and the endocardium of the atria and ventricles. Symptoms such as stroke and myocardial infarction are usually caused by embolization of either the tumor itself or associated thrombus. PFE is known to originate mainly from the left side of the heart, and these cases are—in principle—candidates for surgical resection. On the other hand, cases in which PFE originates from the right side of the heart are rare and reports are limited; thus, the surgical indication is unclear. We herein report a case of symptomatic PFE originating from the tricuspid valve of the heart. In this case, contrast enhanced computed tomography did not show pulmonary embolism; however, lung perfusion scintigraphy showed multiple perfusion defects. The patient was treated by anticoagulant therapy followed by surgical resection. Thereafter, the symptoms disappeared and the multiple perfusion defects improved on lung perfusion scintigraphy, demonstrating the efficacy of the anticoagulant therapy and surgical resection for PFE in the right side of the heart. Learning objective Papillary fibroelastoma (PFE) originating from the tricuspid valve of the heart is rare and reports on this topic are limited. Thus, the surgical indication is unclear. In the present case, lung perfusion scintigraphy indicated pulmonary embolism due to PFE, while contrast enhanced computed tomography did not. Anticoagulant therapy and subsequent surgical resection were considered effective for PFE in the right side of the heart.