Multimodality Imaging of Right-Sided (Tricuspid Valve) Papillary Fibroelastoma: Recognition of a Surgically Remediable Disease
A 61-year-old Hispanic woman presented with recurrent paroxysmal pleuritic chest pain and severe dyspnea. Between episodes, the patient was normally functional and asymptomatic. There was no prior history of structural cardiac, coronary, pulmonary, or systemic disease. Pulmonary CT angiography did not reveal any emboli. Transthoracic and transesophageal echocardiography showed a mobile mass of moderate size and shimmering edges, measuring 1.3 × 0.9 × 1.1 cm, attached to the septal tricuspid valve (TV) leaflet. Pulmonary pressure was minimally elevated at 35–40 mm Hg. Anticoagulation with rivaroxaban (oral factor Xa inhibitor) was initiated. A pedunculated mass with peripheral shimmering frond-like appearance and stalk attached to the septal TV leaflet was seen prolapsing through the TV with tricuspid regurgitation (fig. ). CT pulmonary angiography did not show gross filling defects to suggest pulmonary embolism (PE) but revealed a mobile mass visible in the orthogonal image planes (fig. ). Cardiac MRI revealed a mobile mass measuring ≥1 cm in multiple dimensions, showing hyperintensity on T2-weighted and inversion recovery sequences (fig. ). A differential diagnosis of papillary fibroelastoma (PF) versus cardiac myxoma was entertained. Cardiac surgery was advised due to size (>1 cm), symptomatology, and the established recurrent embolic potential of the tumor. At surgery, shave excision of the sessile mass, and TV repair using a 30-mm Medtronic Contour ring was performed. Gross pathology revealed a friable mass with surface fronds on a short pedicle, with the typical sea anemone appearance of a PF. Microscopic findings showed avascular branching filamentous fronds with a single endothelial cell covering. Each frond had a fibrous core with loose connective tissue rich in mucopolysaccharide (fig. ). Postoperatively, the patient continued anticoagulation for 8 weeks. To date, the patient remains asymptomatic at rest and exercise.