Description

A 50-year-old woman who was a non-smoker presented with worsening dyspnoea (NYHA III) over 3 years. Physical examination, chest x-ray and ECG were unremarkable. Transthoracic echocardiography revealed a small ball-like structure attached to the tricuspid valve. Transoesophageal echocardiogram (figure 1) confirmed a mobile mass, 1×0.8 cm in dimension, on the tricuspid valve suspicious of a papillary fibroelastoma (PFE). A 64-slice ECG-gated multidetector CT (Toshiba Aquilion) was performed to assess coronary anatomy, which was normal and revealed normal appearances of the lungs, excluding gross chronic pulmonary embolic disease. She underwent open surgical resection of the mass on cardiopulmonary bypass without complication (figure 2). Histology confirmed the diagnosis of PFE. Her symptoms of effort dyspnoea disappeared immediately after surgery with a dramatic improvement in exercise capacity.

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Mid-oesophageal right ventricular-inflow view transoesphageal echocardiography image illustrating highly mobile mass (arrow) adherent to tricuspid valve (left panel) and close-up (right panel). AV, aortic valve; LA, left atrium; RA, right atrium; TV, tricuspid valve.

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Intraoperative view at time of surgical resection on cardiopulmonary bypass (left panel), with gross macroscopic appearance of resected papillary mass (right panel).

PFEs, the second commonest primary cardiac neoplasms, are rare; 90% occur on the heart valves with the aortic and mitral valves being most frequently affected.1 Grossly, PFEs resemble sea anemones with large surface area frond-like projections—potentially the nidus for thrombus formation. While most cases are identified incidentally, PFEs may cause serious sequelae, including myocardial infarction, stroke or death. Right-sided PFEs have been cited as rare causes of repeated pulmonary embolism2 and intermittent dyspnoea.3 Embolic phenomena may be secondary to tumour fragments or platelet/fibrin clots arising from the tumour surface. Surgical resection is the treatment of choice, because of the risk of embolisation, and is curative. The dramatic clinical improvement supports the hypothesis of recurrent microembolisation as the cause of dyspnoea in our case.