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BMJ Case Rep. 2010; 2010: bcr10.2009.2376.
Published online Apr 9, 2010. doi:  10.1136/bcr.10.2009.2376
PMCID: PMC3047487
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An unusual cause of severe dyspnoea—papillary fibroelastoma of the tricuspid valve
Mike El-Mourad,1 Arash Yavari,2,3 Eric McWilliams,3 and David Walker3
1Royal Sussex County Hospital, Medicine, Eastern Road, Brighton, BN2 5BE, UK
2University of Oxford, Department of Cardiovascular Medicine, John Radcliffe Hospital, Headley Way, Oxford, Oxfordshire, OX3 9DU, UK
3Conquest Hospital, Cardiology Department, The Ridge, St Leonards-onSea, East Sussex, TN377RD, UK
Correspondence to Eric McWilliams, eric.mcwilliams/at/esht.nhs.uk
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.
Figure 1
Figure 1
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, (more ...)
Figure 2
Figure 2
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.
Acknowledgments
Mr Uday Trevedi, Cardiac Surgeon, Royal Sussex Hospital, Brighton.
Footnotes
Competing interests: None.
Patient consent: Patient/guardian consent was obtained for publication.
REFERENCES
1. Sun Jing Ping, Asher Craig, et al. Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients. Circulation 2001; 103: 2687–93. [PubMed]
2. Waltenberger J, Thelin S. Images in cardiovascular medicine. Papillary fibroelastoma as an unusual source of repeated pulmonary embolism. Circulation 1994; 89: 2433. [PubMed]
3. Georghiou Georgios P, Erez Eldad, Vidne Bernardo A, et al. Tricuspid valve papillary fibroelastoma: an unusual cause of intermittent dyspnoea. Eur J Cardiothorac Surg 2003; 23: 429–31. [PubMed]
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