INTRODUCTION:Primary cardiac tumors have an incidence of 0.02% to 0.33% (1). Papillary Fibroelastoma (PFE) is a benign cardiac tumor, comprises less than 10%, and the most common primary tumor of heart valves. They range from 2-70mm in size. The specific location from most to least common are: aortic valve, mitral valve, tricuspid valve, pulmonic valve, and cardiac chambers.Before echocardiogram, PFE was diagnosed by autopsy or incidentally at surgery. These lesions occur on the valves or endothelial surfaces of the heart and may be detected by echocardiography, cardiac catheterization, open-heart surgery or autopsy. Surgical excision of PFE is justified because of their high potential for embolization and propensity for life-threatening complications. In most cases, valve-sparing management is possible with no observed recurrence after complete excision.
CASE PRESENTATION:A 47-year-old woman was referred to a cardiologist after a diastolic murmur was heard at the left fourth intercostal space with complaints of occasional fatigue after a routine physical exam. A chest x-ray, electrocardiography, stress test, and cardiac catherization were normal. On echocardiogram, a mass with a peduncle was visualized on the atrial aspect of the tricuspid valve. A cardiac surgical consultation was obtained. Patient was admitted for elective open-heart surgery. A median sternotomy and bicaval cannulae was performed. The right atrium was opened and a polypoid gelatinous tumor, measuring 15mm, was visualized on the septal leaflet of the tricuspid valve. After resecting the tumor with the attached piece of valve, the defect was oversewn followed by an annuloplasty. After separation from bypass, transesphogeal echocardiogram revealed a properly functioning tricuspid repair.The patient was discharged home on post-operative day 4 with an uneventful course. The final pathology reported Papillary Fibroelastoma. She was followed up in 6 months with a repeat echocardiogram. No residual or recurrence of disease was identified and her tricuspid valve continued to function normally.
DISCUSSIONS:PFE is usually diagnosed in patients over 40 years of age with no preference for gender. PFEs usually arise from the cardiac valves, although they may occur from the endocardial surface. Growda et al. indicated that 84% of 611 cases developed from the cardiac valves; 44% of the cases involved the aortic valve, the mitral valve in 35% of the cases, the tricuspid valve in 15 % of the cases, and the pulmonary valve in 8% of the cases respectively (2). Often patients with PFE are clinically asymptomatic but they can cause potential problems. PFE arising from the tricuspid valve may cause cardiac symptoms such as chest discomfort and exertional dyspnea possibly secondary to tricuspid incompetence, right ventricular inflow partial obstruction, arrhythmia, and pulmonary embolism. Echocardiography findings in PFE include a pedicel or stalk attachment to the endocardium with a highly mobile tumor measuring usually less than 15mm in size. Papillary fibroelastoma cannot be diagnosed on echocardiography as there are no features to distinguish it from other intracardiac tumors. The only way to definitively diagnosis PFE is through surgical excision and histological examination. Surgical excision of PFE is recommended which is curative and can be preformed with a valve sparing technique in a majority of cases. After surgical resection, no recurrence has been reported with an excellent long term postoperative prognosis (2).
CONCLUSION:Echocardiography cannot definitely distinguish PFE from other intracardiac tumors. With the risk of embolic complications and surgery being curative, surgical excision of PFE should be recommended in symptomatic and asymptomatic patients.
DISCLOSURE:Zubair Hashmi, None.