A 73-year-old male with a prior medical history notable for hypertension, type 2 diabetes mellitus, dyslipidemia, and coronary artery disease presents for a consultative opinion regarding a pulmonic valve mass identified on the referring facility's transthoracic echocardiogram (Video 1). He denies any recent angina symptoms, significant dyspnea, orthopnea, fevers, or chills. He is still able to lead a very active lifestyle. Prior testing includes both an echocardiogram and nuclear perfusion study. He has no history of malignancy and is up-to-date on cancer screening exams. His physical examination is unremarkable. He has a normal complete blood cell count and basic chemistry profile. His electrocardiogram is notable for sinus bradycardia but is otherwise unremarkable. Review of the outside echocardiogram reveals that the mass measures approximately 6-8 mm and is on the outflow tract side of the valve. There is mild pulmonic regurgitation.
Video 1
Which of the following describes the most likely diagnosis?
Show Answer
The correct answer is: C. Papillary fibroelastoma.
Given the benign presentation without any systemic symptoms, the most likely diagnosis is a benign papillary fibroelastoma. Given his age and lack of symptoms, his physicians felt that further testing was not necessary and surgical intervention not warranted. The patient is seen the following year and continues to deny symptoms. Repeat transthoracic echocardiogram (Videos 2a and b) demonstrates that the mass is unchanged from the prior study. The differential diagnosis for a cardiac mass includes fibroelastoma, myxoma, infectious endocarditis, thrombus, or nonbacterial thrombotic endocarditis (NBTE). Fibroelastomas are the third most common benign tumor following myxomas and lipomas. A myxoma is a benign polypoid neoplasm that is usually attached to the interatrial septum. If this mass was of carcinoid etiology, then one would expect tricuspid valve involvement and systemic symptoms, in addition to which the pulmonic valve itself would be expected to be thickened and dysfunctional. If this mass were a thrombus, the morphology would be expected to be more circumscribed. The patient fulfills only one major and no minor criteria for endocarditis. This, combined with the fact that he had no risk factors (in-dwelling catheters, IV drug use, immunosuppression, chronic hemodialysis, etc.) for endocarditis, made the physicians' clinical suspicion for endocarditis very low. Lack of systemic symptoms and no history of malignancy makes NBTE very unlikely. Possible management options for the pulmonary valve mass include resection, symptomatic treatment, or surveillance. A comprehensive review of the current literature recommends that asymptomatic patients with non-mobile fibroelastomas be followed clinically with periodic echocardiography. Symptoms can result from either embolization or valvular dysfunction. Symptomatic patients should be considered for surgical resection.
Video 2a
Video 2b
References
Okada K, Sueda T, Orihashi K, et al. Cardiac papillary fibroelastoma on the pulmonary valve: a rare cardiac tumor. Ann Thorac Surg 2001;71:1677-9.
Saad RS, Galvis CO, Bshara W, et al. Pulmonary valve papillary fibroelastoma. A case report and review of the literature. Arch Pathol Lab Med 2001;125:933-4.
Truscelli G and Gaudio C. Papillary fibroelastoma of the pulmonary valve–a systematic review: advantages of live/real time three-dimensional transthoracic and transesophageal echocardiography. Echocardiography 2014;31:795-6.
Gowda RM, Khan IA, Nair CK, et al. Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases. Am Heart J 2003;146:404-10.
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