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|Number 18-09: Pulmonary valve papillary fibroelastoma|
number 18-09: Pulmonary valve papillary fibroelastoma
Authors: Aditya Mandawat, MD; Denise Garman, BS, RT; Denise Morell, RT; Stephen Darty, RT; Clara Cheek, RN; Tina D. Tailor, MD
Institute: Duke Cardiovascular Magnetic Resonance Center, Duke University, Durham, NC, USA
A 59-year-old female with suspected dilated cardiomyopathy and no valvular pathology was referred for cardiac MRI. Resting EKG showed normal sinus rhythm, left ventricular hypertrophy, and non-specific T wave abnormalities.
Video 1. Cine of the right-ventricular outflow tract demonstrating a mass attached to the ventricular side of the pulmonary valve.
Videos 2 and 3. Axial and en face cine's at the level of the pulmonary valve demonstrating a mass attached to the right cusp of the pulmonary valve.
Figure 1. Long axis view of the right ventricle outflow tract using DGE imaging with prolonged inversion time (TI = 600ms) demonstrating that the pulmonic valve mass is indistinguishable from the blood pool (arrow).
Figure 2. Dark blood DGE imaging in an axial plane, at the level of the pulmonary valve, demonstrating contrast enhancement in the pulmonic valve mass (arrow).
PFE's represent 70%-80% of heart valve tumors. (3) Characteristics features of PFE's include: (a) attachment to the cardiac valves; (b) round, oval, or irregular shape with well-demarcated margins and homogenous texture; (c) small size (99% are less than 20 mm in largest dimension); and (d) small mobile stalks. (4)
The majority of PFE's arise from the aortic side of the aortic valve. (4,5) While rarely causing valvular dysfunction, aortic PFE's have been associated with dynamic coronary ostia obstruction, leading to myocardial ischemia, as well as transient ischemic attacks and stoke. (5) The mitral and tricuspid valves are the second most common sites of involvement. When located in the atrioventricular valves, the tumor most often occurs on the atrial side of the valve. (4,5) Pulmonary valve PFE's are rare, with fewer than 10 cases reported in the literature. (5)
Considerable debate exists regarding the optimal management of PFE's, particularly in asymptomatic patients. In a recent, large case series from the Mayo Clinic, Tamin and colleagues compared patients receiving cardiac surgery to remove PFE's to those managed expectantly. (6) The valve was preserved in 98% of patients undergoing cardiac surgery, and tumor recurrence occurred in only 1.6% of patients by 1.6 years. Follow-up stroke risk at 5 years was 8% in the post-operative group, versus 13% in patients managed expectantly. In patients managed expectantly, patients taking warfarin, aspirin, or clopidogrel had stroke risks similar to those not taking medication (p=0.39), suggesting that anticoagulation may not be necessary.
1. Weinsaft JW, Kim RJ, Ross M et al. Contrast-enhanced anatomic imaging as compared to contrast-enhanced tissue characterization for detection of left ventricular thrombus. JACC Cardiovascular imaging 2009;2:969-79.
4. Sun JP, Asher CR, Yang XS et al. Clinical and Echocardiographic Characteristics of Papillary Fibroelastomas. A Retrospective and Prospective Study in 162 Patients. Circulation. 2001;103:2687-2693.