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a 61-year - old hispanic woman presented with recurrent paroxysmal pleuritic chest pain and severe dyspnea . between episodes , the patient was normally functional and asymptomatic .
there was no prior history of structural cardiac , coronary , pulmonary , or systemic disease .
transthoracic and transesophageal echocardiography showed a mobile mass of moderate size and shimmering edges , measuring 1.3  0.9  1.1 cm , attached to the septal tricuspid valve ( tv ) leaflet .
a pedunculated mass with peripheral shimmering frond - like appearance and stalk attached to the septal tv leaflet was seen prolapsing through the tv with tricuspid regurgitation ( fig .
ct pulmonary angiography did not show gross filling defects to suggest pulmonary embolism ( pe ) but revealed a mobile mass visible in the orthogonal image planes ( fig .
cardiac mri revealed a mobile mass measuring 1 cm in multiple dimensions , showing hyperintensity on t2-weighted and inversion recovery sequences ( fig .
d ) . a differential diagnosis of papillary fibroelastoma ( pf ) versus cardiac myxoma was entertained .
cardiac surgery was advised due to size ( > 1 cm ) , symptomatology , and the established recurrent embolic potential of the tumor . at surgery ,
shave excision of the sessile mass , and tv repair using a 30-mm medtronic contour ring was performed .
gross pathology revealed a friable mass with surface fronds on a short pedicle , with the typical sea anemone appearance of a pf .
each frond had a fibrous core with loose connective tissue rich in mucopolysaccharide ( fig .
postoperatively , the patient continued anticoagulation for 8 weeks . to date , the patient remains asymptomatic at rest and exercise .
pf accounts for 10% of all primary cardiac tumors . of these , right - sided valvular ( tricuspid and pulmonary )
pf are relatively rare presentations of an increasingly recognized benign ( non - metastatic ) though clinically important entity .
histopathologically , it is considered a hamartomatous entity with endothelial genesis secondary to turbulent high - stress flow and secondary endothelial dysfunction with proliferation .
the majority ( 75% ) of fibroelastomas arise from the valvular endothelium , and are left - sided in origin .
right - sided pfs are an important potentially fatal cause of sudden cardiac death , syncope , chest pain , and dyspnea . while pfs are slow growing , their propensity to accumulate superficial thrombus and fragment
, embolizing tumor or thrombus fragments into the distal pulmonary circulation or paradoxically embolizing to the left - sided circulation , is significant [ 2 , 3 ] .
clinical recognition of these tumors is therefore important [ 4 , 5 , 6 ] .
while echocardiographic imaging remains the most prevalent gold standard in use clinically , ct chest and cardiac magnetic resonance ( cmr ) are both increasingly used .
imaging right - sided pfs is important due to the delayed presentation of right - sided versus left - sided tumors . while high - resolution or thin - section multislice ct of the chest offers excellent spatial and temporal resolution ,
we emphasize the incremental value of cmr in pf recognition because it affords versatility of multiplane reconstruction and pathological discrimination based on soft - tissue contrast resolution of water and fat content using t1-/t2-weighted imaging .
steady - state free precession sequences revealed a hyperintense highly mobile pedunculated mass attached to the septal tv ( fig .
in contrast , the thrombus is hypointense on t1- or t2-imaging , while myxomas have isointense t1- and high t2-signal intensity due to their high extracellular water content .
fiesta ( bright blood imaging ) mri sequences of pf show a sharply delineated pedunculated mobile mass with : ( 1 ) hyperintense signal relative to the myocardium but hypointensity relative to the blood pool ( fig .
3a ) and ( 2 ) hyperintense signal by inversion recovery t2 sequences and delayed gadolinium hyperenhancement ( fig .
even the non - high resolution ct pulmonary angiogram clearly demonstrated the tv - pf ( fig .
2 ) . fiesta ( fast imaging employing steady - state acquisition ) is an ultrafast mri technique based on balanced steady - state free precession that offers high - signal - to - noise ratio and excellent image contrast .
inversion recovery sequences , offer differentiation of tumor from fat or muscle by the additive signal intensity effects of prolonged t1 and t2 , and nulling fat signal ( fig .
3d ) is seen in 4050% of benign tumors and varies with capillary permeability or tumor vascularity .
less well appreciated is the morbidity of right - sided pfs . their slow growth pattern and more insidious and life - threatening presentations are noteworthy .
surgical excision appears safe , potentially curative ( however , good long - term follow - up data for recurrence is lacking ) , and is advised where symptoms or large pf tumor size or significant tumor mobility is present [ 4 , 5 , 6 , 7 ] .
there are documented cases of tv - pf with pe [ 2 , 3 ] .
pleuritic chest pain and dyspnea symptoms are common , and may reflect recurrent distal pulmonary circulation embolization , despite overtly negative ct and v / q scan findings for proximal pe . in the largest surgical series of 88 patients ( 93 pf lesions ) right - sided pfs accounted for 10% of all valvular ( tv , pv ) lesions and 16% of all cardiac chamber ( ra , rv ) lesions . mean age at presentation was 60  14 years , 83% required only a shave excision , with 9% requiring shave excision and valve repair . interestingly , right - sided tumor burden was greater than left - sided , presumably reflecting the more indolent growth and presentation of these lesions .
later , this patient developed a new left - sided aortic valve pf 3 months after successful right - sided tumor removal . in a meta - analysis of 725 cases of cardiac pf ( cpf ) in 2003 , gowda et al .  
identified tumor mobility as the independent predictor of pf - related death or non - fatal embolization .
they suggested an 11% rate of right - sided valvular pfs , emphasizing the possibility of multiple tumor locations within a single patient .
most cpf - related death occurred from obstruction of the aortic valve or coronary embolization of left - sided tumors .
however , one death occurred from rv outflow obstruction , and two non - fatal embolizations of the pulmonary circulation resulted .
of these , 12 died of pf - related causes including coronary embolization or obstruction ( 11 cases ) , 4 developed non - fatal distal embolization ( 2 coronary , 1 cerebral 1 pulmonary ) despite anticoagulation . for these reasons ,
many advocate early surgical intervention for symptomatic ( obstruction / valvular dysfunction ) or asymptomatic cases , and bulky ( > 1 cm ) , pedunculated highly mobile tumors whether left- or right - sided , though mortality is clearly higher with left - sided cpfs [ 4 , 5 , 6 ] .
presentation of an increasingly recognized right - sided primary valve tumor of clinical importance : the tricuspid valve papillary fibroelastoma ( pf ) . early recognition and surgical intervention 
 is emphasized for valvular pf , which carries a significant risk of morbidity and mortality . 
 newer imaging techniques , including ct and mri , assist in localizing and differentiating pf from alternative cardiac pathology .