Journal of Cardiology Cases 10 (2014) 125–128

Case Report

Papillary fibroelastoma of the mitral valve chordae with systemicembolization

Pallavi S. Gomadam (MD), Richard B. Stacey (MD), Andrew E. Johnsen (MD),Dalane W. Kitzman (MD), Neal D. Kon (MD), Bharathi Upadhya (MD)*

Wake Forest Baptist Medical Center, Winston Salem, NC, USA

A R T I C L E I N F O

Article history:

Received 9 April 2014

Received in revised form 3 June 2014

Accepted 11 June 2014

Keywords:

Fibroelastoma

Stroke

Embolus

Cardiac tumor

Cardiac magnetic resonance imaging

A B S T R A C T

Papillary fibroelastoma (PFE) is a rare benign tumor that most commonly involves heart valves. Cardiac

PFE involving the mitral chorda is rarer. Most papillary PFE are asymptomatic; rarely, they are diagnosed

because of cardiac symptoms or after an embolic event. In this case, a 70-year-old woman presented

with an acute cerebral ischemic infarct. Evaluation for potential embolic source revealed a mass on the

mitral chordae. The findings of cardiac magnetic resonance (CMR) tissue characterization of the mass

corroborated the diagnosis of mitral valve tumor. She underwent surgical resection of this mass and

histology, which confirmed a PFE.

<Learning objective: PFE are easily detected on echocardiography but are difficult to visualize with

CMR. Their small size and rapid, extensive movement render adequate CMR imaging extremely difficult.

This case highlights the ability of CMR to characterize the tissue characteristics and enhancement

pattern which enables non-invasive assessment for the etiology of intra-cardiac masses and usually

distinguishes a tumor from thrombus.>

� 2014 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

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Journal of Cardiology Cases

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Introduction

Papillary fibroelastomas (PFEs) are rare endocardial tumors,predominantly affecting the cardiac valves. In particular, theymore commonly affect the aortic valve followed by the mitral valve[1]. A non-valvular origin was observed in approximately 16% ofcases [2]. Cardiac PFE involving the mitral chorda is rarer.Transthoracic and especially transesophageal echography (TTE;TEE) is the most sensitive and reliable investigation to establish thediagnosis, exclude multiple locations, and plan the optimalsurgical approach [2]. Whether a multimodality imaging approachoffers significant added value compared with echocardiographyalone in the diagnostic evaluation of cardiac PFE is controversial.

Case report

A 70-year-old Caucasian woman presented with diffuseweakness and expressive aphasia. Her medical history includeda previous left-sided cerebral infarct with no residual deficits.

* Corresponding author at: Wake Forest Baptist Medical Center, Cardiology

Section/Internal Medicine, Medical Center Boulevard, Winston-Salem, NC 27157,

USA. Tel.: +1 336 716 9075; fax: +1 336 716 9188.

E-mail address: bupadhya@wakehealth.edu (B. Upadhya).

http://dx.doi.org/10.1016/j.jccase.2014.06.004

1878-5409/� 2014 Japanese College of Cardiology. Published by Elsevier Ltd. All rights

She underwent a head computed tomography (CT) and brainmagnetic resonance imaging (MRI), which revealed an acuteischemic infarct of the left hypothalamus, insular cortex, posteriorleft frontal lobe, and left caudate nucleus. Given the concern forpotential embolism, a two-dimensional TTE was obtained, whichrevealed a 13 mm � 12 mm mass in relation to the subvalvularaspect of the posterior mitral valve apparatus (Fig. 1). To examinethe characteristics of mass in detail, a TEE was performed, whichshowed a 14 mm � 12 mm mass on the chordae attached to theposterior mitral leaflet and a 2 mm � 3 mm echo density attachedto left coronary cusp of aortic valve (Fig. 1). To further characterizethis mass, imaging was obtained by cardiac magnetic resonance(CMR). It revealed a 7 mm � 8 mm round mass associated withchordae immediately below the P2 scallop of posterior mitralleaflet. This mass showed homogenous signal intensity identical tofibrous tissue on both T1 and T2 weighted sequences; fatsuppression ruled out the presence of fatty lesion components.It did not enhance with first-pass perfusion. T1-weightedinversion-recovery images after intravenous administration ofgadolinium diethylene triamine pentaacetate show enhancementof tumor caused by the fibroelastic tissue of the mass (Fig. 2).Absence of increased signal intensity on contrast-enhanced first-pass perfusion imaging and contrast uptake of the tumor ondelayed-enhancement imaging clearly demonstrated that thismass is neither a malignant tumor, nor a thrombus. Cardiac

reserved.

Fig. 1.(A and B) 2D transthoracic echocardiography showing a mass attached to subvalvular apparatus of posterior mitral leaflet; (C) Transesophageal echocardiography

(TEE) showing a mass attached to subvalvular posterior mitral valve apparatus; (D) TEE showing the echo density attached to the left coronary cusp of aortic

valve.

P.S. Gomadam et al. / Journal of Cardiology Cases 10 (2014) 125–128126

surgery was performed, which revealed a 1 cm � 1 cm friable,gelatinous, tumor. It was associated with the posterior papillarymuscle under the P2 scallop. This was excised along with onetendon which was unable to be separated from the tumor (Fig. 3).Likewise, a 2 mm mass, similar in appearance to the mitral valvetumor was noted on the left coronary cusp and was also excised.The histopathological examination confirmed the diagnosis of aPFE (Fig. 3). The patient made an uncomplicated recovery.

Discussion

Although the prevalence of primary cardiac tumors ranges from0.0017% to 0.28%, the PFEs are the second most common benignneoplasm of the cardiac valves after myxomas [2]. It can be asource of embolization leading to strokes, particularly in thosewithout identifiable risk factors [1]. Kasarskis et al. [3] reportedthat 50% of the patients with a PFE had transient ischemic attacksor previous strokes prior to discovery of the tumor. Embolizationmay occur from the fragile papillary fronds or from a platelet andfibrin aggregation forming on the tumor [4,5].

PFEs are easily detected on echocardiography, but are difficultto visualize with CMR. Their small size and rapid, extensivemovement render adequate CMR imaging extremely difficult. Inthe present case, the high diagnostic image quality of spin-echo

sequences was achieved by freezing of mitral valve motion.The mitral valve rest period was determined from a cine sequencewith a high temporal resolution. Subsequently, spin-echo dataacquisition was restricted to the rest period duration, therebyachieving an almost complete motion freezing of the valve andits lesion. Overall, CMR is the most important modality indifferentiating tumor from thrombus and distinguishing benignfrom malignant cardiac masses [6]. CMR can demonstrate keyindicators of malignancy including invasive behavior, tissueinhomogeneity, and higher tissue vascularity. Nearly all malignanttumors show post gadolinium enhancement and hyperperfusioncharacter [7]. Thrombus appears as a low signal intensity filingdefect in the cardiac chamber. Contrast-enhanced CMR allowsdifferentiation between thrombus and surrounding myocardiumbecause thrombus is avascular and hence is characterized by anabsence of contrast uptake [8]. Late gadolinium enhancementstudies are most useful in thrombus detection as compared to thecine studies [9]. Presence of increased signal intensity ondelayed enhancement clearly differentiates PFE from thrombus.This will also help to delineate the thrombus if it is attached toPFE. This case highlights the ability of CMR to characterize thetissue characteristics, which enables non-invasive assessment forthe etiology of intra-cardiac masses. However, this case revealstwo important limitations of CMR: In the present case, tumor size

Fig. 2.

(A and B) Three-chamber and short-axis view showing a mass, attached to subvalvular posterior mitral valve apparatus (arrow) in a steady-state free precession

cine magnetic resonance image; (C and D) T1-weighted and T2-weighted turbo spin-echo sequences of the mass; (E) First-pass perfusion showing mass with no

enhancement; (F) T1-weighted inversion-recovery images after intravenous administration of gadolinium diethylene triamine pentaacetate show enhancement

of mass.

Fig. 3.(A) Intraoperative photograph shows a mass adhering to the chordae attached to posterior mitral leaflet; (B) Biopsy revealed elongated and branching papillary

fronds with variable amounts of avascular collagen and elastic tissue; (C) Higher power photograph of hypocellular papillary fronds with avascular collagen core

surrounded by acid mucopolysaccharides and lined by hyperplastic endothelium.

P.S. Gomadam et al. / Journal of Cardiology Cases 10 (2014) 125–128 127

P.S. Gomadam et al. / Journal of Cardiology Cases 10 (2014) 125–128128

was underestimated by CMR. This is probably related to thelimitation in choosing the correct plane with the largestdimension. We were unable to detect the presence of PFE onaortic valve. Even with adequate CMR imaging, very small, mobilemasses can be missed on spin-echo sequences that are needed fortexture characterization.

Despite the benign nature of this tumor, it carries asubstantial risk for embolic complications, particularly strokes.Once diagnosed, prompt surgical management is indicated evenin the asymptomatic patient [2,10]. The surgical resection iscurative, safe, and well tolerated [2,10]. Re-growth of the tumorafter resection has not been reported, but it requires long-termTTE follow-up studies to monitor for recurrence.

Conclusions

This report highlights the rare chordal location of a PFEwithout valvular involvement. A dedicated CMR protocolfor comprehensive tissue characterization is indispensable inidentifying soft tissue masses. Prompt surgical resectionwith preservation of valvular integrity is recommended in mostcases.

Contribution

All authors had access to the data and played a role in writingthe manuscript.

Funding sources

None.

Conflict of interest

The authors declare no conflict of interest.

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