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A Large Cardiac Papillary Fibroelastoma Arising
from the Coumadin Ridge: Unusual Location
and Presentation
Bo Xu, MB, BS (Hons), Jorge Betancor, MD, Jonathan Hansen, MD, Carmela D. Tan, MD,
Craig R. Asher, MD, and L. Leonardo Rodriguez, MD, FACC, FASE,
Cleveland, Ohio and Weston, Florida
INTRODUCTION
We report the case of a 59-year-old woman presenting with ST-
segment elevation myocardial infarction, resulting in cardiogenic
shock. Because of advanced cardiomyopathy, she subsequently un-
derwent left ventricular assist device implantation. During her diag-
nostic workup, a large, mobile, left atrial (LA) mass was diagnosed
by transthoracic echocardiography (TTE) and transesophageal echo-
cardiography (TEE), and pathologic examination of the excised
mass confirmed the diagnosis of a cardiac papillary fibroelastoma
(PFE). This case is unique in that the tumor mass was very large and
arose from an unusual location: the coumadin ridge. It is also an
illustrative example of the echocardiographic assessment of cardiac
PFE.
CASE PRESENTATION
A 59-year-old woman presented with left-sided chest pain and ante-
rolateral ST-segment elevation on electrocardiography. Her medical
history was notable for type 2 diabetes mellitus and hypertension.
Coronary angiography demonstrated thrombotic occlusion of the
proximal left anterior descending and proximal left circumflex coro-
nary arteries. The angiographic appearances were consistent with an
acute thromboembolic phenomenon. The patient was managed
with thrombus aspiration and balloon angioplasty. A detailed
workup for a hypercoagulable state (including lupus anticoagulant,
b
2
-glycoprotein, anticardiolipin antibody, lipoprotein[a], and
serum homocysteine level) was unremarkable. The hospital
course was complicated by heparin-induced thrombocytopenia
Figure 1 Serial TTE (3 months apart). (A) Apical five-chamber view demonstrates the absence of a cardiac mass within the left atrium
(asterisk). (B) Three months later, a similar apical five-chamber view demonstrates the presence of a moderate-sized cardiac mass
within the LA chamber (asterisk).
From the Department of Cardiovascular Medicine (B.X., J.B., J.H., L.L.R.) and
Department of Anatomic Pathology (C.D.T.), Heart and Vascular Institute,
Cleveland Clinic, Cleveland, Ohio; and the Department of Cardiovascular
Medicine, Heart and Vascular Institute, Cleveland Clinic Florida, Weston, Florida
(C.R.A.).
Keywords: Cardiac papillary fibroelastoma, Coumadin ridge, Transesophageal
echocardiography, Transthoracic echocardiography, Multimodality cardiovascu-
lar imaging
Conflicts of interest: The authors reported no actual or potential conflicts of interest
relative to this document.
Copyright 2017 by the American Society of Echocardiography. Published by
Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://
creativecommons.org/licenses/by-nc-nd/4.0/)
2468-6441
http://dx.doi.org/10.1016/j.case.2017.05.006
1
(positive platelet factor 4 and serotonin release assays), and the pa-
tient was discharged on standard medical therapies, in addition to
anticoagulation with warfarin for presumed thromboembolic ST-
segment elevation myocardial infarction. Of note, the patient did
not have a history of deep vein thrombosis, and there was no family
history of thrombophilia. The patient never smoked or used oral
contraception. As a result of the myocardial infarction, there was sig-
nificant myocardial dysfunction, with a severely depressed left ven-
tricular ejection fraction (20%), decreased from a previously normal
baseline.
Three months following hospital discharge from the ST-segment
elevation myocardial infarction presentation, the patient re-presented
with worsening dyspnea on exertion, fatigue, and lower extremity
edema. On clinical assessment, she was hypotensive. A positron emis-
sion tomographic viability study showed a large region of viable hiber-
nating myocardium in the left anterior descending coronary artery
territory, without scar. Repeat coronary angiography interestingly
demonstrated absence of significant obstructive coronary artery dis-
ease in all three epicardial vessels. TTE confirmed persistently
depressed left ventricular systolic function (left ventricular ejection
fraction 18%), withakinesis of the apex and extension of severe hypo-
kinesis into the midanterior, midseptal, and midinferior walls. Notably,
a new, large mobile echogenic mass was visualized, which appeared to
be attached to the lateral wall of the left atrium, likely emanating from
the LA appendage (Figure 1B, Video 1). This echogenic mass was not
seen when TTE was performed 3 months earlier (Figure 1A, Video 2).
Figure 2 TEE. (A) Midesophageal four-chamber view at 0with biplane orthogonal 90view (B) focusing on a 2.3 2.0–cm, globular,
heterogeneous, pedunculated mass (arrow), which appears attached to the limbus of the LA appendage, also known as the coumadin
ridge.
Figure 3 TEE. (A,B) Midesophageal view at 0showing the PFE (arrow) within the left atrium attached to the tip of the limbus of the LA
appendage, also known as the coumadin ridge, and demonstrating the spatial relationship between the PFE (arrow), the limbus
(asterisk), and the left upper pulmonary vein (LUPV).
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The echocardiographic appearance and location of the mass were sus-
picious for a LA appendage thrombus.
Further characterization of the mass by TEE occurred (Figure 2,
Video 3). This demonstrated a large heterogeneous mass (measuring
$2.3 2.0 cm) attached to the coumadin ridge (Figure 3,Videos 4
and 5). On detailed assessment by three-dimensional TEE, tiny fronds
were visualized on the surface of this echogenic mass (Figure 4,
Videos 6 and 7). The mass did not arise from the LA appendage,
and no LA thrombus was visualized. On the basis of these imaging
findings, the differential diagnoses considered for the mass included
a PFE, an atypical atrial myxoma, or an atypical thrombus, not arising
from the LA appendage.
The patient underwent surgical excision of the mass and subse-
quently underwent implantation of a HeartMate II left ventricular
assist device (Thoratec, Pleasanton, CA), given her advanced cardio-
myopathy and heart failure (Figure 5,Video 8). Pathologic examina-
tion of the mass confirmed a PFE (Figure 6), consistent with findings
on TEE. The patient was discharged on standard heart failure thera-
pies to a skilled nursing facility for ongoing rehabilitation. She remains
clinically stable.
Figure 4 Three-dimensional TEE demonstrating the location of the PFE (arrow) as it arises from the coumadin ridge (A,B), with the
aortic valve at the top of the image (not visualized) and the LA appendage to the left of the coumadin ridge and the PFE. Note in a
more distant plane the anterior leaflet of the mitral valve in (C) and the mitral annulus in (D).
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DISCUSSION
Primary cardiac tumors are uncommon, and most are benign in na-
ture.
1
Cardiac myxomas were traditionally thought to be the most
common type of primary cardiac tumor, on the basis of surgical series.
However, a recent large, single-center series suggested that cardiac
PFEs may be more common than cardiac myxomas, with a rate of
one PFE per 1,100 echocardiograms from the reporting center’s
referral base.
2
The differential diagnosis of a PFE is wide and includes
thrombus, myxoma, fibroma, cardiac metastases, vegetation, and
degenerative or calcific change.
PFEs are benign primary cardiac tumors consisting of small multi-
ple papillary protrusions attached to the endocardium by a short
stalk with a ‘‘sea anemone’’ appearance.
3
Although often discovered
incidentally, the clinical presentation of PFEs varies widely, and they
can present with severe embolic or ischemic events, such as a
myocardial infarction.
4
The probability of a PFE resulting in an
embolic event likely varies, according to the mobility, friability,
size, and location of the tumor. In addition to cerebral and cardiac
emboli, PFEs have been reported to cause mesenteric, renal, and
limb embolism.
5,6
As a result, surgical resection is generally recom-
mended, especially for those with a high risk for embolization, usu-
ally when the size of the tumor is >1 cm and associated with a
stalk.
7
Surgical excision of PFEs is generally associated with an excel-
lent long-term prognosis.
8
Approximately 84% of PFEs arise from the valvular endocardium,
favoring the aortic valve in most cases.
9
However, involvement of the
pulmonary valve, and even simultaneous involvement of both aortic
and pulmonary valves, has been reported.
10
Nonvalvular PFEs repre-
sent 15% of all cases reported
8
and can arise from the endocardium of
both the left and right ventricles (septum and papillary muscles), atria
(septum, appendages, Eustachian valve, and Chiari network), and the
intimal surface of coronary artery ostia. Most nonvalvular PFEs origi-
nate in the left ventricle, with an incidence of approximately 9%, fa-
voring the interventricular septum.
11
The presence of PFEs in the
left atrium is very rare, and its occurrence has been reported to be
approximately 1.6%–2%.
8,12
LA PFEs have been previously found
to be attached to the free wall,
8,13
interatrial septum, and LA
appendage,
8
but to the best of our knowledge, they have not been
previously reported to arise from the limbus of the LA appendage,
also known as the coumadin ridge. Our case is unique in terms of
the unusual location of the PFE.
Figure 5 Three-dimensional TEE demonstrating the inflow can-
nula position (arrow) after placement of a HeartMate II left ven-
tricular assist device.
Figure 6 Gross and microphotograph of the excised mass. The gross specimen (left) was received fragmented, with the largest piece
measuring 3.2 cm, showing pieces of branching papillary tumor with bulbous gelatinous fronds. On light microscopy (top right,
Hematoxylin and Eosin; bottom right, Movat pentachrome, 400magnification), the fronds showed a central core of fibrous tissue
(yellow in the Movat stain). Each frond consisted of a mucopolysaccharide-rich stroma (green) lined by endothelial cells. The fibrous
cores of some of the papillae contained elastic fibers (black), as demonstrated in the Movat stain.
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Multimodality Cardiovascular Imaging
Before the widespread application of multimodality imaging, PFEs were
often incidentally detected during autopsy or cardiac surgery.
3
Multimodality cardiovascular imaging, including echocardiography
(TTE, TEE, and three-dimensional echocardiography), cardiac magnetic
resonance (CMR), and multidetector computed tomography, has an
important role in the diagnosis of PFEs. On echocardiography, PFEs
appear as mobile, pedunculated masses with a heterogeneous, speckled
appearance. Classically, tiny fronds may be visualized at the surface of
the tumors. Three-dimensional echocardiography may provide incre-
mental value in the characterization of PFEs. On multidetector
computed tomography, PFEs are generally well-delineated lesions with
soft tissue attenuation. CMR can further aid in the tissue characterization
of cardiac masses and tumors. On CMR, PFEs are generally homoge-
neous masses, with an intermediate signal on T1-weighted sequences
and a high signal on T2-weighted sequences, which exhibit a lack of sup-
pression with fat saturation and strong delayed enhancement.
14
The dif-
ferential diagnoses of PFEs can also be effectively assessed by
multimodality cardiovascular imaging, with echocardiography being
the mainstay initial imaging tool. On echocardiography, cardiac myx-
omas typically are visualized as mobile, pedunculated masses arising
from the endocardial surface. Classically, cardiac myxomas arise from
the region of the fossa ovalis. On CMR, atrial myxomas have a hetero-
geneous appearance, with intermediate signal intensity on T1-weighted
sequences and higher signal intensity on T2-weighted sequences.
14
An
intracardiac thrombus typically arises from the LA appendage itself,
rather than the coumadin ridge, and contrast study may aid in character-
izing an intracardiac thrombus by demonstrating lack of contrast uptake.
This finding may be further confirmed by delayed enhancement imaging
on CMR. An intracardiac thrombus appears as a homogeneously low–
signal intensity mass on long–inversion time delayed-enhancement im-
aging, because of lack of contrast uptake, surrounded by high-signal
blood pool.
15
CMR is powerful in its ability to provide detailed tissue
characterization of intracardiac masses and can help differentiate PFEs
from other intracardiac tumors. Lipomas generally occur as sessile ho-
mogeneous masses in the atrium (interatrial septum or atrial wall), which
are hyperintense on T1-weighted turbo spin-echo sequences and hypo-
intense on fat-suppressed sequences, as opposed to PFEs, which do not
show any signal dropout on fat-suppressed sequences.
14
Fibromas are
generally solitary, well-defined intramyocardial masses, which have a
smooth morphology, associated with calcification and a broad base.
They are inhomogeneous and isointense or slightly hyperintense on
T1-weighted sequences and hypointense on T2-weighted sequences,
demonstrate no change with fat saturation, and are associated with
intense delayed enhancement.
14
Malignant cardiac tumors, including
cardiac metastases, may have evidence of tissue invasion morphologi-
cally and, for cardiac metastases, may often be associated with a pericar-
dial effusion.
14
Different types of malignant cardiac tumors exhibit
varying CMR imaging characteristics, and a detailed discussion is beyond
the scope of this article.
CONCLUSION
We describe the comprehensive echocardiographic assessment of a
large PFE attached to the coumadin ridge in a 59-year-old woman.
TTE provided the initial diagnosis of the large mass in the left atrium,
while detailed TEE demonstrated features more suggestive of a PFE
than a thrombus. The findings on TEE were confirmed by pathologic
examination of the excised mass. This case is unique in that the PFE
reported was very large in size and arose from an unusual location,
the coumadin ridge. Additionally, this case serves as an illustrative
example of the imaging diagnostic pathways involved in the assess-
ment of an intracardiac mass.
SUPPLEMENTARY DATA
Supplementary data related to this article can be found at http://dx.
doi.org/10.1016/j.case.2017.05.006.
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