Rare disease

CASE REPORT

Aortic valve fibroelastoma: a rare cause of stroke
Vivek Kumar, Parita Soni, Arsalan Hashmi, Manfred Moskovits
Department of Internal
Medicine, Maimonides Medical
Center, Brooklyn, New York,
USA
Correspondence to
Dr Parita Soni,
sonipss14@gmail.com
Accepted 9 November 2016

SUMMARY
The prevalence of primary cardiac tumours varies from
0.02% to 0.45%. Cardiac papillary fibroelastoma (CPF)
is a rare tumour diagnosed incidentally on imaging. The
clinical manifestations result from thromboembolisation
and include transient ischaemic attack, stroke and
sudden cardiac death. We present a patient aged
57 years with CPF arising from the aortic valve. The
patient presented with right hemiparesis due to acute
stroke. He received tissue plasminogen activator with
complete resolution of neurological symptoms.
Echocardiography revealed a broad-based, gelatinous,
non-mobile mass on the left aortic cusp. The tumour
was excised sparing the aortic valve. The patient
recovered rapidly without any complications. The
histopathological examination confirmed the diagnosis of
CPF. A review of the literature suggests that CPF is a
rare but treatable cause of stroke. The course is not clear
and there are no tumours or patient-related
characteristics which could predict the risk of
thromboembolisation. Surgical treatment is definite and
is relatively safe.

BACKGROUND
Primary cardiac tumours are rare with a prevalence
of 0.02% in an autopsy and 0.45% in an open
heart surgery study.1 Cardiac papillary fibroelastoma (CPF) is the most prevalent valvular and
overall second most common cardiac tumour after
myxoma.2 They affect the aortic valve more often
and are recognised more frequently now due to the
availability of higher resolution imaging techniques.3 CPFs are histologically benign and frequently asymptomatic. Only occasionally, they
manifest with symptoms of transient ischaemic
attacks, stroke, heart failure, myocardial infarction,
cardiac arrest and sudden cardiac death.4 Herein,
we present a rare case of a patient with fibroelastoma of the aortic valve presenting with stroke.

CASE PRESENTATION

To cite: Kumar V, Soni P,
Hashmi A, et al. BMJ Case
Rep Published online:
[please include Day Month
Year] doi:10.1136/bcr-2016217631

An Asian man aged 57 years with a medical history
of hypertension (on losartan), presented to the
emergency room with sudden onset of weakness in
his right upper and lower extremities for 2.5 hours
duration. He denied any facial droop, visual disturbances or slurred speech. On presentation, his vital
signs were stable with blood pressure of 146/
87 mm Hg. Physical examination revealed weakness
in right upper (3/5 power) and lower extremities
( power 4/5). Sensory and cranial nerve examination
was unremarkable. Examination of the left side was
normal. Cardiac examination revealed normal heart
sounds without any murmurs. Laboratory values,
including complete blood count, HBA1C, kidney,

liver and lipid panels, were inconspicuous. ECG
and chest X-ray were also normal. Non-contrast
CT of the head and CT angiogram (CTA) head
were negative for any acute intracranial pathology.
The patient was eligible for treatment with tissue
plasminogen activator and was transferred to the
intensive care unit. His symptoms improved significantly post-therapy. A follow-up non-contrast MRI
was obtained on the next day which did not reveal
any area of restricted diffusion.
Duplex examination was negative for any significant stenosis or thrombosis of carotid and vertebral
arteries. Transthoracic echocardiogram was performed as part of stroke work-up, which revealed a
mass on the aortic valve suspicious for vegetation.
For better evaluation, transoesophageal echocardiogram (TEE) was performed which showed a nonmobile mass on the aortic valve measuring
9×6 mm, gelatinous in consistency with broadbased attachment to the edge of the left coronary
cusp (video 1 and figure 1). Cardiothoracic surgery
team was consulted and the patient underwent
excision of the tumour with sparing of the aortic
valve (figure 2). Histopathological assessment of
the resected specimen confirmed the diagnosis of
fibroelastoma (figure 3).
Postoperative TEE showed no residual tumour
and confirmed the integrity of the valve with only
trace regurgitation. The patient recovered rapidly
without any complications with absolutely normal
neurological examination.

DISCUSSION
Primary tumours of the heart are rare, they are
usually benign and they occur mostly in adults.
Papillary fibroelastoma accounts for <10% of all
cardiac tumours, but they are most common valvular tumours.5 Previous studies have shown that
more than two-thirds of papillary fibroelastomas
are found incidentally preoperatively or intraoperatively. They are solitary tumours mostly <20 mm.2
Most papillary fibroelastomas are located in the left
heart, which increases the risk of systemic embolism.6 The natural history and course of this tumour
is not known.7 The symptoms are mostly due to
thromboembolisation originating from the tumour
itself or from thrombus formation on the tumour
surface.8 The definite treatment is valve-sparing
excision of tumour with low recurrence rate.9
Surgical resection is indicated in all symptomatic
patients and should be offered to incidentally diagnosed patients during evaluation of cardiac surgery
for other reasons.2 In other patients, the treatment
is debatable for incidentally diagnosed tumours and
depends on their size, location, mobility and associated comorbidities. In such patients, surgery is

Kumar V, et al. BMJ Case Rep 2016. doi:10.1136/bcr-2016-217631

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Rare disease

Video 1 Video clip of the transoesophageal echocardiogram showing
the tumour attached to the left aortic valve cusp in the parasternal
long-axis view (left side) and the parasternal short-axis view (right
side).

Figure 1 Long-axis view of the transoesophageal echocardiogram
image showing mass on the left aortic value cusp.

Figure 3 Histological examination under high-power field displaying
a mass composed of multiple fronds of acellular collagen and dense
elastin covered by flat endothelial cells highly characteristic of papillary
fibroelastoma.

reasonable for pedunculated lesions or tumours larger than
1 cm in diameter, mobile lesions on echocardiography or if
located adjacent to coronary ostia.10
Medical management with oral anticoagulation has been
advocated for smaller, non-mobile tumours and in cases where
valve-sparing surgery is not possible. Of note, this approach was
associated with 50% tumour-related mortality in one study and
has not been universally accepted.11
Despite being histologically benign, CPF can result in fatal
complications such as stroke, acute valvular dysfunction, embolism, ventricular fibrillation and sudden death.4 However, cerebrovascular complications have been associated with tumours of
diameter as small as 3 mm.12 Of note, our patient developed
stroke, despite the tumour appearing non-mobile on TEE.
Previous reports also suggest that echocardiographic appearance
may not predict the risk of thromboembolisation.2 The excision
of papillary fibroelastoma is a relatively safe procedure and is
indicated in all symptomatic patients.2 Asymptomatic patients
should also be carefully assessed for surgery to prevent catastrophic events in future.

Learning points

Figure 2 The gross specimen of resected tumour appearing like
gelatinous nodule which is different from typical appearance
resembling a sea anemone described in the literature.

2

▸ Cardiac papillary fibroelastoma most commonly affects the
aortic valve and are an uncommon cause of stroke. Its
diagnosis can be established reliably only on histopathology.
▸ The natural history is unknown and there are no patients or
tumour-related characteristics which could reliably predict
the risk of thromboembolisation.
▸ The shave excision of tumour is a relatively safe procedure.
Therefore, even asymptomatic patients should be actively
assessed for surgery to prevent future complications.
▸ The medical management involves oral anticoagulation in
selected patients to prevent thromboembolisation from
tumour surface; however, such approach has been found to
increase the tumour-related mortality in some series and is
not widely accepted.

Kumar V, et al. BMJ Case Rep 2016. doi:10.1136/bcr-2016-217631

Rare disease
Twitter Follow Arsalan Hashmi at @arsalanhahsmi
Contributors PS contributed to conception and design of the case, drafting the
manuscript, data collection and critical revision of the article. VK contributed to
conception and design of the case, drafting the manuscript, data collection and
critical revision of the article. AH contributed to drafting the article. MM
contributed to data collection, critical revision of the article. This manuscript has
been reviewed, refined and approved by all the authors for publication in BMJ
Case Report.

4
5
6
7
8

Competing interests None declared.
Patient consent Obtained.

9

Provenance and peer review Not commissioned; externally peer reviewed.
10

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1
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3

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Boulmier D, Ecke JE, Verhoye JP. Recurrent myocardial infarction due to obstruction
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Kumar V, et al. BMJ Case Rep 2016. doi:10.1136/bcr-2016-217631

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