CARDIOVASCULAR JOURNAL OF AFRICA • Vol 23, No 7, August 2012
e8
AFRICA
The patient was taken to the operating room for removal
of the fibroelastoma. The chest was opened using a minimally
invasive technique at the level of the third intercostal space. The
aorta was opened 4 cm above the origin of the right coronary
artery. Intra-operative examination of the valve showed a 1-cm
fibroelastoma attached to the edge of the left coronary cusp. Two
small fibroelastomas were noted attached to the right coronary
and non-coronary cusps. All three lesions were excised without
damage to the aortic valve cusps (Fig. 4).
Postoperative TEE showed no decline in LVEF and absence
of aortic regurgitation. The patient tolerated the procedure well
and had an uneventful recovery. The pathological specimen
showed multiple irregular fragments of tan-white soft tissue with
fine papillary excrescencies. Special stains for connective tissue
(Masson) and elastic tissue were positive, confirming that the
specimen was a fibroelastoma.
Discussion
Papillary fibroelastomas are extremely rare primary cardiac
tumours. They usually originate from the valves. The tumours
most commonly involve aortic, mitral, tricuspid and pulmonary
valves (in order of decreasing frequency).
5
However, they can
also affect other endothelial surfaces of the heart and Eustachian
valves. Papillary fibroelastomas are small avascular tumours
derived from endocardial elements and resemble sea anemones
due to their multiple fronds.
4,6
The majority of tumours are asymptomatic. Given the
widespread use of echocardiography, the tumours are sometimes
found during routine transthoracic echocardiograms, as in our
case. Transoesophageal echocardiography is used for more
precise evaluation of the tumour. However, smaller tumours
could be missed even by this technique.
Tumour attachment to the endocardium via a pedicle, its
high mobility, speckled appearance with central echolucency,
and shimmer at the tumour–blood interface are some of the
features which help echocardiographic diagnosis.
7
The evolution
of coronary computed tomography angiography and cardiac
magnetic resonance imaging may provide additional diagnostic
tools for identifying the tumours earlier.
Papillary fibroelastomas can manifest as an embolic
phenomenon in the form of transient ischaemic attack, stroke,
angina, myocardial infarction, pulmonary embolism, retinal
embolism, syncope and death.
5,7
Even though treatment with
antiplatelet medications and anticoagulation with warfarin are
some of the treatment options, it has been suggested that
a surgical approach should be strongly considered, even in
asymptomatic patients, given the severity of the potential
complications.
4,7
Conclusion
Due to the rarity of the tumour and the lack of large randomised
trials, the surgical recommendation is based on isolated case
reports and professional consensus. Valve repair and replacement
carries significant short- and long-term complications including
increased risk of endocarditis, embolic phenomena, deterioration
and malfunction of the prosthesis. In our case, the surgery was
performed using a minimally invasive approach with a valve-
sparing technique, decreasing the chance of both short- and
long-term complications and facilitating earlier recovery of the
patient.
References
1.
Lam KY, Dickens P, Chan ACL. Tumors of the heart: A 20 year experi-
ence with review of 12,485 consecutive autopsies.
Arch Pathol Med
1993;
117
: 1027–1031.
Fig. 3. Soft tissue mass associated with the left coronary
cusp of the aortic valve on CT scan.
Fig. 4. Surgical specimen of the fibroelastomas after
resection.
