The incidence of papillary fibroelastoma is less than 10% of all cardiac tumors.2
In some cases, cardiac fibroelastomas are considered to be congenital; however, in most patients they are acquired.1
These tumors occur equally often in males and females. They were first described as incidental findings at autopsy, with a prevalence of 0.002% to 0.33%, or at surgery.2
The clinical significance of papillary fibroelastomas has been debated extensively; such controversy persists even when the risk of cerebral or coronary embolization indicates surgical excision.
Papillary fibroelastomas originate from the valves or the endothelial surface of the heart. In a recent review of 410 reported cardiac papillary fibroelastomas, 84% arose from cardiac valves.6
Another study reported cardiac papillary fibroelastomas to arise from aortic, mitral, tricuspid, and pulmonic valves in 35%, 25%, 17%, and 13% of cases, respectively.1
These tumors are small, avascular masses, which, in 91% of cases, are single lesions. Frequently, they are mobile with a thin stalk (43%) and have a mean diameter of 3 × 10 mm.7
They have multiple papillary fronds that cause them to resemble sea anemones. Histologically, papillary fibroelastomas consist of a central core of dense connective tissue, surrounded by a layer of loose connective tissue and covered by hyperplastic endothelial cells.
Detection of papillary fibroelastomas has increased with the use of echocardiography. Echodensity of the tumor's central collagen core strongly supports the diagnosis and allows differentiation from other intracardiac tumors, vegetations, or mural thrombi.7
Echocardiographic examination does not reliably reveal multiple lesions, although it is the best diagnostic tool for papillary fibroelastomas. Coronary angiography is not advisable because of the fragile nature of the tumor.
The 1st surgical resection of a cardiac papillary fibroelastoma was performed by Lichtenstein in 1979.8
The presence of a such a tumor is an indication for surgical resection, because even small lesions (3–4 mm) can cause life-threatening complications. After tumor excision, aortic leaflets can be restored with a homograft aortic cusp or an autologous pericardial patch. Tumor resection alone may be sufficient, especially when the mass arises from the cusp-free edge. Normal function and structure of the aortic valve can usually be restored.
It is rare to find multiple lesions of the aortic valve, in part because additional lesions may be missed on TEE, as in our patient. In many cases, a 2nd lesion is detected only upon surgical inspection.4,5
Therefore, the entire heart should be assessed as thoroughly as possible during surgery. Smaller lesions may be attached beneath the leaflet surface. Moreover, multiple cardiac papillary fibroelastomas can involve separate valves or both the left and right cavities.3
Additional lesions most likely represent different stages of the same tumor.9
Recurrence rates of papillary fibroelastomas after surgical excision have not been reported, but careful follow-up is necessary.