Discussion
PFE are second in frequency, after myxomas, among primary cardiac
tumors, representing about 10%. They present macroscopically with the
appearance of a sea anemone with leafy arms, starting from a stalked
central core5, consisting of avascular papillary
tissue. The 80% of PFE originate from the valvular surfaces and the
remaining 20% from other cardiac localizations; aortic localization is
extremely rare and can occur in all aortic segments. To the best of our
knowledge, only three cases of aortic wall PFE have been described in
literature3,6,7.
In aortic wall PFE, although the majority of patients remain
asymptomatic, clinical onset usually occurs with cerebral ischemic or
coronary symptoms due to mass embolization or partial or total coronary
occlusion3,4,6,7. The diagnosis is very difficult
mainly due to their low frequency and consequently the lack of knowledge
of the case; this is the unique case of aortic PFE reported in our
center after about 20,000 cardiac interventions. In addiction
presentation with angina symptoms in a patient with previous PTCA caused
diagnostic confusion. Usually transthoracic echocardiography (TTE) is
sufficient to allow a diagnosis with high sensitivity and specificity
(88.8% and 88.7% respectively)8 where TEE has even
greater sensitivity in smaller PFEs. The execution of a CT scan can be
useful to define the diagnosis itself which, due to its extreme rarity,
is hardly immediately evaluated. In fact, in our case the typical angina
symptoms would probably have led us to perform a new coronary angiogram,
with the associated embolic risk, given the difficulty to think of a PFE
of the aorta. The presence of the mass, already evident in TTE, led us
to the execution of TEE which allowed a diagnosis with high probability;
the CT confirmed the size and position of the mass allowing us to place
the clinical suspicion and the consequent surgical indication to the
removal. Coronary angiography was not performed to avoid mobilization of
the mass.
There are no current guidelines for the treatment of PFE. In patients
with embolic events, surgery is strongly recommended9.
In asymptomatic patients the opinions are different and are based on
mass mobility, position, concomitant surgery etc. We believe that, since
the tumor is positioned in the aortic flow, surgery should always be
indicated for the potential embolic risk, except in asymptomatic
highrisk patients. Given the benign nature of the tumor a simple but
accurate excision is sufficient. After surgical resection no recurrence
of the tumor has ever been reported6.