Key Teaching Points
Never give up. Keep searching for the etiology even when your normal search did not
yield any result.
When you see patients, always have a broad differential in mind, then narrow it down
with history, physical examination, and other investigations.
Common diagnoses are common, but also know that uncommon ones, though uncommon, do
The decision to perform a root aortogram when the initial coronary angiogram and left
ventriculogram did not reveal a culprit is instructive.
Transfer the patient to a more specialized center when you do not have the expertise
or required setup to care for the patient in your facility.
Papillary fibroelastoma is increasingly being diagnosed because of use of more imaging
in clinical practice. This tumor, if found, should be removed to prevent serious complications,
as occurred in our patient. Even though the tumor is benign, it can cause fatal complications.
The prevalence of primary cardiac tumors varies from 0.02% to 0.45%.
Cardiac papillary fibroelastoma (PFE) is a benign endocardial papilloma. It is considered
the second most prevalent primary cardiac tumor after myxoma and the most prevalent
The typical clinical presentation is either cardiac or neurologic, including transient
ischemic attack, stroke, ischemic heart disease, sudden death, ventricular arrhythmia,
heart failure, syncope, and blindness.
We report a rare case of PFE on the aortic valve in a patient with no previous cardiac
history who presented with multiple cardiac arrests from electrical storm despite
intravenous antiarrhythmic drugs that resolved after surgical removal of the tumor.
The patient was a 64-year-old woman with no previous cardiac history and no family
history of sudden cardiac death who had out-of-hospital cardiac arrests from polymorphic
ventricular tachycardia (VT) requiring shock as noted by emergency medical services.
She had return of spontaneous circulation (ROSC). En route to a community hospital
she continued to experience multiple cardiac arrests that required multiple shocks.
Each of these episodes was followed by ROSC between episodes. These episodes continued
in the emergency room (ER) despite continuous infusion of intravenous amiodarone and
lidocaine. Electrocardiography after ROSC showed atrial fibrillation, ST elevation
in leads aVR and aVL, and diffuse ST depression in most of the other leads (Figure 1).
The patient had another episode of cardiac arrest from VT that degenerated to ventricular
fibrillation, which required another shock (Figure 2). Serum electrolytes were normal.
Emergent point-of-care ultrasound showed normal left ventricular ejection fraction
and no wall-motion abnormality at rest. Because of the emergency nature of the patient’s
condition, only a subcostal view on ultrasound was performed and did not show any
mass. Emergency cardiac catheterization revealed normal coronaries and normal left
ventriculogram. The patient continued to have intermittent VT, and the interventionist
decided to perform aortic root angiography, which showed a filling defect in the aortic
root with a masslike structure intermittently occluding the left main coronary artery
and then coming out of the ostium of the left main coronary artery to allow blood
flow (Figure 3). Cardiac catheterization film video showed the PFE occluding and then
coming out of the ostium of the left main coronary artery (Supplemental Video 1).
This was thought to be PFE on the aortic valve.
Twelve-lead electrocardiogram showing ST elevation in leads aVR and aVL with diffuse
ST depression in the other leads.
Strip showing ventricular fibrillation that degenerated from polymorphic ventricular
Aortic root angiogram showing the papillary fibroelastoma obstructing the left main
coronary artery (A) but intermittently not obstructing the artery (B).
The patient was transferred to our facility for cardiothoracic evaluation and surgical
removal of the suspected mass. She experienced another cardiac arrest en route to
our facility and was again successfully resuscitated. The mass was removed emergently
by cardiothoracic surgery. The mass measured 2 cm × 2 cm and was attached to the commissure
of the left and noncoronary cusps. The VT episodes completely resolved after surgery,
with no recurrences. Postoperative transthoracic echocardiography showed normal ejection
fraction, trileaflet aortic valve of normal thickness, and normal aortic valve leaflet
excursion with evidence of mild aortic regurgitation. Histopathologic report confirmed
the mass to be PFE. Hematoxylin and eosin stain showed mucopolysaccharide matrix with
typical filiform projections (Supplemental Figure 1). The macroscopic picture of the
mass is shown in Supplemental Figure 2. The patient was discharged in stable condition
with recommendation for outpatient follow-up with a cardiologist.
Primary cardiac tumors are rare and have a very low prevalence based on 22 large autopsy
They are divided into primary metastatic tumors and intracardiac tumors originating
from infradiaphragmatic organs.
Cardiac PFE is a benign endocardial papilloma that is common in the sixth to eighth
decades of life.
The aortic valve is most commonly involved (prevalence between 35% and 63%), followed
by the mitral valve (9%–35%), tricuspid valve (6%–15%), and pulmonary valve (0.5%–8%).
Although PFE is considered histologically benign, it can cause life-threatening complications
such as embolic phenomena, which range from transient ischemic attack and amaurosis
fugax to acute ischemic stroke, valvular dysfunction, ventricular fibrillation, and
The tumor can occlude the coronary ostia or embolize into the coronary vessels, resulting
in myocardial infarction, atypical angina, or VT.
The diagnosis is usually made incidentally in asymptomatic patients who undergo echocardiography
for other reasons, or the tumor can present with any of the complications previously
Although transthoracic echocardiography can adequately screen for PFE, transesophageal
echocardiography is currently the preferred method because of its high resolution
and optimal imaging capabilities.
A study on a series of patients with PFE found that PFE can be identified by echocardiography
with a high degree of certainty; is associated with embolic events in a high percentage
of patients; does not cause significant valvular dysfunction despite its tendency
to be located on the valvular endocardium; tends to occur in areas of endocardial
damage; and is not associated with recurrence of embolic phenomena after surgical
On echocardiography, PFE usually appears pedunculated and mobile, with a homogeneous
speckled pattern and characteristic stippling along its edges that correlates with
the papillary projections on the surface of the tumor.
A retrospective study on patients with cardiac PFE found typical echocardiographic
features of PFE: the tumor is round, oval, or irregular in appearance, with well-demarcated
borders and a homogeneous texture; most PFEs are small (99% were <20 mm in the largest
dimension); nearly half of PFEs had small stalks, and those with stalks were mobile;
and PFEs may be single or multiple lesions and are most often associated with cardiac
In our case, aortic root angiography showed a filling defect in the aortic root with
a masslike structure intermittently occluding the left main coronary artery.
Management of PFE depends on its clinical presentation. Patients who are asymptomatic
with a small tumor (diameter <1 cm) that is sessile, fixed, and has no evidence of
impingement on the coronary ostia usually require regular follow-up with serial imaging
studies. Surgery should be offered only if the tumor increases in size or if the tumor
is mobile or pedunculated.
Patients who are asymptomatic in whom the tumor was discovered incidentally during
cardiac surgery for other reasons should be offered surgical resection because there
will be no additional surgical risk.
Patients who are experiencing serious complications such as embolic events, myocardial
infarction, or VT should undergo surgical resection.
Medical management with oral anticoagulation has been suggested for patients with
smaller, nonmobile tumors and for those in whom valve-sparing surgery is not possible.
This approach was associated with 50% tumor-related mortality in 1 study and has not
been universally accepted.
It has been recommended that once the diagnosis of PFE is established, prophylactic
anticoagulation therapy should be initiated to prevent the occurrence of any thrombi
until surgical resection is accomplished.
More than 80% of cardiac PFEs can be treated with shave excision. A study conducted
on patients who underwent shave excision noted no recurrence after the procedure.
Ikegami and colleagues
analyzed 21 cases of PFE on the aortic valve. In their study, the 1 patient who presented
with cardiac arrest had a known history of PFE, unlike our own patient, who did not
have a previous diagnosis of PFE. Their patient had only 1 episode of cardiac arrest,
unlike our patient, who had multiple episodes. Bussani and Silvestri
reported a case of sudden death in a woman with PFE on the aortic valve that chronically
occluded the right coronary ostium. The patient had nonspecific ST-T wave changes
on electrocardiography and was asymptomatic before the event. The PFE was later discovered
The mechanism of ventricular arrhythmia in our patient is thought to be ischemia from
the tumor blocking the left main coronary artery and preventing normal coronary blood
flow. Because the tumor intermittently occluded the left main coronary artery and
was not a permanent occlusion, the patient had an arrhythmia-free period and ROSC
between episodes, with the arrhythmia-free period coinciding with normal coronary
blood flow in the left main coronary artery. The size of the tumor is such that it
could have been seen on full echocardiogram. However, the mass was not seen on the
subcostal point-of-care ultrasound. It is important to note potential complications
from placing a pigtail catheter for aortography include embolization of a fragment
or movement of the mass, which did not occur in our patient. It would have been important
to rule out acute type A aortic dissection, which also could cause coronary occlusion;
however, this was unlikely in our patient because she was stable between cardiac arrests.
Causes of ventricular arrhythmia other than ischemia should always be included in
the differential diagnosis. Of note, in this case the decision to perform root aortography
after the initial coronary angiogram did not reveal a culprit was instructive.
Although PFE is considered a benign tumor, surgery is increasingly being considered
a form of treatment because of its potential serious complications. Once PFE is suspected,
we recommend performing transthoracic echocardiography and early referral for consideration
of surgical removal. Asymptomatic patients do not necessarily require surgical removal
unless the tumor is mobile. The recommendations regarding anticoagulation and surgery
are not based on randomized controlled trials.
Our case emphasizes the need to continue searching for the diagnosis in a patient
with multiple cardiac arrests from electrical storm when the electrocardiogram recorded
between cardiac arrests shows evidence of significant ischemia but cardiac catheterization
of the coronary arteries shows no obstructive disease to explain the arrhythmia. Other
causes of ventricular storm should also be considered. Other primary electrical disease
resulting in electrical storm can be considered in the absence of coronary artery
disease and structural heart disease (eg, long QT syndrome, catecholaminergic polymorphic
VT, Brugada syndrome).