Papillary fibroelastoma of the pulmonary valve: a 14-year follow-up
Pedro H B Engster1, MD, Said
Alsidawi2*, MD
1Independent researcher
2Department of Cardiovascular Diseases, Mayo Clinic,
Scottsdale, AZ
*Correspondence to Said Alsidawi, MD, Department of Cardiovascular
Diseases, Mayo Clinic, Scottsdale; Alsidawi.Said@mayo.edu
Additional study information:
The data that support the findings of this study are available from the
corresponding author upon reasonable request.
Funding: None.
No ethical clearance was obtained for writing this case report
Informed consent was obtained and can be presented if requested
Abstract
Primary cardiac tumors are exceedingly rare. Among them, papillary
fibroelastomas (PFEs) are the third most common. These tumors are often
incidental findings but can also precipitate a myriad of clinical
presentations, mainly embolic events. Most common in the left-sided
valves, PFEs rarely occur in the right side of the heart. They are
usually resected surgically following diagnosis, thwarting assessment of
their natural history. We present the case of a woman diagnosed with
pulmonary valve PFE following recurrent pulmonary embolism who did not
undergo surgery, allowing for an extended follow-up of the condition.
Introduction
Primary cardiac tumors are exceedingly rare, with an estimated
prevalence between 0.0017% and 0.028%1-3 in autopsy
series and 0.15% in echocardiographic series.2 Among
these tumors, papillary fibroelastomas (PFEs) are the third most common,
accounting for less than 10% of them.4-7 These
pedunculated lesions chiefly grow in cardiac valves, the most common
heart tumors in this location.7 The pulmonary valve is
least commonly affected.8 Although histologically
benign and most often found incidentally, PFEs can precipitate
life-threatening conditions, usually embolic events, but also heart
failure and sudden cardiac death.2,8 Surgical tumor
removal is considered curative, and a plurality of patients undergo
tumor excision.8 This approach, however, has dampened
attempts to elucidate their natural history. We present the case of an
older woman with recurrent pulmonary emboli attributable to a pulmonary
valve papillary fibroelastoma (PVPFE) who did not undergo surgery and
had a 14-year follow-up.
Case report
A 74-year-old woman presented to a local hospital in 2008 with shortness
of breath and chest pain. Computerized tomography showed filling defects
of the pulmonary artery branches and a small pulmonary valve nodule that
was not initially recognized as pathological (Fig. 1). The patient was
diagnosed with pulmonary embolism (PE) and treated with anticoagulation
therapy for six months.
She presented back in 2016 with recurrent symptoms attributable to
another pulmonary embolus. A repeat CT confirmed the diagnosis of PE and
revealed growth of the pulmonary valve nodule. The nodule measured 8mm
and was attached to the pulmonary valve by a stalk (Fig. 2). A
presumptive diagnosis of pulmonary valve papillary fibroelastoma was
made. Surgical resection of the lesion was considered. However, the
patient deferred surgical intervention, partly due to her several
comorbidities, including advanced age, frailty, and multiple orthopedic
issues such as numerous back and cervical spine surgeries. Instead, the
patient was commenced on long-term anticoagulation with warfarin.
Her latest follow-up was in 2022, with a CT scan showing further growth
of the pedunculated nodule (Fig. 3). It now measured 20mm, representing
a 2mm/year growth rate. Transthoracic and transesophageal
echocardiograms were performed, further delineating the PVPFE (Figs. 4
and 5). They showed normal valve function. The patient had not had any
other embolic events since 2016 and elected to maintain anticoagulation
without surgical intervention.
Discussion
PFEs are rare tumors that can paint a challenging clinical picture.
Their infrequent nature combined with variable manifestations demands
clinicians to maintain a high suspicion index for them. In our case,
even with proper imaging, the tumor was not immediately recognized.
Imaging is the mainstay of diagnosis, particularly echocardiograms,
which allow for evaluation of the mass and assessment of valve
function.9
When imaged, PFEs usually present as round, oval, or irregular nodules.
Sun et al. have reported that about half of them have stalks, which are
associated with increased tumor mobility.10 These
tumors are best evaluated with TTE or TEE, as CT and MRI may fail to
detect smaller tumors. However, CT and MRI may still have a role in
diagnosing neoplasms in atypical locations and detecting concomitant
extracardiac disease.9 Increasing imaging quality and
application of new techniques should improve sensitivity for these
exams.9,11 They can also help evaluate a patient with
established PFE for preoperative planning or assessment of alternate
diagnoses.
The differential diagnosis of PFEs encompasses vegetations, thrombi, and
other cardiac tumors - mainly myxomas.8,11 Compared to
PFEs, myxomas are more likely to be located in the right atrium, be
larger, and cause symptoms.9 Thrombi can be
differentiated by their laminated appearance, irregular contours, and
absence of a pedicle.8,11 Vegetations can be similar
to PFEs in imaging; however, patients usually have clinical signs of
endocarditis, valvular dysfunction or destruction, and evolving lesions
and clinical course. Other less common differential diagnoses include
Libman-Sacks vegetations and Lambl’s excrescences.8
The pathogenesis of PFEs remains unknown. On the one hand, some believe
they are reactive processes to trauma and hemodynamic stress, while on
the other hand, others have postulated them to be
hamartomas.9,12 The presence of other tumors in
patients with PFEs has been reported, but the meaning of this
association is unknown.12
Histologically, PFEs are small and highly papillary masses often
attached to cardiac tissue through a stalk. This appearance has led some
researchers to liken them to sea anemones.5,10,13 They
are also firmly attached to the endocardium, and embolization may occur
from its fragile papillary fronds or a thrombus formed on its surface
rather than dislodgement of the tumor itself.8
PFEs are usually found incidentally during cardiac surgery, imaging, or
autopsy. Their management usually involves surgical resection to prevent
complications.8 This has made it hard to assess their
natural history and the effectiveness of medical treatment to prevent
complications such as embolization. This is especially true for PVPFEs
since the pulmonary valve is one of the least common sites for the
development of PFEs, representing only 8% of cases.8
Our patient, an older woman with multiple comorbidities, elected not to
have her tumor excised. The 14-year follow-up she received is, to the
best of our knowledge, the longest described follow-up of a patient with
PVPFE in the literature. This allowed us to highlight the natural
history of PVPFEs and their growth rate. Our findings suggest that PFEs
may have a variable rate of growth than the previously described growth
rate of 0.5 ± 0.9 mm/year.14
Conflicts of interest
All authors declare that they have no conflicts of interest.
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