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* From the Department of Internal Medicine (Drs. Gray and Alvarado), Denver Health Medical Center; and Department of Medicine (Dr. Chan), National Jewish Medical and Research Center, Denver, CO.
Correspondence to: Julia Alvarado, MD, Department of Internal Medicine, Denver Health Medical Center, 660 Bannock St, M.C. 4000, Denver, CO 80204; e-mail: Julia.Alvarado{at}dhha.org
A 48-year-old woman presented to the hospital with a 3-week history of dyspnea, chest pain, productive cough, and intermittent low-grade fevers. She denied weight loss, night sweats, or hemoptysis. She acknowledged recent IV heroine and cocaine use and a 12-pack-year history of cigarette smoking. Her only tuberculosis risk factor was a period of imprisonment 6 years prior to presentation.
Physical Examination
Her physical examination revealed a temperature of 38.4°C, heart rate of 118 beats/min, BP of 184/90 mm Hg, respiratory rate of 20 breaths/min, and a room air oxygen saturation of 98%. She was cachectic. On cardiovascular examination, she was tachycardic with a loud pulmonic component of the second heart sound. Chest examination was clear to auscultation bilaterally. There was no evidence of lymphadenopathy. Her extremities were without clubbing, cyanosis, or edema. All other aspects of the physical examination were unremarkable.
Laboratory Data
Laboratory data exhibited a WBC count of 28,900/µL with 89% neutrophils, 6% monocytes, 4% lymphocytes, and 1% eosinophils. Hemoglobin, platelet count, serum electrolytes, and lactate dehydrogenase were all within normal limits. Blood, urine, and sputum culture results were negative. Sputum acid-fast bacilli smear and culture results were also negative. Laboratory evaluation for HIV, Legionella, Coccidioides, and Mycoplasma were negative.
Imaging Studies
A chest radiograph (Fig 1 ) and chest CT (Fig 2 ) were obtained.
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Diagnosis: Papillary fibroelastoma of the tricuspid valve confirmed by transesophageal echocardiography
Cardiac masses are 100 to 1,000 times more likely to represent metastatic disease than primary cardiac tumors. Primary cardiac tumors are rare, with an estimated prevalence of 0.02% of the population, of which myxoma is the most common type. Seventy-five percent of primary cardiac tumors are benign, and papillary fibroelastomas are the second-most- common type. These tumors are thought to have an equal distribution among the sexes, and are most often detected in adults. Approximately 90% of tumors are valvular, involving the aortic valve (45%), mitral valve (35%), tricuspid valve (15%), and pulmonic valve (15%). Their etiology is unknown. Yet, the observation has been made that they often occur in areas of endocardial irritation, such as thickened or stenosed cardiac valves and may, therefore, represent a degenerative process.
Grossly, papillary fibroelastomas resemble a sea anemone when immersed in water, as they are composed of elongated, branching papillary fronds that attach to a narrow stalk. Most tumors measure < 1.5 cm in diameter and are solitary. Histologically, these tumors are avascular and have a dense connective tissue core composed of collagen, fibrin, and elastin fibers with an acid mucopolysaccharide matrix. The entire structure is lined by endocardial cells.
Prior to the widespread use of echocardiography, most papillary fibroelastomas were discovered at autopsy. The sensitivity of transthoracic echocardiography to detect a papillary fibroelastoma as small as 0.2 cm is approximately 88.9%. Their unique echocardiographic features include small size, attachment to the endocardium via a stalk or pedicle that is highly mobile, refractive appearance, and areas of echolucency within the tumor itself. Transesophageal echocardiography, with its higher resolution, provides a more comprehensive and accurate assessment of the tumor extent, size, and anatomic attachment.
These tumors may occasionally be visualized by CT. MRI, however, is superior to CT because this modality allows for imaging in multiple planes and improved soft-tissue characterization. MRI has not been directly compared to transesophageal echocardiography in the imaging of cardiac papillary fibroelastomas. In fact, MRI may be superior in imaging cardiac tumors because it is more likely to suggest an etiology, characterize the relationship of the tumor to adjacent structures, and identify associated extracardiac lesions.
Papillary fibroelastomas are most often incidental findings when a patient undergoes echocardiography, cardiac catheterization, cardiac surgery, or postmortem examination. Even though most are clinically silent, they have the potential to cause significant morbidity and mortality due to mass effect or when pieces of the papillary fronds or attached thrombi embolize. Papillary fibroelastomas originating from either side of the heart may cause right- or left-heart failure secondary to conduction abnormalities, as a chamber-occupying lesion or outflow tract obstruction. Additionally, the tumor itself may have profound clinical consequences through obstructive mechanisms; syncope and sudden death have resulted from complete occlusion of both the orifice of the mitral and tricuspid valves.
Predictors of embolization of papillary fibroelastomas include tumor mobility and location on the aortic valve. Papillary fibroelastomas of the left heart have been reported to cause cerebrovascular accidents through embolization. These lesions may also result in occlusion of the left or right coronary ostia or embolization to coronary arteries, resulting in angina pectoris, acute myocardial infarction, and sudden death. Left-sided emboli may also cause mesenteric, renal, or limb ischemia and infarction. As in the case described herein, papillary fibroelastomas affecting the right heart may result in pulmonary embolism and pulmonary hypertension through embolization into the pulmonary vasculature.
No definitive randomized controlled trials or outcome studies have been undertaken to examine treatment outcomes. It is generally thought that cardiac papillary fibroelastomas that are > 1 cm, mobile, and have caused embolic events or are at high risk for causing embolic events are best managed through surgical excision. Fortunately, surgery appears to be curative in that there have been no documented recurrences of these tumors. It has been proposed that asymptomatic patients with nonmobile tumors be managed with close follow-up until they become symptomatic or the tumor becomes mobile as visualized on echocardiography. However, this management approach may be associated with an increased incidence of embolic events when compared to surgical excision.
Long-term anticoagulation or antiplatelet therapy have also been used in nonsurgical candidates and in those awaiting surgery. This treatment approach might prevent the formation of adherent thrombi that may embolize but would not theoretically limit the embolization of tumor fragments themselves. No studies exist evaluating the efficacy of anticoagulation or antiplatelet agents in the treatment of papillary fibroelastomas.
Clinical Course
The chest radiograph revealed an irregular, ill-defined density in the left lower lobe (Fig 1). CT of the chest showed prominent hilar adenopathy, multiple pulmonary nodules, with irregular margins, and a mass in the right ventricle (Fig 2). Based on the imaging findings, transesophageal echocardiography was performed and confirmed a 2.6 x 2.9-cm mass in the right ventricular cavity that appeared adherent to the septum. Unfortunately, tricuspid regurgitation was not detected via echocardiography and, therefore, pulmonary artery pressures could not be estimated.
The patient was treated for community-acquired pneumonia, and after a completed antibiotic course she was taken to the operating room for surgical excision of the right ventricular mass. The papillary fibroelastoma measured approximately 2.5 x 4.0 cm and arose from a papillary muscle attached to the posterior leaflet of the tricuspid valve. This is one of the largest papillary fibroelastomas reported in the literature. It is thought that the pulmonary nodules visualized on the chest CT were emboli originating from either adherent thrombus and/or tumor fragments themselves. It may be presumed that the hilar adenopathy detected on the CT scan was likely a reactive process secondary to the pneumonia. The pathologic findings of the resected papillary fibroelastoma are demonstrated in Figure 3 .
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Clinical Pearls
Footnotes
The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.
Received for publication November 6, 2005. Accepted for publication January 19, 2006.
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