his planned chemotherapy for myelodysplastic syndrome had been stopped a month before due to fungal infection of the orbit .
the initial absolute neutrophil count was only 130/mm , and antibiotic therapy was initiated under the possibility of pneumonia or aggravation of orbital infection . even after 7 days of antibiotic therapy , the fever persisted and no evidence of infection was found .
we performed transthoracic echocardiography under suspicion of infective endocarditis ; a 13 mm echogenic mass was found on the atrial side of the tricuspid valve ( fig .
based on the clinical symptoms and the echocardiographic findings , the patient was highly suspected of having infective endocarditis of the tricuspid valve .
he was scheduled for emergency surgical removal due to the possibility of fungal endocarditis and the poor clinical course of infective endocarditis in immunocompromised patients . with a cardiopulmonary bypass
, the right atrium was opened after aortic cross - clamping and cardioplegic arrest . on the exploration , a solitary polypoid gelatinous mass 1316 mm in size infiltrating the septal leaflet of the tricuspid valve
2 ) . although the tumor had a stalk , simple resection without making a defect in the septal leaflet appeared to be impossible , because the leaflet was too thin .
therefore , the mass was excised with part of the septal leaflet of the tricuspid valve using the quadrangular resection technique , followed by reconstruction with autologous pericardium .
after successful weaning from the cardiopulmonary bypass , intraoperative transesophageal echocardiography was performed , showing no residual tumors .
papillary fibroelastoma was pathologically confirmed , and no evidence of infective endocarditis was found ( fig .
follow - up transthoracic echocardiography showed no residual or recurrent tumor , with uneventful postoperative recovery .
primary cardiac tumor is very rare , and its frequency is approximately 0.02% according to the data from 22 autopsy cases . cardiac papillary fibroelastoma is the third most common benign primary cardiac neoplasm and the most common cardiac valvular tumor . grossly , cardiac papillary fibroelastoma is a gelatinous mass with multiple narrow papillary fronds , and it attaches to the endocardium via a short pedicle .
when the tumor is put in saline immediately after excision , it typically has a sea anemone - like appearance .
the microscopic appearance is an avascular finger - like process with a core of elastin surrounded by collagen , which is covered by a single layer of endothelium .
the size of a cardiac papillary fibroelastoma varies from 2 to 70 mm , but the majority of tumors are approximately 10 mm in diameter .
the tumor is usually solitary , but rare cases of multiple cardiac papillary fibroelastoma have been reported .
most of the tumors are located on the valvular surface , especially near or on the aortic valve ( 44% ) and mitral valve ( 35% ) .
less frequently , the tumor infiltrates the pulmonary valve ( 8% ) or the leaflets of the tricuspid valve ( 15% ) .
the clinical presentation of a cardiac papillary fibroelastoma varies from no symptoms to severe thromboembolic complications , including transient ischemic attack , stroke , and myocardial infarction .
other complications include pulmonary embolism , congestive heart failure , near syncope , and sudden death .
cardiac papillary fibroelastoma occasionally manifests with fever , which abates after removal of the tumor .
our patient with cardiac papillary fibroelastoma had a fever , which did not subside even after several days of antibiotic therapy . however , after surgical removal of the tumor , the fever disappeared during the hospital stay .
occasionally , a small cardiac papillary fibroelastoma may not be detected with transthoracic echocardiography ; therefore , transesophageal echocardiography is required to further assess such a small tumor .
computed tomography and magnetic resonance imaging are also helpful in detecting cardiac papillary fibroelastoma , but their diagnostic accuracy is relatively low . the mobility and location of the papillary fibroelastoma that has developed on the valvular surface can be misdiagnosed as infective vegetation . in comparison with cardiac papillary fibroelastoma , infective vegetation is usually associated with clinical signs of endocarditis and valvular destruction , and may resolve or change over a short period after treatment .
it is obvious that surgical excision is necessary in symptomatic patients with intracardiac tumors proven by echocardiography , regardless of the type and the location of tumors .
however , when the tumor is a left - sided lesion , surgical excision is recommended due to high - risk thromboembolic events .
controversially , right - sided lesions are observed and surgically removed only in case they become symptomatic
. the risk of cardiac surgery in patients with hematologic malignancies remains to be established .
fecher et al .   demonstrated , in a study of 24 patients with hematologic malignancies undergoing cardiac surgery , that there was 1 in - hospital patient death ( 4.1% ) , 12 patients ( 50% ) with a minor or major complication , and 7 patients requiring reoperations within 30 days due to bleeding , infection , and other clinical conditions .
chan et al .   reported , in a study of 26 patients with hematologic malignancies , that although there was no significant difference in mortality between patients with and without hematologic malignancies , there were significantly higher rates of transfusion , pneumonia , and sepsis in patients with hematologic malignancies than in patients without .
the patient in this study improved after removal of the cardiac papillary fibroelastoma without any complications , and he is still alive at the 6-month follow - up .
 we report a 72-year - old male with known myelodysplastic syndrome who presented to the emergency department with a 7-day history of fever and dyspnea . 
 echocardiography revealed a round echogenic mass 1316 mm in size attached to the atrial side of the tricuspid valve . 
 considering the high risk of infective endocarditis in the patient with a low absolute neutrophil count ( 130/mm3 ) , emergency surgery was performed . 
 intraoperatively , a single gelatinous neoplasm was resected , and subsequent reconstruction of the involved leaflet was accomplished using autologous pericardium . 
 the tumor was pathologically confirmed as papillary fibroelastoma with no evidence of infective endocarditis . 
 papillary fibroelastoma is a rare cardiac neoplasm that occurs in either the mitral or aortic valves . 
 interestingly , a few cases of tricuspid valve papillary fibroelastoma have been reported so far . 
 similar echocardiographic findings between vegetation and tricuspid valve neoplasm make it difficult to distinguish these two disease entities . 
CASE REPORT
DISCUSSION