CASE REPORT

Cardiac papillary fibroelastoma. Experience of an institution

Suzelle F. de M. Oliveira; Ricardo Ribeiro Dias; Fábio Fernandes; Noedir A. G. Stolf; Charles Mady; Sérgio Almeida de Oliveira

IInstituto do Coração do Hospital das Clínicas (FMUSP) - São Paulo, SP - Brazil

Correspondence

ABSTRACT

Primary intracardiac tumors are rare, with prevalence between 0.0017% and 0.19% from non-selected autopsy studies. Approximately 75% are benign and almost half of them are myxomas. The remaining tumors are divided among rabdomyomas, lipomas and fibroelastomas. Myxomas are the most common intracardiac tumors in adult age and rabdomyomas the most common among pediatric population.
Papillary fibroelastoma (PFE) is a relative rare benign heart tumor, corresponding to approximately 8% of intracardiac tumors. They most commonly manifested in cardiac valves¹. In the past, they either consisted of necropsy findings or were found in surgical procedures at random. In vivo diagnosis was sporadic². With the improvement of echocardiography techniques, PFE has been more frequently diagnosed. They are usually described as a movable, pedunculate, well-delimited mass and with predilection for valve endocardium. Therapeutic proposal, when they are pedunculate, is surgical resection, preventing cerebral, pulmonary, coronary or peripheral embolic phenomena^1,3. Five cases diagnosed in our institution, in the period from August 1995 to June 2004, will be presented.

Case Reports

Case 1 - A 27-year-old female patient, under Turner Syndrome follow-up, sent due to echocardiographic finding of tumoral, pedunculate, movable in tricuspid valve topography image. She did not show symptoms and her physical exam was normal, except for bodily marks from the syndrome itself. Echocardiogram evidenced a round echodense movable image, located at right atrium, adhered to apical portions of tricuspid valve septal leaflet, with signs of left ventricular filling obstructions. The 1.8 cm x 1.2 cm mass was submitted to surgical exeresis (fig. 1). Microscopic exam confirmed papillary fibroelastoma diagnosis.

Case 2 - Female patient, 67 years old, clinically asymptomatic, submitted to physical examination in which discreet systolic cardiac murmur was detected. Transesophageal echocardiogram showed pedunculate movable mass in aortic valve, which moved towards the aorta (fig. 2). Magnetic nuclear resonance imaging evidenced the referred mass in aortic valve leaflet (fig. 3). She was submitted to tumor exeresis. Diagnostic confirmed papillary fibroelastoma.

Case 3 - A 63-year-old female diabetic patient, with mitral commissurotomy history due to rheumatic mitral stenosis and chronic atrial fibrillation. She made use of oral anticoagulant with coumarinic and showed recurring episodes of transitory ischemic attack (TIA). Echocardiogram evidenced mitral stenosis with valve area of 1.4 cm² and anomalous echoes of 1.8 cm x 1.8 cm in tendinous chord, suggestive of vegetation. There were left atrium thrombus signs. She was sent to surgical treatment and, as a finding, a characteristic tumoral formation with a diameter of, 2 cm, which extended to the papillary muscle, was evidenced in mitral valve anterior leaflet. The exchange of mitral valve through biological prosthesis and tumor exeresis were performed. Microscopic exam confirmed papillary fibroelastoma diagnosis (fig. 4).

Case 4 - Male patient, 59 years old, withhistory severe mitral regurgitation due to myxomatousdegeneration, made use of coumarinic in force of chronic atrialfibrillation and showed a TIA episode. He was under a follow-updue to functional class III heart failure (NYHA). He wassubmitted to surgical treatment, in which chord rupture wasevidenced and quadrangular resection of posterior cuspid wascarried out, followed by raffia and posterior annuloplasty with abovine pericardium strip. Besides chronic valvopathy withfibrosis and myxoid material deposit, tumorgenicity compatiblewith papillary fibroelastoma was evidenced in microscopicexam.

Case 5 - A 49-year-old male patient, withhistory of rheumatic mitral lesion, under follow-up due tofunctional class III (NYHA). He was submitted to mitral valveexchange for biological prosthesis. Histopathological finding ofmaterial sent for study was compatible with papillaryfibroelastoma, located in one of tendinous chords.

Patients evolved without immediate complications and free fromreincidences in all five cases.

Discussion

Papillary fibroelastoma is a low-prevalence benign tumor, withtendency to valvar involvement^2,4,5. Concerningfrequency, it corresponds to the third most common primaryintracardiac tumor, preceded by myxomas andlipomas⁶. It represents less than 10% from allprimary intracardiac tumors, either those studied in autopsy orafter resection^1,7,8.

Approximately 90% of PFE undertakes cardiac valves, usually asa single lesion, on the atrial face of atrioventricular valves oron any one of the sides of semilunar valves^3,4. Theyrarely occur as multiple lesions^1,6.

Approximately 44% of PFE is found in aortic valves, followedby the undertaking of mitral valve in 35% of the cases, in 15%,in tricuspid valves and in 8%, in pulmonary ones⁹.Reports of cases on those tumors have demonstrated undertaking ofall endocardial surfaces, including papillary muscles, tendinouschords, the septum or free walls from any of cardiacchambers^1,5,8,10.

The size of described PFE varied from 0.1 to 4 cm, and most ofthem were smaller than 1cm of diameter⁴. Their genesisremains controversial. Real neoplasias until hamartomas,organized thrombi, reactive responses to mechanic trauma, tosurgical or radiotherapy damage^1,3,4,8,9 have beenconsidered. Prevalence is unknown due to the group ofnon-diagnosed silent tumors⁴.

The age of patients is variable, from cases in neonates towell-advanced age patients^1,4,7. Most of it isdescribed in adults, over 50 years of age, and there is nodifference between sexes^1,4,9.

PFE is an incidental finding in most cases, although amongsymptomatic patients the clinical presentation is variable anddependent on location, motility and size of tumor^4,9.As most of it originates from left chambers (more than 95% of thecases), the most feared complication is the systemicembolization, especially for cerebral or coronarycirculation^4,9. It is not clear whether the embolus istumor- or platelet-origin and if systemic anticoagulation couldprevent from such events^2,4,9. The most commonclinical presentation described was stroke or transitory ischemicattack (TIA). Other described manifestations were: angina,myocardial infarction, sudden death, heart failure, syncope,pulmonary embolism, blindness, peripheral embolism of renalinfarction⁹. In aortic valve tumor patients, suddendeath and myocardial infarction were the most commonmanifestations. In rebuttal, stroke was the prevailingpresentation⁹ in mitral valve tumors. Tumoral motilityis the only independent mortality and non-fatal embolizationpredictor⁹.

Electrocardiographic findings are non-specific, as atrialarrhythmias may occur. Thoracic radiography may demonstrate signsof increase of cardiac chambers, pulmonary hypertension orcongestion, especially if the tumor is occluding the mitralvalve. Transthoracic echocardiogram is the ideal method for tumordiagnosis and characterization, as it usually demonstrates themass with its varied proportions, motile or not, well-delimited,pedunculate or sessile, of round, oval or irregularshape². They are mostly small (99% smaller than 2.0cm)². In a case-control study, the sensitivity andspecificity of echocardiogram was 88.9% and 87.8%,respectively². Magnetic resonance imaging demonstratesthe mass in valve leaflet or cardiac chamber, and the presence ofenhancement with gadolinium in tumoral mass, increases thesuspicion level⁹. Cardiac catheterization does notcontribute for the diagnosis. In coronary angiography it ispossible to visualize total occlusions of coronary arteries, aswell as aneurysmatic dilatations and secondary narrowing totumoral emboli^1,9.

PFE has characteristic appearance, resembling a sea anemone,with multiple ramifications held by a pedicle to endocardium. Athistological exam, it consists of an endothelium coat, whichcovers a connective tissue matrix with variable amounts ofcollagen, smooth muscle cells and elasticfibers^2,4.

For symptomatic patients, surgical exeresis is the choice fortreatment, by trying to always preserve valve tissue and itsfunction⁷. Among asymptomatic individuals, surgicalprocedure is controversial, as tumoral motility is thedetermining factor of surgical indication, for being anindependent embolization and death predictor. Surgery is curatoryand there is no report on reincidences^4,9. Follow-upof asymptomatic patients who were not submitted to surgery mustinclude anticoagulation, although its efficacy in protectionagainst embolic phenomena is controversial².Management before a left side isolated lesion includes surgicalremoval, when the mass is big and/or movable or in the presenceof patent ovale foramen due to the possibility of paradoxicalembolism².

In the last years, PFE has progressed from an autopsyincidental finding to an in vivo diagnosed disease withpotential injurious complications, which require proper diagnosisand treatment. Due to its rareness, data on the treatment andfollow-up are mostly derived from accounts from patients andexperiences as those described in our work.

REFERENCES

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Correspondence to
Charles Mady
Av. Dr. Enéas de Carvalho Aguiar, 44
05403-000 - São Paulo, SP - Brazil
E-mail: charles.mady@incor.usp.br

Received on 09/09/04
Accepted on 03/04/05