Tricuspid Valve Papillary Fibroelastoma: Echocardiographic Characterization James T. Wolfe 111, MD, Sanford J. Finck, MD, Robert E. Safford, MD, PhD, and Scott T. Persellin, MD Sections of Pathology, Cardiovascular and Thoracic Surgery, Cardiovascular Diseases, and Rheumatology, Ma yo Clinic Jacksonville, Jacksonville, Florida
We report a tricuspid valve papillary fibroelastoma initially detected by transthoracictwo-dimensional echocardiography and subsequently characterized by transesophageal two-dimensional echocardiography. The mass was excised during open heart operation, and the diagnosis was verified grossly and histopathologically.
P
apillary fibroelastoma (PFE) is one of the most common benign primary heart tumors after myxoma [l]. Although usually an asymptomatic incidental finding at autopsy or operation, PFEs occasionally have been associated with transient ischemic attacks, cerebral infarctions, chest pain, or sudden death attributed to occlusion of coronary ostia [l]. Within the last decade, PFEs have been detected echocardiographically in fewer than 20 patients; only two PFEs were related to the tricuspid valve [2, 31. We are aware of only two PFEs studied by transesophageal two-dimensional echocardiography, and neither was associated with the tricuspid valve [4, 51. A 75-year-old woman was examined after a recently resolved left hemiparesis affecting principally her left hand. She had had cerebral ischemic attacks for at least 15 years. A brain scan showed multiple old bilateral cerebral infarcts. A diagnosis of systemic lupus erythematosus was made on the basis of positive serologic tests for antinuclear antibody, anti-double-stranded DNA antibody, anticardiolipin antibody (immunoglobulin G 1:4; immunoglobulin M negative), and rheumatoid factor, low concentration of third component of complement, increased erythrocyte sedimentation rate, grade 1 proteinuria, nonerosive polyarthritis, and Raynauds phenomenon. A two-dimensional echocardiogram showed a large pedunculated right ventricular mass, apparently associated with the septal leaflet of the tricuspid valve, that prolapsed into the right atrium during systole. There was also mild mitral and aortic regurgitation but no evidence of left-sided masses or intracardiac shunting. A transesophageal two-dimensional echocardiogram demonstrated a 2.7-cm-diameter mass that had at least two lobes and was attached just beneath the septal leaflet Accepted for publication June 13, 1990. Address reprint requests to Dr Wolfe, Mayo Clinic Jacksonville, 4500 San Pablo Rd, Jacksonville, FL 32224. 0 1991 by The
Society of Thoracic Surgeons
Transesophageal echocardiography usually provides images far superior to those from transthoracicechocardiography and may be a useful adjunct for intraoperative localization of intracardiac tumors for excision.
(Ann Thorac Surg 1991;51:116-8)
of the tricuspid valve (Fig 1). The mass prolapsed into the right atrium in systole. Left heart catheterization and coronary angiography demonstrated normal left ventricular wall motion, mild mitral regurgitation, and a clinically significant flow-limiting lesion in the mid-right coronary artery. At operation, a polypoid mass attached to the closing edge of the septal leaflet of the tricuspid valve was removed in two pieces without injuring the leaflet. A right coronary artery saphenous vein bypass graft was then performed. Grossly, the neoplasm resembled a sea anemone and was composed of many fronds with minute filiform surface projections (Fig 2A). Microscopically, there were several papillomata, each composed of branching fibroelastic cores with a thin endocardia1 covering (Fig 28).
Fig I . Fibroelastoma of the septal leaflet of the tricuspid valve visualized on the ventricular side of the leaflet in late diastole. (AS = atrial septum; AV = aortic valve; LA = left atrium; LV = left ventricle; MV = mitral valve; RA = right atrium; RV = right ventricle; T = tumor.) 0003-4975/91/$3.50
Ann Thorac Surg 1991;51:11&8
CASE REPORT WOLFE ET AL TRICUSPID PAPILLARY FIBROELASTOMA
117
associated with atheromatous embolism, and many other old infarcts. The heart weighed 406 g and showed left ventricular hypertrophy with severe coronary atherosclerosis. The saphenous vein graft from the aorta to the right coronary artery was patent. An additional 0.4-cm PFE was noted on one chorda to the septal leaflet of the tricuspid valve. No atrial or ventricular septal defects were noted. No masses or clots were found in the left side of the heart.
Comment
A
B Fig 2. (A) Fine filiform projections of pom-pomlike masses offibroelastoma. (Underwater gross photograph.) (B) Branching fibroelastic cores of connective tissue covered by a single layer of endocardia1 cells. (Verhoef-van Gieson; x 100 before 37% reduction.)
The patient was hemodynamically stable postoperatively but with signs of recurrent right cerebral infarction (increased left-sided spastic hemiparesis and decreased mental status). Pneumonia due to Pseudornonas arrugirzosn subsequently developed, and the patient died on postoperative day 15. Postmortem examination showed marked right upper lobe pneumonia with abscess formation, a recent hemorrhagic infarct of the right occipital lobe of the brain
Papillary fibroelastoma has been described in patients 25 to 86 years of age [l], usually after 60 years of age. In one series [l], it constituted 9.5% of all primary tumors and cysts of the heart and pericardium in adults and represented 42 of 241 patients with benign tumors of the heart, making PFE the third most frequent benign tumor of the heart after myxoma (118 patients) and lipoma (45 patients). Among the 42 patients, 45 PFEs occurred equally in the left and right sides of the heart. Since the advent of echocardiography, fewer than 20 PFEs have been detected in patients aged 20 to 75 years, with only 7 in patients aged 50 years or older [2, 6-10, current report]. Among patients studied echocardiographically, some had symptoms that could be attributed to tumor emboli to the brain [4, 6, 71 or had cardiac-related symptoms [2, 3, 5, 9, 101; one has had no symptoms [lo]. All echocardiographically studied lesions occurred in the left side of the heart, except for three on the tricuspid valve [2, 3, current report]. It may be that left-sided lesions are more likely to be symptomatic and therefore are detected by echocardiography at an earlier age. Transesophageal echocardiography is an excellent technique for studying intracardiac masses because the images obtained usually are far superior to those from the transthoracic approach. The transesophageal approach also allows intracardiac shunts to be detected, if present, so that cardiac catheterization studies usually can be limited to coronary angiography alone in patients likely to have coronary artery disease. Additionally, intraoperative transesophageal echocardiography can be a useful adjunct for precise localization of intracardiac tumors for surgical excision and for assessing the adequacy of septal and valve repairs when necessary.
References 1. McAllister HA Jr, Fenoglio JJ Jr. Tumors of the cardiovascular system. In: Atlas of tumor pathology. Second series, fascicle 15. Washington DC: Armed Forces Institute of Pathology, 1978. 2. Frumin H, ODonnell L, Kerin NZ, Levine F, Nathan LE Jr, Klein SP. Two-dimensional echocardiographic detection and diagnostic features of tricuspid papillary fibroelastoma. J Am Coll Cardiol 1983;2:1016-8. 3. Mohan JC, Goel PK, Gambhir DS, Khanna SK, Arora R. Calcified mobile papillary fibroelastoma of the tricuspid valve: a case report. Indian Heart J 1987;39:237-9. 4. Topol EJ, Biern RO, Reitz BA. Cardiac papillary fibroelastoma and stroke: echocardiographic diagnosis and guide to excision. Am J Med 1986;80:129-32.
118
CASE REPORT WOLFE ET AL TRICUSPID PAPILLARY FIBROELASTOMA
5. de Virgilio C, Dubrow TJ, Robertson JM, et al. Detection of multiple cardiac papillary fibroelastomas using transesophageal echocardiography. Ann Thorac Surg 1989;48:119-21. 6. Kasarskis EJ, OConnor W, Earle G. Embolic stroke from cardiac papillary fibroelastomas. Stroke 1988;19:1171-3. 7. Ong LS, Nanda NC, Barold SS. Two-dimensional echocardiographic detection and diagnostic features of left ventricular papillary fibroelastoma. Am Heart J 1982;103:917-8. 8. Almagro UA, Perry LS, Choi H, Pintar K. Papillary fibroelas-
Ann Thorac Surg 1991;51:11&8
toma of the heart: report of six cases. Arch Pathol Lab Med 1982;106318-21. 9. Cha SD, Incarvito J, Fernandez J, Chang KS, Maranhao V, Gooch AS. Giant Lambl's excrescences of papillary muscle and aortic valve: echocardiographic, angiographic and pathologic findings. Clin Cardiol 1981;4:51-4. 10. Shub C, Tajik AJ, Seward JB, et al. Cardiac papillary fibroelastomas: two-dimensional echocardiographic recognition. Mayo Clin Proc 1981;56:62%33.
Notice From the American Board of Thoracic Surgery The American Board of Thoracic Surgery began its recertification process in 1984. Diplomates interested in participating in this examination should maintain a documented list of the operations they performed during the year prior to application for recertification. This practice review should consist of 1 year's consecutive major operative experiences. (If more than 100 cases occur in 1 year, only 100 need to be listed.) They should also keep a record of their attendance at approved postgraduate medical education activities for the 2 years prior to application. A minimum of 100 hours of approved CME activity is required. In place of a cognitive examination, candidates for recertification will be required to complete both the general thoracic and cardiac portions of the SESATS IV syllabus (Self-Education/Self-Assessmentin Thoracic Surgery). It is not necessary for candidates to purchase
SESATS IV booklets prior to applying for recertification. SESATS IV booklets will be forwarded to candidates after their applications have been accepted. Diplomates whose 10-year certificates will expire in 1993 may begin the recertification process in 1991. This new certificate will be dated 10 years from the time of expiration of the original certificate. Recertification is also open to any diplomate with an unlimited certificate and will in no way affect the validity of the orignal certificate. The deadline for submission of applications is July 1, 1991. A recertification brochure outlining the rules and requirements for recertification in thoracic surgery is available upon request from the American Board of Thoracic Surgery, One Rotary Center, Suite 803, Evanston, IL 60201.
We report a tricuspid valve papillary fibroelastoma initially detected by transthoracictwo-dimensional echocardiography and subsequently characterized by transesophageal two-dimensional echocardiography. The mass was excised during open heart operation, and the diagnosis was verified grossly and histopathologically.
P
apillary fibroelastoma (PFE) is one of the most common benign primary heart tumors after myxoma [l]. Although usually an asymptomatic incidental finding at autopsy or operation, PFEs occasionally have been associated with transient ischemic attacks, cerebral infarctions, chest pain, or sudden death attributed to occlusion of coronary ostia [l]. Within the last decade, PFEs have been detected echocardiographically in fewer than 20 patients; only two PFEs were related to the tricuspid valve [2, 31. We are aware of only two PFEs studied by transesophageal two-dimensional echocardiography, and neither was associated with the tricuspid valve [4, 51. A 75-year-old woman was examined after a recently resolved left hemiparesis affecting principally her left hand. She had had cerebral ischemic attacks for at least 15 years. A brain scan showed multiple old bilateral cerebral infarcts. A diagnosis of systemic lupus erythematosus was made on the basis of positive serologic tests for antinuclear antibody, anti-double-stranded DNA antibody, anticardiolipin antibody (immunoglobulin G 1:4; immunoglobulin M negative), and rheumatoid factor, low concentration of third component of complement, increased erythrocyte sedimentation rate, grade 1 proteinuria, nonerosive polyarthritis, and Raynauds phenomenon. A two-dimensional echocardiogram showed a large pedunculated right ventricular mass, apparently associated with the septal leaflet of the tricuspid valve, that prolapsed into the right atrium during systole. There was also mild mitral and aortic regurgitation but no evidence of left-sided masses or intracardiac shunting. A transesophageal two-dimensional echocardiogram demonstrated a 2.7-cm-diameter mass that had at least two lobes and was attached just beneath the septal leaflet Accepted for publication June 13, 1990. Address reprint requests to Dr Wolfe, Mayo Clinic Jacksonville, 4500 San Pablo Rd, Jacksonville, FL 32224. 0 1991 by The
Society of Thoracic Surgeons
Transesophageal echocardiography usually provides images far superior to those from transthoracicechocardiography and may be a useful adjunct for intraoperative localization of intracardiac tumors for excision.
(Ann Thorac Surg 1991;51:116-8)
of the tricuspid valve (Fig 1). The mass prolapsed into the right atrium in systole. Left heart catheterization and coronary angiography demonstrated normal left ventricular wall motion, mild mitral regurgitation, and a clinically significant flow-limiting lesion in the mid-right coronary artery. At operation, a polypoid mass attached to the closing edge of the septal leaflet of the tricuspid valve was removed in two pieces without injuring the leaflet. A right coronary artery saphenous vein bypass graft was then performed. Grossly, the neoplasm resembled a sea anemone and was composed of many fronds with minute filiform surface projections (Fig 2A). Microscopically, there were several papillomata, each composed of branching fibroelastic cores with a thin endocardia1 covering (Fig 28).
Fig I . Fibroelastoma of the septal leaflet of the tricuspid valve visualized on the ventricular side of the leaflet in late diastole. (AS = atrial septum; AV = aortic valve; LA = left atrium; LV = left ventricle; MV = mitral valve; RA = right atrium; RV = right ventricle; T = tumor.) 0003-4975/91/$3.50
Ann Thorac Surg 1991;51:11&8
CASE REPORT WOLFE ET AL TRICUSPID PAPILLARY FIBROELASTOMA
117
associated with atheromatous embolism, and many other old infarcts. The heart weighed 406 g and showed left ventricular hypertrophy with severe coronary atherosclerosis. The saphenous vein graft from the aorta to the right coronary artery was patent. An additional 0.4-cm PFE was noted on one chorda to the septal leaflet of the tricuspid valve. No atrial or ventricular septal defects were noted. No masses or clots were found in the left side of the heart.
Comment
A
B Fig 2. (A) Fine filiform projections of pom-pomlike masses offibroelastoma. (Underwater gross photograph.) (B) Branching fibroelastic cores of connective tissue covered by a single layer of endocardia1 cells. (Verhoef-van Gieson; x 100 before 37% reduction.)
The patient was hemodynamically stable postoperatively but with signs of recurrent right cerebral infarction (increased left-sided spastic hemiparesis and decreased mental status). Pneumonia due to Pseudornonas arrugirzosn subsequently developed, and the patient died on postoperative day 15. Postmortem examination showed marked right upper lobe pneumonia with abscess formation, a recent hemorrhagic infarct of the right occipital lobe of the brain
Papillary fibroelastoma has been described in patients 25 to 86 years of age [l], usually after 60 years of age. In one series [l], it constituted 9.5% of all primary tumors and cysts of the heart and pericardium in adults and represented 42 of 241 patients with benign tumors of the heart, making PFE the third most frequent benign tumor of the heart after myxoma (118 patients) and lipoma (45 patients). Among the 42 patients, 45 PFEs occurred equally in the left and right sides of the heart. Since the advent of echocardiography, fewer than 20 PFEs have been detected in patients aged 20 to 75 years, with only 7 in patients aged 50 years or older [2, 6-10, current report]. Among patients studied echocardiographically, some had symptoms that could be attributed to tumor emboli to the brain [4, 6, 71 or had cardiac-related symptoms [2, 3, 5, 9, 101; one has had no symptoms [lo]. All echocardiographically studied lesions occurred in the left side of the heart, except for three on the tricuspid valve [2, 3, current report]. It may be that left-sided lesions are more likely to be symptomatic and therefore are detected by echocardiography at an earlier age. Transesophageal echocardiography is an excellent technique for studying intracardiac masses because the images obtained usually are far superior to those from the transthoracic approach. The transesophageal approach also allows intracardiac shunts to be detected, if present, so that cardiac catheterization studies usually can be limited to coronary angiography alone in patients likely to have coronary artery disease. Additionally, intraoperative transesophageal echocardiography can be a useful adjunct for precise localization of intracardiac tumors for surgical excision and for assessing the adequacy of septal and valve repairs when necessary.
References 1. McAllister HA Jr, Fenoglio JJ Jr. Tumors of the cardiovascular system. In: Atlas of tumor pathology. Second series, fascicle 15. Washington DC: Armed Forces Institute of Pathology, 1978. 2. Frumin H, ODonnell L, Kerin NZ, Levine F, Nathan LE Jr, Klein SP. Two-dimensional echocardiographic detection and diagnostic features of tricuspid papillary fibroelastoma. J Am Coll Cardiol 1983;2:1016-8. 3. Mohan JC, Goel PK, Gambhir DS, Khanna SK, Arora R. Calcified mobile papillary fibroelastoma of the tricuspid valve: a case report. Indian Heart J 1987;39:237-9. 4. Topol EJ, Biern RO, Reitz BA. Cardiac papillary fibroelastoma and stroke: echocardiographic diagnosis and guide to excision. Am J Med 1986;80:129-32.
118
CASE REPORT WOLFE ET AL TRICUSPID PAPILLARY FIBROELASTOMA
5. de Virgilio C, Dubrow TJ, Robertson JM, et al. Detection of multiple cardiac papillary fibroelastomas using transesophageal echocardiography. Ann Thorac Surg 1989;48:119-21. 6. Kasarskis EJ, OConnor W, Earle G. Embolic stroke from cardiac papillary fibroelastomas. Stroke 1988;19:1171-3. 7. Ong LS, Nanda NC, Barold SS. Two-dimensional echocardiographic detection and diagnostic features of left ventricular papillary fibroelastoma. Am Heart J 1982;103:917-8. 8. Almagro UA, Perry LS, Choi H, Pintar K. Papillary fibroelas-
Ann Thorac Surg 1991;51:11&8
toma of the heart: report of six cases. Arch Pathol Lab Med 1982;106318-21. 9. Cha SD, Incarvito J, Fernandez J, Chang KS, Maranhao V, Gooch AS. Giant Lambl's excrescences of papillary muscle and aortic valve: echocardiographic, angiographic and pathologic findings. Clin Cardiol 1981;4:51-4. 10. Shub C, Tajik AJ, Seward JB, et al. Cardiac papillary fibroelastomas: two-dimensional echocardiographic recognition. Mayo Clin Proc 1981;56:62%33.
Notice From the American Board of Thoracic Surgery The American Board of Thoracic Surgery began its recertification process in 1984. Diplomates interested in participating in this examination should maintain a documented list of the operations they performed during the year prior to application for recertification. This practice review should consist of 1 year's consecutive major operative experiences. (If more than 100 cases occur in 1 year, only 100 need to be listed.) They should also keep a record of their attendance at approved postgraduate medical education activities for the 2 years prior to application. A minimum of 100 hours of approved CME activity is required. In place of a cognitive examination, candidates for recertification will be required to complete both the general thoracic and cardiac portions of the SESATS IV syllabus (Self-Education/Self-Assessmentin Thoracic Surgery). It is not necessary for candidates to purchase
SESATS IV booklets prior to applying for recertification. SESATS IV booklets will be forwarded to candidates after their applications have been accepted. Diplomates whose 10-year certificates will expire in 1993 may begin the recertification process in 1991. This new certificate will be dated 10 years from the time of expiration of the original certificate. Recertification is also open to any diplomate with an unlimited certificate and will in no way affect the validity of the orignal certificate. The deadline for submission of applications is July 1, 1991. A recertification brochure outlining the rules and requirements for recertification in thoracic surgery is available upon request from the American Board of Thoracic Surgery, One Rotary Center, Suite 803, Evanston, IL 60201.
