Papillary Fibroelastomas of the Heart

Abstract

Cardiac papillary fibroelastomas (CPFs), the second most common primary cardiac tumor, are benign endocardial papillomas predominantly affecting the cardiac valves. Although CPFs are rare and benign tumors, they may result in life-threatening complications. Early diagnosis of this condition is important, since it represents a surgically correctable cause of systemic embolism, myocardial infarction, stroke, acute valve dysfunction, and sudden cardiac death. This review summarizes the significance and clinical approach for the diagnosis of this cardiac entity. The differential diagnosis, histological characteristics of CPF and current treatment strategies are also discussed.

cardiovascular surgery - heart valve surgery - heart tumors - papillary fibroelastoma

References

• 1 Reynen K. Frequency of primary tumors of the heart.  Am J Cardiol. 1996;  77 107
• 2 Goldberg H P, Glenn F, Dotter C T, Steinberg I. Myxoma of the left atrium; diagnosis made during life with operative and post-mortem findings.  Circulation. 1952;  6 762-767
• 3 Lam K Y, Dickens P, Chan A C. Tumors of the heart. A 20-year experience with a review of 12 485 consecutive autopsies.  Arch Pathol Lab Med. 1993;  117 1027-1031
• 4 Silvestri F, Bussani R, Pavletic N, Mannone T. Metastases of the heart and pericardium.  G Ital Cardiol. 1997;  27 1252-1255
• 5 Edwards F H, Hale D, Cohen A, Thompson L, Pezzella A T, Virmani R. Primary cardiac valve tumors.  Ann Thorac Surg. 1991;  52 1127-1131
• 6 Sun J P, Asher C R, Yang X S. et al . Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients.  Circulation. 2001;  103 2687-2693
• 7 Klarich K W, Enriquez-Sarano M, Gura G M, Edwards W D, Tajik A J, Seward J B. Papillary fibroelastoma: echocardiographic characteristics for diagnosis and pathologic correlation.  J Am Coll Cardiol. 1997;  30 784-790
• 8 Joffe I I, Jacobs L E, Owen A N, Ioli A, Kotler M N. Rapid development of a papillary fibroelastoma with associated thrombus: the role of transthoracic and transesophageal echocardiography.  Echocardiography. 1997;  14 287-292
• 9 Fitzgerald D, Gaffney P, Dervan P, Doyle C T, Horgan J, Nelligan M. Giant Lambl's excrescence presenting as a peripheral embolus.  Chest. 1982;  81 516-517
• 10 Grinda J M, Latremouille C, Berrebi A. et al . [Cardiac fibroelastoma. Six operated cases and review of the literature].  Arch Mal Coeur Vaiss. 2000;  93 727-732
• 11 Ni Y, von Segesser L K, Dirsch O, Schneider J, Jenni R, Turina M. Cardiac papillary fibroelastoma.  Thorac Cardiovasc Surg. 1996;  44 257-260
• 12 Boone S, Higginson L A, Walley V M. Endothelial papillary fibroelastomas arising in and around the aortic sinus, filling the ostium of the right coronary artery.  Arch Pathol Lab Med. 1992;  116 135-137
• 13 Mazzucco A, Bortolotti U, Thiene G. et al . Left ventricular papillary fibroelastoma with coronary embolization.  Eur J Cardiothorac Surg. 1989;  3 471-473
• 14 Roberts W C. Papillary fibroelastomas of the heart.  Am J Cardiol. 1997;  80 973-975
• 15 Thomas M R, Jayakrishnan A G, Desai J, Monaghan M J, Jewitt D E. Transesophageal echocardiography in the detection and surgical management of a papillary fibroelastoma of the mitral valve causing partial mitral valve obstruction.  J Am Soc Echocardiogr. 1993;  6 83-86
• 16 Kawada M, Oda K, Hirose K, Fukutomi T, Yamashiro T, Ogoshi S. [A case report of surgical treatment for left ventricular papillary fibroelastoma].  Nippon Kyobu Geka Gakkai Zasshi. 1996;  44 1159-1162
• 17 al-Mohammad A, Pambakian H, Young C. Fibroelastoma: case report and review of the literature.  Heart. 1998;  79 301-304
• 18 Lembcke A, Meyer R, Kivelitz D. et al . Images in cardiovascular medicine. Papillary fibroelastoma of the aortic valve: appearance in 64-slice spiral computed tomography, magnetic resonance imaging, and echocardiography.  Circulation. 2007;  115 e3-e6
• 19 Vander Salm T J. Unusual primary tumors of the heart.  Semin Thorac Cardiovasc Surg. 2000;  12 89-100
• 20 Kuon E, Kreplin M, Weiss W, Dahm J B. The challenge presented by right atrial myxoma.  Herz. 2004;  29 702-709
• 21 Keeling I M, Oberwalder P, Anelli-Monti M. et al . Cardiac myxomas: 24 years of experience in 49 patients.  Eur J Cardiothorac Surg. 2002;  22 971-977
• 22 Jelic J, Milicic D, Alfirevic I. et al . Cardiac myxoma: diagnostic approach, surgical treatment and follow-up. A twenty years experience.  J Cardiovasc Surg. 1996;  37 113-117
• 23 Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases.  Medicine. 2001;  80 159-172
• 24 Burke A, Virmani R. Papillary fibroelastoma: tumors of the heart and great vessels.  AFIP Atlas of Tumor Pathology. 1996;  16 47-54
• 25 Pucci A, Gagliardotto P, Zanini C, Pansini S, di Summa M, Mollo F. Histopathologic and clinical characterization of cardiac myxoma: review of 53 cases from a single institution.  Am Heart J. 2000;  140 134-138
• 26 Burke A P, Rosado-de-Christenson M, Templeton P A, Virmani R. Cardiac fibroma: clinicopathologic correlates and surgical treatment.  J Thorac Cardiovasc Surg. 1994;  108 862-870
• 27 Cho J M, Danielson G K, Puga F J. et al . Surgical resection of ventricular cardiac fibromas: early and late results.  Ann Thorac Surg. 2003;  76 1929-1934
• 28 Oostenbrug L E, Ottervanger J P, Dompeling E C, Sie T H, Beukema W P. A bleeding disorder caused by a cardiac tumor: case report.  Neth J Med. 2001;  58 232-235
• 29 Madu E, Myles J, Fraker Jr T D. Pseudopapillary fibroelastoma of the mitral valve.  J Natl Med Assoc. 1995;  87 68-70
• 30 Nair C K, Aronow W S, Sketch M H. et al . Clinical and echocardiographic characteristics of patients with mitral annular calcification. Comparison with age- and sex-matched control subjects.  Am J Cardiol. 1983;  51 992-995
• 31 Shahian D M, Labib S B, Chang G. Cardiac papillary fibroelastoma.  Ann Thorac Surg. 1995;  59 538-541
• 32 Lichtenstein H L, Lee J C, Stewart S. Papillary tumor of the heart: incidental finding at surgery.  Human Pathology. 1979;  10 473-475
• 33 Fishbein M C, Ferrans V J, Roberts W C. Endocardial papillary elastofibromas. Histologic, histochemical, and electron microscopical findings.  Arch Pathol. 1975;  99 335-341
• 34 Heath D, Best P V, Davis B T. Papilliferous tumours of the heart valves.  Br Heart J. 1961;  23 20-24
• 35 Pomerance A. Papillary “tumours” of the heart valves.  J Pathol Bacteriol. 1961;  81 135-140
• 36 Boone S A, Campagna M, Walley V M. Lambl's excrescences and papillary fibroelastomas: are they different?.  Can J Cardiol. 1992;  8 372-376
• 37 Tsukube T, Ataka K, Taniguchi T, Yokoyama M, Hanioka K. Papillary fibroelastoma of the left atrial appendage: echocardiographic findings.  Ann Thorac Surg. 2000;  70 1416-1417
• 38 Cha S D, Incarvito J, Fernandez J, Chang K S, Maranhao V, Gooch A S. Giant Lambl's excrescences of papillary muscle and aortic valve: echocardiographic, angiographic, and pathologic findings.  Clin Cardiol. 1981;  4 51-54
• 39 de Menezes I C, Fragata J, Martins F M. Papillary fibroelastoma of the mitral valve in a 3-year-old child: case report.  Pediatr Cardiol. 1996;  17 194-195
• 40 Espada R, Talwalker N G, Wilcox G, Kleiman N S, Verani M S. Visualization of ventricular fibroelastoma with a video-assisted thoracoscope.  Ann Thorac Surg. 1997;  63 221-223
• 41 Rona G, Feeney N, Kahn D S. Fibroelastic hamartoma of the aortic valve producing ischemic heart disease. Associated pulmonary glomus bodies.  Am J Cardiol. 1963;  12 869-874
• 42 Gunter C, Jenni R. [What is your diagnosis? Papillary fibroelastoma of the right coronary aortic sinus - embolism with 2 transient cerebral ischemia attacks].  Schweiz Rundsch Med Prax. 1998;  87 1009-1011
• 43 Prahlow J A, Barnard J J. Sudden death due to obstruction of coronary artery ostium by aortic valve papillary fibroelastoma.  Am J Forensic Med Pathol. 1998;  19 162-165
• 44 Rieckmann C, Liebau G, Hellberg K, Seeliger H. [Papillary fibroelastoma of the aortic valve: primary echocardiography diagnosis in an asymptomatic patient].  Z Kardiol. 1990;  79 208-210
• 45 Pede S, Vergallo L, Pico C, Renna A, Faggiano C, Faggiano F. [Papillomatosis of the mitral valve as an unusual cause of myocardial ischemia. Description of a case and review of the literature].  G Ital Cardiol. 1988;  18 76-79
• 46 Bossert T, Diegeler A, Spyrantis N, Mohr F W. Papillary fibroelastoma of the aortic valve with temporary occlusion of the left coronary ostium.  J Heart Valve Dis. 2000;  9 842-843
• 47 Howard R A, Aldea G S, Shapira O M, Kasznica J M, Davidoff R. Papillary fibroelastoma: increasing recognition of a surgical disease.  Ann Thorac Surg. 1999;  68 1881-1885
• 48 Nakao T, Hollinger I, Attai L, Oka Y. Incidental finding of papillary fibroelastoma on the atrial septum.  Cardiovasc Surg. 1994;  2 423-424
• 49 Watchell M, Heritage D W, Pastore L, Rhee J. Cytogenetic study of cardiac papillary fibroelastoma.  Cancer Genet Cytogenet. 2000;  120 174-175
• 50 Schuetz W H, Welz A, Heymer B. A symptomatic papillary fibroelastoma of the left ventricle removed with the aid of transesophageal echocardiography.  Thorac Cardiovasc Surg. 1993;  41 258-260
• 51 Lee K S, Topol E J, Stewart W J. Atypical presentation of papillary fibroelastoma mimicking multiple vegetations in suspected subacute bacterial endocarditis.  Am Heart J. 1993;  125 1443-1445
• 52 Schiller A L, Schantz A. Papillary endocardial excrescence of the right atrium: report of two cases.  Am J Clin Pathol. 1970;  53 617-621
• 53 Gallas M T, Reardon M J, Reardon P R, DeFelice C A, Raizner A E, Mody D R. Papillary fibroelastoma. A right atrial presentation.  Tex Heart Inst J. 1993;  20 293-295
• 54 Bapat V N, Varma G G, Hordikar A A, Sivaraman A, Agrawal N B, Tendolkar A G. Right-ventricular fibroma presenting as tricuspid stenosis - a case report.  Thorac Cardiovasc Surg. 1996;  44 152-154
• 55 Uchida S, Obayashi N, Yamanari H, Matsubara K, Saito D, Haraoka S. Papillary fibroelastoma in the left ventricular outflow tract.  Heart Vessels. 1992;  7 164-167
• 56 Wasdahl D A, Wasdahl W A, Edwards W D. Fibroelastic papilloma arising in a Chiari network.  Clin Cardiol. 1992;  15 45-47
• 57 Shigemitsu O, Hadama T, Mori Y, Miyamoto S, Sako H, Uchida Y. [Surgical treatment of right atrial papillary fibroelastoma, originated from the Eustachian valve - a case report].  Nippon Kyobu Geka Gakkai Zasshi. 1995;  43 403-406
• 58 Schwinger M E, Katz E, Rotterdam H, Slater J, Weiss E C, Kronzon I. Right atrial papillary fibroelastoma: diagnosis by transthoracic and transesophageal echocardiography and percutaneous transvenous biopsy.  Am Heart J. 1989;  118 1047-1050
• 59 Anderson K R, Fiddler G I, Lie J T. Congenital papillary tumor of the tricuspid valve. An unusual cause of right ventricular outflow obstruction in a neonate with trisomy E.  Mayo Clin Proc. 1977;  52 665-669
• 60 Kondo T, Tobe S. Images in clinical medicine. Papillary fibroelastoma of the pulmonary artery.  N Engl J Med. 2007;  356 e1
• 61 McAllister Jr H A, Fengolio Jr J J. Tumors of the cardiovascular system. Rosai J, Sobin LH Atlas of Tumor Pathology. 2nd series. Washington DC; Armed Forces Institute of Pathology 1978: 20-25
• 62 Gowda R M, Khan I A, Nair C K, Mehta N J, Vasavada B C, Sacchi T J. Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases.  Am Heart J. 2003;  146 404-410
• 63 Alawi A, Kassabian E B, Ashoush R, Jebara V A. Aortic valve papillary fibroelastoma.  Cardiovasc Surg. 2002;  10 65-67
• 64 Sydow K, Schrepfer S, Franzen O. et al . Coincidence of aortic valve stenosis and regurgitation and multiple cardiac papillary fibroelastomas in a young male adult.  J Thorac Cardiovasc Surg. 2007;  133 564-565
• 65 Touati G D, Carmi D, Sevestre H, Poulain H. Multiple aortic valve papillary fibroelastoma: do not miss the other one.  Eur J Cardiothorac Surg. 2002;  21 596-597
• 66 Kanarek S E, Wright P, Liu J. et al . Multiple fibroelastomas: a case report and review of the literature.  J Am Soc Echocardiogr. 2003;  16 373-376
• 67 Eslami-Varzaneh F, Brun E A, Sears-Rogan P. An unusual case of multiple papillary fibroelastoma, review of literature.  Cardiovasc Pathol. 2003;  12 170-173
• 68 Giannesini C, Kubis N, N'Guyen A, Wassef M, Mikol J, Woimant F. Cardiac papillary fibroelastoma: a rare cause of ischemic stroke in the young.  Cerebrovasc Dis. 1999;  9 45-49
• 69 Colucci V, Alberti A, Bonacina E, Gordini V. Papillary fibroelastoma of the mitral valve. A rare cause of embolic events.  Tex Heart Inst J. 1995;  22 327-331
• 70 Grinda J M, Couetil J P, Chauvaud S. et al . Cardiac valve papillary fibroelastoma: surgical excision for revealed or potential embolization.  J Thorac Cardiovasc Surg. 1999;  117 106-110
• 71 Grandmougin D, Fayad G, Moukassa D. et al . Cardiac valve papillary fibroelastomas: clinical, histological and immunohistochemical studies and a physiopathogenic hypothesis.  J Heart Valve Dis. 2000;  9 832-841
• 72 Ragni T, Grande A M, Cappuccio G. et al . Embolizing fibroelastoma of the aortic valve.  Cardiovasc Surg. 1994;  2 639-641
• 73 Fiorilli R, Tomasco B, De Sanctis D, Tesler U F. Aortic valve fibroelastoma: transesophageal echocardiographic diagnosis and surgical excision.  G Ital Cardiol. 1998;  28 153-157
• 74 Ngaage D L, Mullany C J, Daly R C. et al . Surgical treatment of cardiac papillary fibroelastoma: a single center experience with eighty-eight patients.  Ann Thorac Surg. 2005;  80 1712-1718
• 75 Scalia D, Basso C, Rizzoli G. et al . Should right-sided fibroelastomas be operated upon?.  J Heart Valve Dis. 1997;  6 647-650
• 76 Pinelli G, Carteaux J P, Mertes P M, Civit T, Trinh A, Villemot J P. Mitral valve tumor revealed by stroke.  J Heart Valve Dis. 1995;  4 199-201
• 77 Sastre-Garriga J, Molina C, Montaner J. et al . Mitral papillary fibroelastoma as a cause of cardiogenic embolic stroke: report of two cases and review of the literature.  Eur J Neurol. 2000;  7 449-453