Papillary fibroelastoma

A papillary fibroelastoma is a primary tumor of the heart that typically involves one of the valves of the heart. Papillary fibroelastomas, while considered generally rare, make up about 10 percent of all primary tumors of the heart.cite journal | author=Palecek T, Lindner J, Vitkova I, Linhart A. | title=Papillary Fibroelastoma Arising from the Left Ventricular Apex Associated with Nonspecific Systemic Symptoms. | journal=Echocardiography | year=2008 | pmid=18177380 | doi=10.1111/j.1540-8175.2007.00617.x | volume=25 | pages=526] They are the third most common type of primary tumor of the heart,cite journal | author=Matsumoto N, Sato Y, Kusama J, Matsuo S, Kinukawa N, Kunimasa T, Ichiyama I, Takahashi H, Kimura S, Orime Y, Saito S. | title=Multiple papillary fibroelastomas of the aortic valve: case report. | journal=Int J Cardiol | year=2007 | volume=122 | issue=1 | pages=e1–3 | pmid=17196273 | doi=10.1016/j.ijcard.2006.11.026] behind cardiac myxomas and cardiac fibromas.

ymptoms

A papillary fibroelastoma is generally considered benign, however can be associated with syncopecite journal | author=Maestroni A, Zecca B, Triggiani M | title=Cardiac papillary fibroelastoma presenting with acute coronary syndrome and syncope. | journal=Acta Cardiol | year=2006 | volume=61 | issue=3 | pages=363–5 | pmid=16869462 | doi=10.2143/AC.61.3.2014843] , chest pain, heart attack, strokecite journal | author=Liebeskind DS, Buljubasic N, Saver JL. | title=Cardioembolic stroke due to papillary fibroelastoma. | journal=J Stroke Cerebrovasc Dis | year=2001 | volume=10 | issue=2 | pages=94–5 | pmid=17903807 | doi=10.1053/jscd.2001.24654] and sudden cardiac death.

Symptoms due to papillary fibroelastomas are generally due to either mechanical effects of the tumor or due to embolization of a portion of the tumor to a distal organ. In particular, chest pain or syncope may be due to transient occlusion of the left main coronary artery by the tumor, while a heart attack or sudden cardiac death may be due to embolization of a portion of the tumor into a coronary artery.cite journal | author=Takada A, Saito K, Ro A, Tokudome S, Murai T. | title= Papillary fibroelastoma of the aortic valve: a sudden death case of coronary embolism with myocardial infarction.| journal=Forensic Sci Int | year=2000 | volume=113 | issue=1-3 | pages=209–14 | pmid=10978627 | doi= 10.1016/S0379-0738(00)00207-3]

Treatment

If the tumor is found incidentally in an asymptomatic person, the treatment approach is controversial. Certainly a conservative approach is warranted in certain individuals.cite journal | author=Mutlu H, Demir IE, Leppo J, Levy WK. | title=Nonsurgical Management of a Left Ventricular Pedunculated Papillary Fibroelastoma: A Case Report. | journal=J Am Soc Echocardiogr | year=2008 | pmid=18191538] If the tumor is large and pedunculated, a case may be made for surgical excision prior to symptoms developing due to the higher risk of embolism. However, this is still considered controversial.cite journal | author=Boodhwani M, Veinot JP, Hendry PJ. | title=Surgical approach to cardiac papillary fibroelastomas. | journal=Can J Cardiol | year=2007 | volume=23 | issue=4 | pages=301–2 | pmid=17380224]

If the papillary fibroelastoma is associated with symptoms, surgical excision is generally recommended for relief of symptoms. A minimally invasive approach may be possible if the tumor involves the aortic valvecite journal | author=Hsu VM, Atluri P, Keane MG, Woo YJ. | title=Minimally invasive aortic valve papillary fibroelastoma resection. | journal=Interact Cardiovasc Thorac Surg | year=2006 | volume=5 | issue=6 | pages=779–81 | pmid=17670711 | doi=10.1510/icvts.2006.133702] or right atrium.cite journal | author=Kim RW, Jeffery ME, Smith MJ, Wilensky RL, Woo EY, Woo YJ. | title=Minimally invasive resection of papillary fibroelastoma in a high-risk patient. | journal=J Cardiovasc Med (Hagerstown) | year=2007 | volume=8 | issue=8 | pages=639–41 | pmid=17667039] In the case of aortic valve involvement, excision of the tumor is often valve-sparing, meaning that replacement of the valve with a prosthetic valve is not necessary. Repair of the native valve with a pericardial patch has been described.cite journal | author=Westhof FB, Chryssagis K, Liangos A, Batz G, Diegeler A. | title=Aortic valve leaflet reconstruction after excision of a papillary fibroelastoma using autologous pericardium. | journal=Thorac Cardiovasc Surg | year=2007 | volume=55 | issue=3 | pages=204–7 | pmid=17410513 | doi=10.1055/s-2006-924439]

References