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Interact CardioVasc Thorac Surg 2009;9:921-922. doi:10.1510/icvts.2009.212274 © 2009 European Association of Cardio-Thoracic Surgery
Multiple papillary fibroelastoma in left ventricle associated with obstructive hypertrophic cardiomyopathyDepartment of Cardiovascular Surgery, Tokyo Woman's Medical University, Tokyo, Japan Received 21 May 2009; received in revised form 28 July 2009; accepted 29 July 2009
*Corresponding author. Tokyo Woman's Medical University, 8-1, Kawada-cyo, Shinjuku-ku, Tokyo 162-8666, Japan. Tel.: +81-3-33538111; fax: +81-3-33560441.
A 68-year-old man was referred to our hospital with a left ventricular (LV) mass and obstructive hypertrophic cardiomyopathy (HOCM). Although he was treated for the LV thrombosis and received anticoagulation therapy, the mass did not decrease in size for three years. His past history was colon cancer which was resected in its early phase. Laboratory studies revealed the absence of any inflammatory and tumor marker symptoms. Transthoracic and transesophageal echocardiography revealed a mass of 24x11 mm in diameter attached to the septal wall of LV and another two or three masses detected in LV wall. He received surgical treatment with complete mass excision with LV dissection and hypertrophied ventricular muscle was resected. Surgical resection of these LV masses and septal myectomy was performed. The histological examination showed that the lesions had a papillary configuration with an avascular connective tissue core lined by a single layer of endothelial cells, which was sufficient for a diagnosis of cardiac papillary fibroelastoma (CPF). The patient recovered without any complications.
Key Words: Papillary fibroelastoma; Obstructive hypertrophic cardiomyopathy; Cardiac tumor
The incidence of primary cardiac tumors is low at 0.002–0.33%. Cardiac papillary fibroelastoma (CPF) is a rare primary tumor that accounts for 8% of all cardiac tumors, and is the third most common benign cardiac tumor after myxoma and lipoma [1, 2]. As rare as these tumors are, the incidence of multiple fibroelastoma in left ventricle (LV) is even rarer [3–5]. We describe an unusual case of multiple CPFs of the LV. A 68-year-old man was referred to our hospital from an outside facility with a LV mass in 2006, and he had no symptom. Since 2004, he was treated for the LV thrombosis and received anticoagulation therapy; the mass did not decrease in size for three years. His past history was colon cancer which was resected in its early phase. By transthoracic and transesophageal echocardiography, a round and cystic mass of 24x11 mm in diameter was found attached to the septal wall of LV and another two or three masses detected in LV wall (Fig. 1). Valve function and LV wall motion was good. LV outflow tract was stenosis by septal wall thickness. A ventricular wall was thickened, accelerated blood flow in LV outflow tract was 4.8 m/s, and it was diagnosed as the obstructive hypertrophic cardiomyopathy (HOCM). Scintigraphy and computed tomography showed no evidence of another tumor and embolism. Laboratory studies revealed the absence of any inflammatory and tumor marker symptoms. Catheter angiography showed that there was no feeding artery for this tumor and coronary artery was intact. Result of physical examination and laboratory profile concluded tumor markers were unremarkable. Surgical excision was recommended in consideration of the known potential for coronary, cerebral, and systemic embolization.
The patient was brought to the operating room. Bicaval and ascending aorta cannulation were accomplished and total cardiopulmonary bypass was initiated. The stenosis of LV outflow tract made the transaortic approach less desirable. Transmitral valve approach was not adequate because there were masses at the back of the mitral valve. A ventricular incision was chosen. On opening the LV, there were masses attached to the septal wall of LV and another three masses detected in LV wall. A pedicled mass with delicate papillary fronds resembling a sea anemone was seen arising from the endocardial surface at the LV septal wall (Fig. 2). Resection of these masses and septal myectomy was performed. Intraoperative transesophageal echocardiography demonstrated no residual mass and accelerated blood flow in LV outflow tract had decreased to 2.8 m/s.
The histological examination showed that the lesions had a papillary configuration with an avascular connective tissue core lined by a single layer of endothelial cells, which was sufficient for a diagnosis of CPF. The postoperative course was uncomplicated.
CPF is a rare primary tumor that accounts for 8% of all cardiac tumors, and is the third most common benign cardiac tumor after myxoma and lipoma [1, 2]. Between 77% and 90% of CPFs appear on cardiac valves, and the remainder have been reported to originate from nearly all portions of the non-valvular endocardium including the LV septum, the LV mural endocardium, the right ventricular outflow tract, the right atrium, the atrial septum, the papillary muscles, the chordae tendinea, the eustachian valve, and the Chiari network. Multiple CPF were detected in 8% [3]. Despite their small size, CPFs have been reported to cause angina and sudden death from either coronary emboli or direct ostial occlusion by prolapsing tumor. Although CPFs are considered benign tumors, their clinical course is deceptive. CPFs should be resected even in the patients who have no symptoms. It is often avoided by the LV resection with its potential complications; bleeding, arrhythmia and declined LV function. However, a ventricular incision was very useful for this patient complicated with HOCM and multiple masses because all masses could be observed and aberrant cardiac muscle could be resected well.
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Abstract
1. Introduction
