Cardiac papillary fibroelastoma is classified as a rare, benign primary cardiac tumor accounting for approximately 10% of all cardiac tumors[2]. The etiology of this tumor is still unclear. There are many hypotheses of the original mechanism including neoplasms, hamartomas, organized thrombi, and unusual endocardial responses to infection or hemodynamic trauma[3-5]. The histological feature is described as an inner vascular core of connective tissue covered by endothelium that surrounds a layer of acid mucopolysaccaride, which is often with a peduncule or stalk, resembling a sea anemone[6].
As a past report presented, cardiac papillary fibroelastoma can be found anywhere inside the heart such as valvular surfaces, papillary muscles, chordae tendinease, ventricular septum or endocardial surface[7]. Frequently, it appears on aortic valve and mitral valve, especially on the aortic valve[7, 8].The certain location of the tumor may be associated with the occurrence of the complications.
Although most patients are asymptomatic, serious complications have been reported, such as neural symptoms, angina, myocardial ischemia, distal embolic events[9-12].The mechanism is thought to be intermittent obstruction of the coronary ostia or valve by the mobile tumor mass and embolization of tumor mass or platelet thrombi on the tumor into the coronary arteries or distal arteries[13].It can also cause a sudden death by occlusion of the coronary artery reported through an autopsy[14].As a result, the tumor will bring potential risks in to patients who have yet been symptomatic.
The diagnosis in living patients was rare before the introduction of echocardiography[15]. With the development of radiological technology, computed tomography scans(CT) or magnetic resonance imaging(MRI) has been introduced a limited use of diagnosing this tumor[16, 17]. Above all these techniques, echocardiography has been defined as a convenient and noninvasive way to diagnose and should be the first choice of tests to search for cardiac papillary fibroelastoma which can be demonstrated as a pedunculated, mobile and echodense mass. And the use of transoesophageal echocardiography (TEE) provides a more visualized image to identify the location and guide the excision. CT and MRI can be supplements of echocardiography.
Surgical excision is the only therapy of cardiac papillary fibroelastoma[18].For those symptomatic patients, excision is recommended regardless of the size. For those asymptomatic patients, mobile lesions or lesions in the specific locations like coronary ostia also should be surgically excised because of the higher risk of obstructive and thromboembolic complications. And a valvuloplasty or a valve replacement often needs to be performed after cutting of the tumor. Other patients with nonmobile lesions less than 1 cm in diameter can be managed expectantly or with anticoagulation. In addition, recurrence of PFE is very low[6].
In this case reported here, the patient presented with angina and was admitted by the primary diagnosis of acute coronary syndrome and paroxysmal atrial fibrillation. But the coronary angiogram was normal, while the definite symptom, electrocardiogram, enzymatic test suggested there was a myocardial infarction on this patient. Following tests of echocardiography and computed tomography scans (CT) both revealed a mass on the left coronary cusp margin of the aortic valve. With the evidence of persistent symptom and aortic valvular mass, surgical excision was performed. Intraoperative findings identified the location of this mass. The histological test confirmed the mass was cardiac papillary fibroelastoma. According to other cases reported, embolization of tumor fragments or thrombotic material could not be excluded from the potential mechanism of myocardial infarction, but it is more possible that the tumor may block the coronary ostia causing the coronary ischemia. To our knowledge this might be the most comprehensive case of cardiac papillary fibroelastoma, so we have meticulously recorded the data of primary symptoms, enzymatic test, electrocardiogram, angiogram, echocardiogram, CT and histological test included before and after the operation. It is our belief that these might be more helpful in diagnosis and treatment of the tumor.