|Home | About | Journals | Submit | Contact Us | Français|
Diagnostic evaluation of embolic neurologic events requires the consideration of cardiac causes. We recently encountered a case that emphasizes the importance of cardiac tumor as a source of embolic events. We present herein the case of a 42-year-old woman who suffered a transient ischemic attack caused by a papillary fibroelastoma that originated from the aortic valve.
Diagnostic evaluation of embolic neurologic events requires the consideration of cardiac causes. We recently encountered a case that emphasizes the importance of cardiac tumor as a source of embolic events. Primary cardiac tumors are rare: their prevalence in a series of multiple autopsies1 was only 0.02%. Cardiac papillary fibroelastoma, the 2nd most common primary benign cardiac tumor after cardiac myxoma, accounts for 10% of all cardiac tumors and is the most common tumor of the heart valves. Papillary fibroelastoma occurs slightly more often in men than in women (55% vs 45%), and the mean age of patients is 60 years.2 Although some authors associate papillary fibroelastoma with an underlying risk of heart disease, no risk factors have been established, and the histogenesis of these tumors remains a controversial topic.3,4 Concomitant valvular dysfunction is attributed only rarely to these tumors.5 Clinical manifestations of papillary fibroelastoma include cerebrovascular accidents, myocardial infarction, pulmonary embolism, congestive heart failure, and sudden cardiac death. Most patients, however, remain asymptomatic or display symptoms incidental to the tumor itself.2,3
In July 2006, a 42-year-old woman was referred to our department for evaluation and surgical treatment of an aortic valve tumor. The tumor was discovered with the aid of transthoracic echocardiography (TTE) during a diagnostic evaluation after a transient ischemic attack (TIA) that had been characterized by left-sided weakness. The patient's medical history was unremarkable, except for the recent TIA.
Upon admission to our cardiovascular surgery service, the patient had no symptoms of heart failure. Preoperative transesophageal echocardiography (TEE) confirmed the presence of a mobile mass (3–4 mm in diameter) on the leading edge of the right coronary cusp (Fig. 1). The aortic valve appeared to be structurally normal. The patient's left ventricular ejection fraction was 0.53.
The patient was taken to the operating room, where cardiopulmonary bypass was initiated after ascending aortic and central venous cannulation. A transverse aortotomy was performed. The 3-mm papillary fibroelastoma was then excised from the leaflet edge of the right coronary cusp, which spared the aortic valve. The other leaflets were carefully inspected, and no other tumors were found. Postoperative TEE showed no evidence of aortic stenosis or regurgitation (Fig. 2). Subsequent histopathologic examination confirmed the diagnosis of a benign papillary fibroelastoma of the aortic valve.
The patient was discharged from the hospital with no neurologic or cardiovascular symptoms.
The case illustrates the importance of echocardiography in considering a cardiac source, such as papillary fibroelastoma, as the cause of unexplained embolic events.4 The medical literature describes several cases of recurrent cerebrovascular accidents associated with permanent neurologic damage, the source of which was recurrent embolization from papillary fibroelastoma.6 Even very small papillary fibroelastomas (in our patient's case, 3 mm in diameter) can lead to neurologic damage.7,8 The transient nature of our patient's cerebrovascular accident makes her case highly unusual.
Transthoracic echocardiography and TEE have proved to be sensitive tools for the diagnosis of rare cardiac tumors, and the echocardiographic characteristics of each variety of tumor have been established.5
According to Sun and colleagues,4 sensitivity in the detection of papillary fibroelastomas is 61.9% for TTE and 76.6% for TEE; sensitivity increases considerably when tumors smaller than 2 mm are excluded from the study (88.9% for TTE). Papillary fibroelastomas are usually mobile and are attached to the endocardium by a stalk. Findings in histologic examination of these tumors include a matrix consisting of mucopolysaccharides, varying layers of elastic fibers, and rare spindle cells covered by a single hyperplastic endothelial layer.3
Papillary fibroelastomas occur predominantly on valvular surfaces (77%)—most often the aortic valve (44%), followed by the mitral valve (35%), the tricuspid valve (15%), and the pulmonary valve (8%). More than 95% of these tumors are found in the left side of the heart, and most of them are small (< 1 cm) and solitary. However, there have been reports of patients with multiple tumors at various locations within the heart, which emphasizes the need for careful intraoperative inspection.2,4,9
In the patient described herein, the tumor was excised using a valve-sparing technique, which is the treatment of choice in symptomatic patients. This technique yields excellent outcomes and is usually curative, although we recommend annual follow-up of these patients to check for reoccurrence. If the valve has been mechanically compromised, however, repair or replacement may be necessary. Thorough inspection of the other leaflets and valves should be undertaken to rule out multiple tumors. In symptomatic patients who have surgical contraindications, anticoagulation therapy may be an option.2
Address for reprints: Mehmet H. Akay, MD, 2475 Underwood, Apt. #172, Houston, TX 77025. E-mail: moc.liamtoh@yakahm