Coinciding anomalous coronary artery and papillary fibroelastoma

The primary cardiac tumors are extremely rare tumors that arise from the normal cardiac tissues. There are benign variants (mostly myxoma) and malignant tumors. Fibroelastoma (FE) is the most common primary tumor, of which myxoma is the most common subtype followed by papillary fibroelastomas (PFE). They account for the majority of primary heart valvular tumors that mainly involve the left (mitral and aortic) valves, and only few cases reported pulmonary valve PFE [1]. We present a case of 61 year old female who had incidental pulmonary valve papillary fibroelastoma finding on echo along with anomalous coronary arteries.     

Incidental Papillary Fibroelastoma Multimodal: Imaging and Surgical Decisions in 2 Patients

Papillary fibroelastoma is a rare, benign cardiac tumor typically found on the heart valves. It is usually discovered incidentally on echocardiography. The clinical presentation of cardiac papillary fibroelastoma varies from no symptoms to severe embolic sequelae. We report the incidental finding of papillary fibroelastoma in 2 patients. In each, we chose to excise the tumor. The relevant medical literature provides little guidance regarding whether to excise a small papillary fibroelastoma in an asymptomatic patient. Multimodal imaging, which we discuss in the context of our patients'' cases, aids the cardiologist and cardiovascular surgeon in more accurately evaluating papillary fibroelastoma preoperatively.Key words: Chordae tendineae/pathology/ultrasonography, echocardiography, fibroma/epidemiology/pathology/surgery, heart neoplasms/diagnosis/epidemiology/pathology/surgery/ultrasonography, heart valves/pathology, incidental findings, tomography, x-ray computedPapillary fibroelastoma is the third most common primary tumor, and it usually involves the cardiac valves. It can be asymptomatic, or it can cause major thromboembolic sequelae. Factors in deciding to proceed with surgery can be complex, because of difficulties in evaluating the tumor and predicting the patient''s prognosis. Multimodal imaging enables a more accurate evaluation of papillary fibroelastoma. We report our incidental discovery of papillary fibroelastoma in 2 patients, discuss the role of multimodal imaging in our surgical decisions, and review ideas for managing these tumors in asymptomatic patients.     

Papillary Fibroelastoma of the Aortic Valve as a Cause of Transient Ischemic Attack

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.Key words: Aortic valve/pathology, echocardiography, transesophageal, heart neoplasms/pathology, heart valve diseases/ultrasonography, heart valves/pathology, fibroma/complications/diagnosis/therapy/ultrasonography, transient ischemic attackDiagnostic 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     

Embolic stroke and cardiac papillary fibroelastoma

Papillary fibroelastomas are rare, benign, primary cardiac tumors. They are, however, the most common primary tumor of the cardiac valves and may cause great morbidity risk from embolization. This paper reports the case of a healthy 34-year-old man who presented with symptoms of a right occipital embolic stroke. Transesophageal echocardiography revealed a papillary fibroelastoma on the anterior leaflet of the mitral valve. The papillary fibroelastoma was surgically excised and he has had no recurrent symptoms. This case illustrates the importance of obtaining a transesophageal echocardiogram to investigate a possible embolic source in patients with ischemic stroke.     

Papillary fibroelastoma of the pulmonary valve

A papillary fibroelastoma is rare, but it is the most common primary tumor of the cardiac valves. Most papillary fibroelastomas affect the left-sided heart valves, such as the aortic and mitral valves; however, they also rarely affect the pulmonary valve. Generally, surgical removal is strongly recommended to prevent its potential thromboembolic risks, especially in cases of left-sided cardiac involvement. However, there are few reports on the treatment of asymptomatic, small, right-sided cardiac fibroelastomas. We present a rare case of an asymptomatic papillary fibroelastoma occurring on the pulmonary valve, which was detected by transthoracic echocardiography, transesophageal echocardiography, and a 64-slice cardiac computed tomography scan, and which was surgically removed.     

Papillary Fibroelastoma of the Interventricular Septum Mimicking a Cardiac Myxoma

Papillary fibroelastomas are benign cardiac tumors that involve cardiac valves. These tumors are usually asymptomatic and are found incidentally during cardiac surgery or during echocardiographic evaluation in a patient who exhibits cardiac symptoms. However, these tumors may cause major thromboembolic complications. Herein, we describe the surgical management of a patient who had an unusual appearance and location of a papillary fibroelastoma that was attached to the interventricular septum.Key words: Fibroelastoma/diagnosis/pathology/surgery, heart neoplasms/pathology/surgery, treatment outcomePapillary fibroelastoma (PFE) is a rare and benign cardiac tumor that usually involves cardiac valves. It may cause major thromboembolic complications. The tumor is diagnosed more commonly today by virtue of advances in medical imaging than by evaluation of symptoms, as in the past. Here, we describe a patient who had an unusual appearance and location of a PFE that was treated surgically.     

Papillary fibroelastoma of the left ventricle: report of two cases

Papillary fibroelastoma is a relatively rare cardiac tumor. We report two cases of papillary fibroelastoma. The first case involved a 45-year-old woman who presented with rheumatic valves and three tumors developing from the papillary muscle and left ventricle. The second case involved a 68-year-old man who was asymptomatic and whose tumor was detected incidentally on echocardiogram. Both cases were treated surgically. An additional 71 cases of papillary fibroelastoma reported in the medical literature in Japan are reviewed.     

Papillary fibroelastomas of the heart

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.     

心脏瓣膜原发性肿瘤和瘤样病变的临床病理分析

目的分析原发于心脏瓣膜的肿瘤和瘤样病变的病理特征.方法从384例心脏原发肿瘤中收集瓣膜肿瘤的病理资料,加做免疫组化染色,分析其病理特征和临床意义.结果共检出瓣膜肿瘤和瘤样病变10例,占心脏肿瘤的2.60%.8例位于二尖瓣,1例三尖瓣,1例肺动脉瓣.病理类型有乳头状纤维弹力瘤1例,再发粘液瘤1例,脂肪瘤1例,畸胎瘤1例,海绵状血管瘤1例,钙化性假瘤2例,血囊肿1例,间叶肉瘤2例.肿物直径0.3 cm～3 cm,外观形态多样.临床可出现心力衰竭、偏瘫和晕厥.肿瘤的外形及有无瘤蒂不是良恶性的鉴别点.2例恶性肿瘤临床误诊.10例中7例外科做了瓣膜置换术.结论瓣膜肿瘤较易影响瓣膜功能,且易脱落引起栓塞,建议临床一旦发现应及时手术.由于其良恶性肉眼较难判别,术中冰冻切片检查对临床处理有较大的参考价值.     

Echocardiographic diagnosis of papillary fibroelastoma of the mitral and tricuspid valve apparatus

Papillary fibroelastomas are rare and normally benign cardiac tumors typically attached to cardiac valves. This report describes two patients who were evaluated for intermittent dyspnea in one case and for the source of cerebral embolism in the other. In both patients transthoracic echocardiography revealed a pedunculated mobile mass adjacent to an atrioventricular valve, suggestive of papillary fibroelastoma. Postoperative histology was confirmatory of papillary fibroelastoma with a typical hyalinized hypocellular stroma covered by a single layer of endocardial cells.     

Papillary Fibroelastoma in Differential Diagnosis of Left Atrial Appendage Masses

Papillary fibroelastomas are benign tumors that usually originate from cardiac valves but may have other endocardial origins. We report the cases of 2 patients in whom left atrial appendage masses were initially diagnosed as thrombus. They were treated for embolic stroke and their symptoms resolved; however, their left atrial appendage masses did not regress. After surgery, histologic analysis of the resected masses revealed papillary fibroelastoma in both cases. We discuss the diagnostic and therapeutic dilemmas encountered in patients with papillary fibroelastomas and cardiac masses other than thrombus.     

A rare cause of sudden vision loss in a young male: papillary fibroelastoma of the aortic valve

Cardiac papillary fibroelastoma (CPF) is a rare cardiac neoplasm predominantly affecting the cardiac valves. Although it is most commonly an incidental finding, it can result in life-threatening complications, such as coronary and cerebral embolism, acute valvular dysfunction, and sudden death. In this report, we describe a case of a patient with aortic valve papillary fibroelastoma, which presented with sudden vision loss due to branch retinal artery occlusion. To the best of our knowledge, this is the third case of CPF-related retinal artery embolism in English literature.     

Left Main Coronary Artery Embolus: Unusual Presentation of Papillary Fibroelastoma of the Aortic Valve

The usual cause of left main coronary artery obstruction is atherosclerotic occlusion resulting from plaque rupture and subsequent thrombus formation. In previously reported cases, tumor embolization into the coronary arteries caused sudden death and was detected only at autopsy. Herein, we report an unusual presentation of cardiac papillary fibroelastoma of the aortic valve in a 62-year-old man. The fibroelastoma caused a left main coronary artery embolus and symptoms of acute coronary syndrome. The fibroelastoma was successfully excised during a valve-sparing surgical procedure. We believe that this is the 1st report of tumor embolization to the left main coronary artery—and in a living patient.Key words: Aortic valve/pathology/surgery, coronary disease/etiology, fibroma/complications/diagnosis/pathology/surgery/ultrasonography, heart neoplasms/physiopathology/surgery, heart valve diseases/pathology, incidental findings, papilloma/pathology, treatment outcomePapillary fibroelastomas are histologically benign neoplasms. These avascular tumors are small (mean diameter, 3–10 mm) and almost always occur singly. Often, they are mobile and have a thin stalk.¹ They have multiple papillary fronds, so that they resemble sea anemones. Papillary fibroelastomas consist of a core of dense connective tissue, an intermediate layer of loose connective tissue, and a covering of hyperplastic endothelial cells. Although cardiac papillary fibroelastoma is rare, it is the 2nd most common primary benign cardiac tumor (myxoma has been reported more often),² and it is the most common primary tumor of the heart valves. As with myxoma, its origin is not clear. Papillary fibroelastomas can develop on any cardiac valve or cardiac endothelial surface.³ Most are diagnosed incidentally.Clinical manifestations of cardiac papillary fibroelastomas include syncope, angina pectoris, transient ischemic attack, stroke, myocardial infarction, pulmonary embolism, congestive heart failure, and sudden death. Coronary ischemia has been caused by the prolapse of pedunculated coronary cusp tumors into the coronary ostia,⁴ and, alternatively, by the direct embolization of organized thrombus from a cusp lesion to a coronary artery.^3,5 In previously reported cases,^5-7 tumor embolization into the coronary arteries caused sudden death and was detected only at autopsy. Here, we present the case of a patient who presented with symptoms of acute coronary syndrome. The diagnosis was a left main coronary artery (LMCA) embolus from a cardiac papillary fibroelastoma.     

Cardiac papillary fibroelastoma originating from pulmonary vein--a case report

Cardiac papillary fibroelastoma is a primary cardiac neoplasm that typically affects the cardiac valves, mainly the aortic and mitral valves, and very rarely the endocardium of cardiac chambers. Cardiac papillary fibroelastoma is rarely diagnosed during life, as the majority are incidental findings at autopsy, but with the advent of echocardiography, it is being increasingly recognized. Although the tumor is usually small and histologically benign, it may have a malignant propensity for life-threatening complications, such as a cerebrovascular accident, myocardial ischemia or infarction, or sudden death. The patient reported here presented with an embolic stroke from a thrombus on the surface of a left atrial papillary fibroelastoma. The papillary fibroelastoma was originating from the lower portion of the left inferior pulmonary vein and was protruding into the left atrial cavity. Papillary fibroelastoma originating from the pulmonary veins has not been reported before. The tumor was successfully removed by intraoperative transesophageal echocardiography-guided cardiac surgery. Grossly, the surface of the tumor was smooth and translucent. The gelatinous membrane on the surface tore easily, and soft papillary tumor with multiple fronds was visible. Histology confirmed the mass was a papillary fibroelastoma. Postoperative recovery was uneventful. Follow-up transthoracic echocardiogram revealed no residual or recurrence of tumor. The patient was in excellent health at 2-year follow-up. The case is described and the clinical characteristics of cardiac papillary fibroelastoma are reviewed.     

Papillary Fibroelastoma of the Aortic Valve: Operative Approaches upon Incidental Discovery

Papillary fibroelastomas are the most common benign neoplasms of the cardiac valvular structures, and they are being recognized more frequently because of higher-resolution imaging technology. Papillary fibroelastomas are associated with substantial complications that are secondary to systemic embolism. Incidentally discovered papillary fibroelastomas are treated on the basis of their size, mobility, and associated comorbidities and symptoms. Surgical resection should be offered to all patients who have symptoms and to asymptomatic patients who have pedunculated lesions or tumors larger than 1 cm in diameter. Valve-sparing excision produces good long-term results in most instances.Herein, we present the case of a patient who was scheduled for elective myocardial revascularization and in whom an aortic valve papillary fibroelastoma was discovered incidentally during routine intraoperative transesophageal echocardiography. The timely recognition of this asymptomatic tumor enabled a modified operative approach: the patient underwent myocardial revascularization with concomitant valve-sparing resection of the tumor. We discuss the pathophysiology of fibroelastomas of the aortic valve and operative approaches to the management of these tumors when they are discovered incidentally.Key words: Aortic valve/pathology/surgery/ultrasonography, cardiac surgical procedures/methods, echocardiography, transesophageal, fibroma/complications/pathology/surgery/ultrasonography, heart neoplasms/diagnosis/pathology/surgery/therapy/ultrasonographyPrimary cardiac tumors are rare. Papillary fibroelastomas are the 2nd most common type of cardiac tumor, surpassed only by myxomas, which arise 6 times more often.¹ Papillary fibroelastomas are recognized more frequently now than in the past because of advances in imaging technology. Most of these tumors are discovered incidentally during imaging before cardiac surgery. The routine use of intraoperative transesophageal echocardiography (TEE) substantially increases the ability of the surgical team to evaluate the structural and functional anatomy of the heart before any operative procedure is undertaken.Here, we report our treatment of an aortic valve papillary fibroelastoma that was discovered incidentally, during routine intraoperative TEE, in a patient who was scheduled for elective myocardial revascularization. We discuss the pathophysiology of fibroelastomas of the aortic valve and operative approaches for managing these tumors when they are incidentally discovered.     

Concurrent Primary Cardiac Tumors of Differing Histology and Origin: Case Report with Literature Review

Primary cardiac tumors are rare and are diverse in histology and anatomic origin. Approximately 75% are benign, and nearly 50% of these are myxomas. Herein, we report concurrent myxoma and papillary fibroelastoma, which tumors were found attached to the left atrial septum and aortic valve, respectively. Concurrent primary cardiac tumors of differing histology and origin are rare, and, to our knowledge, this is one of the few such cases reported in the medical literature.Key words: Echocardiography, fibroma, heart neoplasms/primary/diagnosis/surgery, myxoma, neoplasms, multiple primary/diagnosis/surgeryPrimary cardiac tumors are rare, with an incidence ranging from 0.0017% to 0.19% in autopsy series in unselected patients.^1–3 Myxomas are the most common cardiac neoplasm, accounting for as many as 50% of all benign tumors.⁴ Papillary fibroelastoma, the 3rd most common cardiac neoplasm, occurs in adults and is frequently diagnosed postmortem.⁵ Concurrent primary cardiac tumors of differing histology and origin are rare, and few cases have been reported in the medical literature. We are reporting a case of concurrent intracardiac myxoma and papillary fibroelastoma.     

Papillary fibroelastoma of the pulmonary valve: assessment by live/real time three-dimensional transthoracic echocardiography

The differential diagnosis of a cardiac valve mass includes fibroelastoma, myxoma, lipoma, Lambl's excrescences, thrombus, and vegetation. Fibroelastomas are extremely rare primary cardiac tumors. Their incidence is 0.00017-0.033% in autopsy series and 0.019% in clinical series identified on echocardiography. Although rare, fibroelastomas are the most common tumors affecting the cardiac valves with aortic, mitral, tricuspid, and pulmonary valves being involved in this order. In the current report we describe a case of pulmonary valve mass in which a confident prospective diagnosis of fibroelastoma could be made utilizing the technique of three-dimensional transthoracic echocardiography.     

Papillary Fibroelastoma of the Aortic Valve: Analysis of 21 Cases,Including a Presentation with Cardiac Arrest

Cardiac papillary fibroelastoma is a rare, benign tumor, arising predominantly from cardiac valves. This tumor can cause a variety of symptoms due to thromboembolism. We describe our single-center surgical experience with papillary fibroelastoma of the aortic valve.From April 2004 through June 2013, 6,530 patients underwent cardiac surgery. Of those, 6,098 patients were included in the final analysis. Twenty-one patients (0.34%) underwent surgical resection of 30 papillary fibroelastomas of the aortic valve.Most patients (67%) were incidentally diagnosed to have cardiac papillary fibroelastoma. The usual symptom was cerebral infarction (in 5 of 7 symptomatic patients). A rare presentation of papillary fibroelastoma in one patient was cardiac arrest caused by left main coronary artery ostial obstruction. Tumor size was not related to patient age (Pearson correlation coefficient, 0.34; P=0.13). Neither the number of tumors (1.43 ± 0.72 vs 1.43 ± 0.62) nor tumor size (8.14 ± 2.42 vs 8.07 ± 3.31 mm) was significantly different between symptomatic and asymptomatic patients. All lesions were resected by means of the simple shave technique. There were no operative or 30-day deaths. Follow-up echocardiograms showed no tumor recurrence (mean follow-up duration, 17 ± 14 mo).We identified no significant relationship among tumor size, number of tumors, symptoms, or patient age. Because simple shave excision of the tumor can be safely achieved without evidence of tumor recurrence, we conclude that surgical resection can be reasonable in asymptomatic patients.     

Occurrence of a second primary papillary fibroelastoma

Papillary fibroelastomas are rare, benign cardiac tumours that most commonly arise from cardiac valves, and often present with signs and symptoms of embolization. A patient presented initially with transient ischemic attacks. He was found to have a papillary fibroelastoma on his tricuspid valve, which was successfully resected. Nine years later, he presented again with cerebral embolic symptoms and was found to have a new fibroelastoma on his mitral valve. The presence of a new lesion should be considered if signs or symptoms recur.     

Relative AV-insufficiency--morphometric and morphologic investigation of the AV-valve apparatus

Circumference and surface area of AV-valves were determined in 200 hearts formalin-fixed in their postmortem contracted state. It was found that, with increasing ventricular weight, the increase of the surface area of the valves exceeds that of the valve circumference. According to our findings, it is unlikely that relative valvular incompetence in eccentric cardiac hypertrophy is solely due to a disproportion between the valve surface area and circumference. Degenerative vascular changes of papillary muscle arteries and fibrosis of the papillary muscles may play one etiological role in the relative insufficiency of these valves.