Valve-Sparing Excision of Multiple Papillary Fibroelastomas in the Aortic Valve and Left Ventricular Outflow Tract / 日本心臓血管外科学会雑誌

Primary cardiac fibroelastoma is a relatively rare tumor and is often detected incidentally by echocardiography. We report a case of multiple fibroelastomas that were found incidentally by follow-up echocardiography for hypertrophic cardiomyopathy and were treated with valve-sparing excision. The patient was a 71-year-old man, in whom a 10-mm tumor on the ventricular septum below the right coronary cusp and 3-mm tumors on the left ventricular side of the left and right coronary cusps were detected. Although he had no symptoms, because the tumors were mobile, surgery was performed for preventing embolization and making a definitive diagnosis. The tumor on the ventricular septum was excised together with the surrounding endocardium and part of the myocardium. The tumors on the valve leaflets were excised with the aortic valve cusps spared. Histopathologically, all the tumors were fibroelastomas. The postoperative course was uneventful.

A Case of Multiple Cardiac Papillary Fibroelastoma in the Aortic Valve / 日本心臓血管外科学会雑誌

We report a case of multiple papillary fibroelastoma (PFE) on the aortic valve. A healthy woman in her 60 s was referred to a nearby doctor with the chief complaint of palpitation and was admitted to our hospital for detailed examination. A mobile tumor was found by transthoracic echo, and she was introduced to us. We undertook excision surgery for her. Initially, it was thought to be a single tumor, and a pedunculated tumor adhering to the central aortic side of the left coronary cusp with a length of 7 mm was removed. After closing the aorta, we recognized a remaining mobile tumor by transesophageal echo. We decided to perform cardiac arrest again, and we recognized one tumor with 6 mm long string-like mobile mass on the right and non-coronary commissure of cardiac side, and a thorn-like mass of about 1.5 mm on the non-coronary cusp of the cardiac side. We removed both tumors from the aortic valve using shaving resection and preserved the aortic valve. All of these were found to be PFE pathologically as if they were three tumors that followed the developmental stage of PFE. After surgery, PFE recurrence is extremely rare. In the case of a pedunculated tumor, it is possible to remove the tumor and preserve the valve. However, as in this case, if both sides of the valve were not carefully inspected, it can be overlooked. In addition, small tumor resection could avoid the risk of new cerebral infarction and myocardial infarction due to recurrence and could avoid reoperation.

A Case of Left Atrial Villous-Type Myxoma Mimicking Papillary Fibroelastoma / 日本心臓血管外科学会雑誌

A female in her 60 s with a mobile cardiac tumor in the left atrium was referred to our hospital. The tumor was diagnosed as myxoma by echocardiography, CT scan, and MRI. Under cardiac arrest using cardiopulmonary bypass, we resected the tumor through the superior trans-septal approach. We resected two tumors (tumor 1 and 2) with adequate rims of the interatrial septum to avoid recurrence. Although the tumor had a broad base and extended to near the mitral annulus, mitral valve repair was not necessary. Tumor 1 was gelatinous and seemed to be a myxoma ; tumor 2 had a lot of papillary structures attached to the solid mass and presented with the figure of a sea anemone when it was put in saline, which is typical for papillary fibroelastomas. The defect of the interatrial septum was closed with the autologous pericardium. The postoperative course was uneventful. Unexpectedly, both tumor 1 and 2 were diagnosed as myxomas on pathological examination. Tumor 2 (papillary lesion) included a few tumor cells with positive staining of calretinin typical for myxoma and was diagnosed as a “villous-type myxoma”. Because it is difficult to distinguish myxoma from papillary fibroelastoma by pre- and intraoperative findings, it would be recommended to resect tumors with an adequate margin to prevent recurrence.

Multiple Papillary Fibroelastomas Involving All Four Heart Valves / 日本心臓血管外科学会雑誌

Papillary fibroelastoma (PFE) is a rare primary cardiac tumor that usually involves an aortic or mitral heart valve. We encountered a case of a 32-year-old woman, who presented with syncope and was found to have multiple PFEs involving all four heart valves during surgery. The echocardiography was performed and showed two mobile masses near the tricuspid and mitral valves. Moreover, the enhanced computed tomography (CT) showed thickened aortic cusps, which may indicate the possibility of heart tumor. Intraoperatively, we first found multiple tumors at each cusp of the aortic valve, ranging in size from 5 to 10 mm which were excised without injury of aortic cusps themselves. These showed a sea anemone-like appearance and were suspected to represent PFE. We then observed the tricuspid and mitral valves, and both valves showed tumors of similar appearance in each cusp. Furthermore, we found a tumor at the pulmonary valve, even though there had been no evidence of its presence on echocardiography or CT. We confirmed that these masses were PFEs by histological study after the operation. We should keep in mind that PFE can develop in multiple valves. To the best of our knowledge, this is the first description of multiple PFEs involving all four heart valves.

A Case of Resection of Fibroelastoma by Endoscopy / 日本心臓血管外科学会雑誌

We report a case of a 64-year-old woman who presented with symptoms for the common cold, for a medical examination. The chest X-ray showed enlargement of the heart, and echocardiography detected a mobile mass suggestive of a tumor 21×14 mm in the left ventricle. Because there was a risk of embolism, we decided to perform an operation. The tumor was elastic and soft and had a stalk arising from the wall of the left ventricle. We considered that it would be difficult to observe and expose the left ventricular tumor by direct vision. Therefore, we chose to perform a complete endoscopic resection. The tumor was determined histopathologically to be a papillary fibroelastoma. Here, we report the relatively rare benign tumor, a papillary fibroelastoma, and include a discussion of the literature.

Fibroelastoma papilar cardíaco: estudio retrospectivo: Presentación clínica y resultados quirúrgicos / Fibroelastoma papilar cardíaco: estudio retrospectivo: Presentación clínica y resultados quirúrgicos

Los fibroelastomas papilares cardíacos son pequeños tumores benignos con potencial embolígeno. Con la incorporación de la ecocardiografía y la mejoría en la resolución de las imágenes, el diagnóstico clínico es cada vez más frecuente, y en series recientes su frecuencia supera a la del mixoma cardíaco. Nuestro objetivo fue analizar las características de una serie de casos de fibroelastoma papilar cardíaco con confirmación histológica operados en nuestro hospital. Analizamos retrospectivamente los tumores cardíacos y las características clínicas de los pacientes operados desde junio de 1992 a febrero de 2017. De 108 operados, 18 presentaron fibroelastoma papilar. La edad media del grupo con fibroelastoma papilar fue 58 años (22-77); 10 eran varones. Las localizaciones más frecuentes fueron la válvula aórtica (7) y la válvula mitral (5). Ninguno presentó disfunción valvular significativa. Por ecografía transesofágica, el tamaño (diámetro mayor) fue 13.33 ± 5.55 mm (6.6-28.0). Cuatro pacientes eran sintomáticos; uno presentaba disnea, otro aleteo auricular, dos con fibroelastoma en válvula aórtica habían sufrido un accidente cerebrovascular. Los restantes, asintomáticos, fueron intervenidos preventivamente. En 15 pacientes el tumor se extirpó sin necesidad de reemplazo valvular. No hubo mortalidad quirúrgica ni recidiva tumoral en el seguimiento de 2.6 años. Se concluye que los fibroelastomas papilares cardíacos pueden ser extirpados con buenos resultados a mediano plazo y, en su mayoría, con preservación valvular. Sin embargo, no existen aún estudios aleatorizados que avalen la escisión quirúrgica en pacientes asintomáticos. Por el momento, esta última depende de la experiencia del grupo quirúrgico tratante.

Papillary ibroelastomas are small benign intracardiac tumors known for their embolic potential. Since the introduction of echocardiography with improved resolution and transesophageal imaging techniques, they are being increasingly detected in clinical practice. In recent series, papillary fibroelastoma is considered the most frequent benign tumor of the heart. Our objective was to analyze characteristics and midterm surgical outcome of histologically-confirmed cases of papillary fibroelastoma. We conducted a retrospective study on patients with cardiac tumors submitted to surgical excision between June 1992 and February 2017. Out of 108 patients, 18 had papillary fibroelastomas. Their mean age was 58 years (22-77); 10 were men. The most frequent localizations were the aortic valve (7) and the mitral valve (5). None had significant valvular dysfunction. By transesophageal echocardiography, the tumor size (larger diameter) was 13.33 ± 5.55 mm (6.6-28.0). Two patients, both with tumor in the aortic valve, had suffered a stroke; other two had dyspnoea and atrial flutter, respectively. The remaining 14 patients were asymptomatic and their tumors were incidental findings. In 15 patients the valve was preserved. There was neither surgical mortality nor recurrence after 2.6 years of follow-up. In conclusion, most papillary fibroelastomas can be surgically removed with valve preservation and favorable clinical outcome. However, until the results of randomized trials support the decision, an aggressive surgical approach in asymptomatic patients needs to be defined in the context of surgical expertise.

Clinical and echocardiographic characteristics of cardiac papillary fibroelastoma in adults / 中国医学影像技术

Objective To explore the clinical features and echocardiographic characteristics of cardiac papillary fibroelastoma (CPF) in adults.Methods Clinical features,echocardiographic characteristics,surgical procedures and outcomes were retrospectively evaluated in 13 patients with CPF confirmed by pathology.Results The clinical features of CPF were atypical.The most common symptoms were chest distress and short breath.All the 13 patients were single lesions,9 cases (9/13,69.23％) involved the valves (4 on the aortic valve,3 on the mitral valve,2 on the tricuspid valve) and 4 cases (4/13,30.77％) involved the chambers (2 in the right atrium,1 in the right ventricle,1 in the left ventricle).The largest diameters of ＞2 mm were in 8 cases and ≤2 mm were in 5 cases.Eight cases were detected by echocardiographic examination and 5 cases were missed.Surgical excision was performed in 8 patients and prosthetic valve replacement was performed in 5 patients.Conclusion The clinical symptoms of CPF are variable.When the size of CPF is too small,echocardiography is difficult to detect.Most of CPFs originate on the valves,predominantly on the aortic valve.The prognosis of CPF is excellent.

Clinical and echocardiographic characteristics of cardiac papillary fibroelastoma in adults / 中国医学影像技术

Objective To explore the clinical features and echocardiographic characteristics of cardiac papillary fibroelastoma (CPF) in adults.Methods Clinical features,echocardiographic characteristics,surgical procedures and outcomes were retrospectively evaluated in 13 patients with CPF confirmed by pathology.Results The clinical features of CPF were atypical.The most common symptoms were chest distress and short breath.All the 13 patients were single lesions,9 cases (9/13,69.23％) involved the valves (4 on the aortic valve,3 on the mitral valve,2 on the tricuspid valve) and 4 cases (4/13,30.77％) involved the chambers (2 in the right atrium,1 in the right ventricle,1 in the left ventricle).The largest diameters of ＞2 mm were in 8 cases and ≤2 mm were in 5 cases.Eight cases were detected by echocardiographic examination and 5 cases were missed.Surgical excision was performed in 8 patients and prosthetic valve replacement was performed in 5 patients.Conclusion The clinical symptoms of CPF are variable.When the size of CPF is too small,echocardiography is difficult to detect.Most of CPFs originate on the valves,predominantly on the aortic valve.The prognosis of CPF is excellent.

Cardiac papillary fibroelastoma; when, how, why.

We would like to present an interesting case operated on in our department and discuss the international bibliography about this issue. We also present some interesting images of this case. Our material is composed from a 68‑year‑old woman treated by the authors. She presented with a small murmur in the auscultation while she was asymptomatic and then she diagnosed with a tumor on of the left coronary cusp of the aortic valve with the characteristics of papillary fibroelastoma. On the basis of the potential embolic risk either of the mass itself or of associated thrombus and the possibility of further enlargement, the patient although asymptomatic at the time of diagnosis was referred for elective surgical excision of the mass. She underwent on median sternotomy and through extracorporeal circulation the mass has been excised with the preservation of the well‑functioning valve. Through this case, we would like to discuss the bibliography for the decision making in these cases. Hence, the aim of our study is that we have to keep in mind that this kind of friable mass may be the cause of embolism, stroke or coronary artery occlusion and must be excided in a conservative setting, sparing the aortic valve.

Coexisting Left Atrial Myxoma and Aortic Valve Papillary Fibroelastoma / 日本心臓血管外科学会雑誌

<p>Primary cardiac tumors are rare. Myxoma is the most common type of benign cardiac tumor and papillary fibroelastoma (PFE) is the second most common. We report a case of coexisting left atrial myxoma and aortic valve PFE. A 77-year-old Japanese woman with a left atrial mass was referred to our hospital for further diagnostic evaluation and surgical treatment. The mass was detected by coronary computed tomography, which was performed by her general practitioner. Investigation with an echocardiogram revealed a mass on the fossa ovalis, extending into the left atrium. Intraoperative transesophageal echocardiography (TEE) showed another mass attached between the left coronary cusp (LCC) and the right coronary cusp of the aortic valve. After cardiopulmonary bypass and cardioplegic arrest, we performed an aortotomy, and observed the aortic valve. We found a mobile mass on the LCC and resected it. After left atriotomy, a left atrial myxoma was identified and resected, together with its margin. Postoperatively, sinus bradycardia, sinus pauses, and atrial fibrillation tachycardia were identified. Therefore, we implanted a permanent pacemaker on the 29th postoperative day. The patient was discharged on the 38th postoperative day. Simultaneous existence of two different primary cardiac tumors is rare. We believe that preoperative and intraoperative TEE in patients with cardiac tumors is important.</p>

Simple Excision for Cardiac Fibroelastomas Arising from Three Leaflets of the Aortic Valve / 日本心臓血管外科学会雑誌

Cardiac papillary fibroelastomas are rare but are still the second most common benign cardiac tumor ; after myxoma. While cardiac papillary fibroelastomas are benign, there is the potential for severe complications related to embolism. Consequently, a surgical treatment approach is generally recommended. Nevertheless, from the risk of the recurrence of tumor and the valve insufficiency, the excision range is still controversial, particularly with tumors arising from the valve. We report the case of a 66-year-old woman who underwent resection of cardiac papillary fibroelastomas arising from three leaflets of the aortic valves. We performed simple excision without valve surgery and obtained an uneventful prognosis. At 18 months after surgery, no recurrence of tumors was recognized. We consider that it is possible to resect cardiac papillary fibroelastomas without performing valve repair or replacement if they are removed carefully even if the tumors arise from three leaflets of an aortic valve.

Symptomatic Papillary Fibroelastoma- Case Report and Literature Review.

Primary cardiac tumors represent a small subset of cardiac neoplasms. The papillary fibroelastoma (PFE) is a benign tumor that typically affects the cardiac valvular system and is second in prevalence only to myxomas. Though typically asymptomatic and diagnosed incidentally, few patients with PFE may also experience neurologic and cardiac symptoms that prompt further diagnostic investigation. We present a case of a patient with transient monocular vision loss with a later occurrence of bilateral lower extremity weakness and confusion over a short period of time. The alarming symptomatology eventually led to diagnosis by transesophageal echocardiogram of a PFE involving the aortic valve. We include a review of the current literature on PFE to further elucidate etiology of this neoplasm, symptomology, prognosis, and treatment. Currently, the role of anticoagulation in PFE remains unclear. Due to the embolic nature of this neoplasm as evidenced by the case presentation, surgical resection, anticoagulation or a combination of both provides patients with maximal prevention of stroke.

A Surgical Case of Papillary Fibroelastoma Located in the Tricuspid Valve Chordae / 日本心臓血管外科学会雑誌

Papillary fibroelastoma (PFE) is the second most common type of benign cardiac tumor after myxoma, and is most commonly found in the left side of the heart. In this study, we report a case of PFE located in the tricuspid valve chordae. The patient was a 65-year-old woman in whom a heart murmur was detected during a routine medical examination. A follow-up examination identified a cardiac tumor, and she was subsequently referred to our medical department. Echocardiography and contrast-enhanced computed tomography indicated a mobile mass of approximately 1 cm in the right ventricle, and she was determined to be a candidate for surgery. After cardiopulmonary bypass, we instigated cardioplegic arrest, made an incision in the right atrium, and observed the right ventricle through the tricuspid valve. We observed a yellowish sea-anemone-like mass approximately 9 mm from the chordae of the anterior leaflet of the tricuspid valve. Thus, the mass was resected with the chordae and tricuspid annuloplasty. Postoperative progress was satisfactory, and the subject was discharged on the 19th day of hospitalization. Histopathological examination indicated a mixture of thick fibrous and adipose tissues, which led to the diagnosis of PFE. PFE is a relatively rare disease that comprises 8% of primary cardiac tumors. IA often occurs in the left heart and can cause cerebral infarction, myocardial infarction, and other symptoms of embolism. However, because small masses and those that occur in the right heart are not accompanied by clinical symptoms, such cases are often discovered during routine echocardiography. The present study reports a case of PFE that occurred in the tricuspid valve chordae that was diagnosed via echocardiography.

A Papillary Fibroelastoma in the Left Ventricle / 日本心臓血管外科学会雑誌

A 75-year old woman in whom a left ventricular tumor had been detected by echocardiography 2 years before referral to our hospital, presented with blurry vison for one month. Acute cerebral infarction was diagnosed. We suspected that the infarction was occurred by an embolus from the intraventricular tumor, and resected it through left atrial incision. The resected tumor was 10 mm in size and it resembled a sea anemone. The tumor was pathologically diagnosed as papillary fibroelastoma. The postoperative course was good, with no recurrence for the last 18 months.

A Case of Aortic Valve Papillary Fibroelastoma with Atrial Septal Defect / 日本心臓血管外科学会雑誌

A 68-year-old woman with a diagnosis of atrial septal defect (ASD) presented with dyspnea. Chest radiography demonstrated cardiomegaly and infiltration in both lungs, suggestive of cardiac decompensation due to ASD. Detailed evaluation with transthoracic echocardiography revealed a mobile tumor on the aortic valve. Intraoperatively, tumors were identified on all aortic cusps. Preservation of the aortic valve was difficult. We therefore performed aortic valve replacement and patch closure of the ASD. The existing literature suggests that mobile papillary fibroelastoma should be excised irrespective of size, to prevent the risk of embolism. Excision of the tumor alone is usually sufficient. However, the present case showed clustered tumors on the aortic valve, so preservation of the cusps could not be achieved in this case.

Successful Surgical Management of a Papillary Fibroelastoma in the Left Ventricle / 日本心臓血管外科学会雑誌

Papillary fibroelastoma is a rare benign cardiac tumor generally arising from the valvular endocardium. We describe the successful surgical management of a patient who had a papillary fibroelastoma attached to a false tendon of the left ventricle. A 71-year old man was admitted with a left ventricular tumor. Routine transthoracic echocardiography revealed a mobile, 6×8 mm mass, which was attached to a false tendon in the apical area of the left ventricle. Continuous intravenous heparin was commenced to avoid the embolism, and then an urgent operation was performed, consisting of left ventriculotomy following establishment of a standard cardiopulmonary bypass. A mobile gelatinous mass with a short stalk, 7 mm in diameter, was attached to the false tendon. The mass was excised including a part of the false tendon. The excised tumor changed its shape in saline to a sea-anemone like tumor. The histopathological findings were consistent with the diagnosis of papillary fibroelastoma. The patient made an uneventful recovery and was discharged from the hospital on postoperative day 12.

Papillary Fibroelastoma Mimicking Vegetation of the Mitral Valve

Although cardiac papillary fibroelastoma is rare, it is the most common primary tumor of cardiac valves. The clinical presentation of these tumors varies from asymptomatic to embolic complications. We report an asymptomatic case of papillary fibroelastoma of mitral valve which was diagnosed by transthoracic echocardiography. The tumor was successfully resected by surgery.

An Operative Case of Papillary Fibroelastoma of the Aortic Valve / 日本心臓血管外科学会雑誌

An echocardiogram revealed a mobile mass attached to the left coronary cusp of the aortic valve in an 81-year-old woman. The tumor was surgically removed without valve replacement. The tumor was whitish in color, with a sea anemone-like appearance, and it measured 10 mm in maximum dimension. It was histopathologically defined as papillary fibroelastoma (PFE), and the postoperative course was uneventful. Primary cardiac tumors are rare, and the majority are myxomas. However recent advances in noninvasive examination and surgery may increase the detection of PFE, which occurs most frequently on the endocardial surface of the cardiac valve. We report a case of cardiac PFE with a review of the pertinent literature.

A Case of Cardiac Papillary Fibroelastoma in a Tricuspid Valve / 日本心臓血管外科学会雑誌

Cardiac papillary fibroelastoma (CPFE) is a rare tumor, and is usually located in the atrioventricular or ventriculoarterial valves. It is occasionally identified by echocardiography or surgery. It can also be an unexpected finding at autopsy. As this tumor often occurs in left-sided cardiac chambers, early aggressive surgical resection is required in order to prevent severe systemic embolic complications. However, the operative indications of tumors on the right cardiac chamber are controversial. The patient was a 73 year-old man. He had had cerebral infarction at age 58. Before the currently reported operation, we found CPFE on the tricuspid valve but we could not find a patent foramen ovale (PFO) by the usual examinations. During surgery, we found a CPFE on the tricuspid valve that had a short stalk and PFO. We cut the short stalk of the CPFE easily, and closed the PFO directly. This patient did not need complicated valve repair. We speculated that this cerebral infarction was caused by a CPFE on the tricuspid valve and patent foramen ovale. Echocardiography is very useful in diagnosing CPFE. However, we should not neglect the possibility of PFO before surgery. The postoperative course was uneventful. We concluded that early surgical resection of CPFE, even in right-sided cardiac chambers, should be performed in order to prevent severe embolic complications, even without PFO diagnosis.

Primary Papillary Fibroelastoma in the Left Ventricle / 日本心臓血管外科学会雑誌

A 76-years-old woman was admitted with a left ventricular tumor that was accidentally found by a transthoracic echocardiogram. The tumor was located in the papillary muscle near the apex, had an irregular surface, and was well mobile. The urgent operation was planned because the tumor might have caused of embolism. A cardiopulmonary bypass was established with distal ascending aortic cannulation and bicaval venous cannulations. The resection of the tumor was performed by a transaortic approach. Pathological examination confirmed the tumor as primary papillary fibroelastoma. During the operation, a thoracoscope was used to assist the resection of the tumor. It was useful for a good visualization in the left ventricle. In this case report we describe a rare case of primary papillary fibroelastoma in the left ventricle and a technique of resection using a transaortic approach with an assistance of a thoracoscope.