Cardiac Myxoma

Ten patients with cardiac myxoma were reviewed. They ranged from 23 months to 60 years old. Echocardiography was the most helpful noninvasive diagnostic technique. The tumor was demonstrated by angiocardiography, left atrial myxomas frequently migrating to the left ventricle in diastole. Hemodynamically, left atrial myxomas were associated with moderately severe pulmonary hypertension and simulated mitral stenosis or insufficiency and right atrial myxomas, with right atrial hypertension. There were 7 myxomas in the left atrium, 2 in the right atrium, and 1 in the right ventricle.Eight patients underwent open-heart operation with removal of the myxoma, 1 had concomitant tricuspid valve replacement, and 1 had biopsy of the right ventricle only. The other patient was a Jehovah's Witness and refused operation. One patient died of cardiac arrest intraoperatively, and another died of a bilateral cerebral infarct. One patient had recurrence requiring reoperation. Postoperative hemodynamic and clinical improvement was more striking in patients with a left atrial myxoma presumably due to a normal mitral valve in contradistinction to the tricuspid valve.     

Multiple recurrence of cardiac myxoma in a Carney complex patient 4 years after the first operation

Carney complex is a rare syndrome which includes cardiac myxoma, hyperactive endocrine neoplasm, spotty pigmented skin, and extracardiac myxomatous tumors. We report a case of a 26-year-old woman with Carney complex in whom recurrent multiple cardiac myxomas were resected 4 years after the first operation for left atrial (LA) myxoma. She had a history of left adrenalectomy in 1997 for Cushing syndrome due to primary pigmented nodular adrenocortical disease (PPNAD). In February 2001, she was diagnosed with Carney complex because of evidence of LA myxoma, her spotty pigmented skin lesions, her past history and family history of cardiac myxoma in her mother. Then, LA myxoma was successfully resected through the superior trans-septal approach and has been followed-up by ultrasound cardiography (UCG) every 6-month after discharge.In January 2005, UCG revealed 2 masses in the LA and the right ventricle outflow tract. The 2nd surgery was performed in February 2005. We found the 3rd myxoma during surgery, resembling a flat polyp in the LA just at the inflow of the right upper pulmonary vein. All 3 myxomas were successfully resected. Sixteen months after the 2nd operation, she has been doing well without any sign of recurrence of myxoma.     

An atypically localized atrial myxoma: a case report

Atypical cardiac myxomas are rare occurrences and may present with a variety of clinical manifestations depending on the location and morphology. A 46-year-old woman had a 4 x 3 x 2-cm myxoma originating from the superior wall of the left atrium, found by echocardiography and multislice tomography. The tumor was successfully treated by surgical excision. The resected tumor was a well-defined encapsulated mass with a narrow-base stalk originating from the right wall of the left atrium in between the right upper and lower pulmonary vein. The patient recovered without complication and was discharged 6 days after the operation. At 1-year follow-up, echocardiography revealed normal cardiac function without reccurence in terms of mass. Although up to 80% of myxomas are localized in the left atrium, of which 75% involve in the interatrial septum, it should not be forgotten that myxomas can appear in an atypical localization, as occurred in our case.     

Cardiac papillary fibroelastomas: report of 2 cases

We report 2 cases of cardiac papillary fibroelastomas in adults. Case 1: A 61-year-old man was admitted because echocardiography showed a 1 cm pedunculated papillary tumor in the left atrium. In an operation, it was located in the left atrium near the mitral valve and was resected along with a 5 mm margin of endocardium. Case 2: A 60-year-old woman had a 1 cm mobile tumor in the right ventricle near the tricuspid valve located by echocardiography in a preoperative examination of a ventricular septal defect. In an operation, a pedunculated tumor located in the right ventricle was resected. In these 2 cases, histopathology showed the tumor to be a papillary fibroelastoma. Almost all cardiac papillary fibroelastoma are closely related to the cardiac valve, but in these cases, the tumors were located in the left atrium, and the right ventricle, respectively, which is quite rare.     

Diagnosis and treatment of 52 cases of cardiac myxoma

43 patients with cardiac myxoma had undergone surgical removal of the tumors under CPB 38 tumors were located in the left atrium, 5 in right atrium, and 1 in right ventricle respectively. The sites of attachment of the tumor stalk observed during operation were as follows: 34 left atrial myxomas were attached to the interatrial septum and 4 left atrial myxomas to the atrial wall. 5 right atrial myxomas were attached to the interatrial septum and 1 right ventricular myxoma to the ventricular wall. 1 patient with a right ventricular myxoma which had been removed one year ago appeared new one in the left atrium and the tumor was cut too. The diagnosis of cardiac myxoma was confirmed by 2D-echo-cardiography in all of our cases. Mitral valve replacement was done in 1 patient in this series. There was one death postoperatively. Besides these 43 cases, there were 9 patients who had not been operated upon, because of refusal of operation in 5, sudden death from cardiac arrest before operation in 2, coma from cerebral thrombosis in 1, and serious pulmonary tuberculosis in another one respectively.     

Determinação da massa residual em ventrículo esquerdo por ecocardiografia transesofágica intraoperatória após ressecção de mixoma gigante e flutuante em átrio esquerdo

Atrial myxoma is a benign tumor of the heart that occurs primarily in the left atrium. Floating or large left atrial myxomas frequently cause functional mitral stenosis, may also affect mitral valve structure and flow, and lead to mitral regurgitation. Systemic embolization occurs in around 30% of cases either from tumor fragmentation or complete tumor detachment hence it should be removed as soon as it is detected. Intraoperative transesophageal echocardiography has a vital importance in the surgery. After resection of myxoma, intraoperative transesophageal echocardiography must be performed to rule out residual mass. The case here reported is of a 48‐year old female, who presented with giant and floating left atrial myxoma. Residue mass was detected with intraoperative transesophageal echocardiography in the left ventricle after the resection of myxoma. Subsequently, the residue mass was successfully removed. Complete resection must be required to prevent possible complications such as recurrence, embolization in atrial myxomas. Transesophageal echocardiography performed intraoperatively is vital importance to confirm that the myxoma is completely resected.     

Novel strategies for recurrent cardiac myxoma

A 21-year-old man presented with a stroke. Subsequent investigations revealed cardiac tumors in his left atrium and right ventricle. These were removed by conventional techniques. Histopathology confirmed them as myxomas. He had a complete neurologic recovery, but presented 2 years later with recurrence of a cardiac myxoma in his left ventricle and left atrium. This time, however, endoscopically assisted techniques were used. The tumor was excised with supplementary cryoablation. The patient made a quick recovery from this second procedure and has had no recurrence after a 1-year follow-up.     

Giant biatrial myxoma with two different gross findings

We experienced a giant biatrial myxoma concomitant with hepatocellular carcinoma. Most of myxomas originate from the left atrium, and biatrial myxomas are extremely rare. Excision of the giant cardiac tumor was performed to avoid risks of life-threatening complications. The resected mass was grossly composed of two parts with the border of interatrial septum and with the shape of peanut shell. Although microscopic examinations revealed enlarged vessels, hemorrhages and hemosiderosis in the left part and high cellularity with chronic inflammation in the right part, spindle-shaped cells in a loose myxoid stroma were observed in both parts of the tumor, consistent with the diagnosis of myxoma. His second operation for hepatic cancer was successfully performed following 1 month after the first operation. Surgical treatment should be considered for giant atrium tumor which has risk of life-threatening complications even if patients have another cancer.     

The surgical treatment of 30 patients with cardiac myxomas: A comparison of clinical features according to morphological classification

Thirty patients with cardiac myxomas whose main clinical symptoms included congestive heart failure, tachyarrhythmia, chest pain and emboli, were successfully treated with surgery. The cardiac myxomas were found in the left atrium in 23 patients, the right atrium in 6, and the right ventricle in 1. Complete follow-up was conducted from 1 month to 15 years (mean 5.4 years) on 28 patients, 24 of whom were in New York Heart Association (NYHA) Class I, and 2 of whom were in NYHA Class II. The actuarial survival rate was 89% 15 years after surgery and no recurrent myxomas have been identified clinically or by echocardiography in any of the patients. Thus, an aggressive surgical approach is recommended prior to the development of heart failure or other complications whenever cardiac tumors are detected.     

右心粘液瘤的诊治

目的　探讨右心粘液瘤的诊治及疗效。方法　自 1984年 8月至 1999年 6月 ,16例右心粘液瘤病人接受外科治疗 ,术前超声心动图明确右房粘液瘤 11例 ,右室粘液瘤 5例 ,4例右房粘液瘤同时合并左房粘液瘤。均在体外循环下行粘液瘤摘除术 ,1例同时行三尖瓣置换术。结果　无围术期死亡 ,1例术后出现左心功能衰竭 ,运用左心辅助装置治疗后好转。随访 1个月到 15年 ,平均 4 8年。 1例术后13年粘液瘤原位复发再次手术治疗 ,复发率 6 .2 5 %。结论　右心粘液瘤一经确诊应立即手术 ,且效果好、安全。超声心动图对右心粘液瘤的诊断具有极其重要的作用。     

Familial cardiac myxoma with multiple and contralateral recurrence

This report described a familial recurrent cardiac myxoma involving mother and daughter. The mother, at 27 year of age, had developed recurrent multiple myxomas in both left and right atrium and right ventricle 4 years after surgical excision of left atrial myxoma. Excision was successful and remains well without signs of recurrence 9 years postoperatively. In an asymptomatic 13-year-old daughter, a recurrent left atrial myxoma was found 3 years after the excision of right atrial myxoma by echocardiographic follow-up at 6 month intervals. Excision of left atrial myxoma was performed and histology showed the essentially the same findings as primary myxoma without signs of malignancy. From an experience of this familial recurrent myxoma and a review of 38 cases of 17 familial cardiac myxoma, it is recommended that wide excision of tumor including surrounding tissues, thorough search for multiple heterotopic tumors at surgery, close postoperative echocardiographic follow-up for at least 5 years, and examination of skin and breast tumor, and endocrine disorder for "complex" myxoma.     

A case of biatrial multiple myxomas with glandular structure.

A 45-year-old male, who had been indicated by brain magnetic resonance imaging to have cerebral infarctions, was found by echocardiography to have a tumor in the left atrium. He had experienced several of the constitutional disturbances associated with myxoma. At the ages of 19 and 35 he had had two episodes associated with embolisms, and at the later one he was diagnosed as having multiple cerebral aneurysms. He received an urgent operation in which three left atrial tumors and one right atrial tumor were resected. Histologically, the tumors were myxomas, and the left atrial main tumor had glandular structure. In view of his clinical history, this patient seems to have had cardiac myxomas for a long period. The multiple growths that occurred in this case may be a good argument for allowing this condition to last for so long. To our knowledge, the present case was the first report of cardiac myxoma with glandular structure in Japan.     

A case of tricuspid valvular myxoma in a child]

A six-year old boy had hospitalization because of cardiac murmur and syncope attack. Laboratory data including immunoglobulin and erythrocyte sedimentation rate were normal, but echocardiography and cardioangiography indicated tricuspid valvular myxoma in the right atrium. The tumor was successfully removed with the septal tricuspid valve using the cardiopulmonary bypass. The tumor was 18 x 20 x 12 mm in size and pathological findings showed a myxoma originated from tricuspid valve. Now he shows normal cardiac function and no recurrent sign of tumor. There has been reported a few cases of valvular myxoma in Japan. However, this is the first case which was successfully resected by surgery.     

Cardiac tumors in 19 years of private practice

Since 1966 the authors have encountered nine cardiac tumors: eight myxomas and one tumor initially thought to be a sarcoma but histologically a squamous carcinoma. Seven myxomas were left atrial and the other were right atrial in location. The carcinoma was predominantly located in the right ventricle. Eight of the nine patients were female; ages ranged from 32 to 85 years. Of the myxomas, five displayed fever and dyspnea, two congestive heart failure and one transient ischemic episodes. The patient with carcinoma was in right heart failure. All patients underwent cardiac catheterization as well as echocardiography when it became available. Five myxomas were correctly diagnosed preoperatively while three were thought to have mitral stenosis. The patient with carcinoma showed a large right ventricular filling defect and a picture of constriction. All myxoma patients underwent surgery--three via left thoracotomy and five via sternotomy. Seven patients survived; however, the patient with right atrial myxoma was explored via the left chest for mitral stenosis and the myxoma was not discovered. She died of a pulmonary embolism pathologically confirmed as myxoma. All survivors have been followed by yearly echocardiograms and are free of recurrence. The patient with carcinoma died before surgery could be performed. Autopsy revealed almost complete replacement of the right ventricle by tumor and constrictive pericarditis. The terminal event was pulmonary embolus of squamous carcinoma. Although cardiac tumors are a rare entity, a high incidence of suspicion plus modern diagnostic methods can yield the diagnosis and allow a curative operation.     

Tumorectomy with right thoracotomy for synchronous left atrial myxomas from Carney complex: report of a case

This report describes a case of synchronous left atrial myxomas from Carney complex resected through a right thoracotomy. The patient was a 30-year-old female that had previously been diagnosed with Carney complex following a genetic examination. Preoperative echocardiography showed a left atrial tumor, but intraoperative inspection revealed another tumor in the left atrium. Carney complex was first described in the 1980s and cardiac myxoma from Carney complex can occur in any cardiac chamber, presenting multiple times with postoperative recurrences, occurring at any age and without any predilection for gender, and is inherited in an autosomal-dominant manner. Treatment for cardiac myxoma from Carney complex is very important for patient mortality and morbidity and, despite the endocrine nature of the disorder, cardiologists and cardiac surgeons play an important role.     

Myxoma of the heart: clinical and experimental observations.

During the past 12 years, 13 patients with atrial (10 left and 3 right) myxoma have been treated. The tumors of the left atrium produced signs and symptoms of mitral valve obstruction and/or subacute bacterial endocarditis and those of the right atrium manifestations of tricuspid valve disease or of pulmonary embolus or hypertension. The diagnosis was established by angiocardiography in 8 patients, at surgery performed for suspected mitral stenosis in 3 patients, and at autopsy in 2 patients. Resection of the atrial myxoma alone in 5 patients or with atrial septum where the atrial myxoma was attached in 4 or with the whole right atrial wall where the atrial myxoma was attached in one patient was performed and all are doing well without evidence of recurrence. Studies of experimentally produced 1.5-3 cm in diameter left atrial thrombus in 30 dogs divided into 5 groups and followed cineangiocardiographically and sacrificed from 14 days to 6 months indicated that the implanted thrombus is absorbed over a 3 to 6 month period. These experimental and human left atrial thrombi were found to be histologically and histochemically different from human atrial myxomas. The electron microscopic studies performed on some of the resected atrial myxomas suggested that the atrial myxoma cells are active cells of endotheilial origin. These observations suggest that atrial myxoma is a primary tumor of the heart which can mimic other clinical entities, and the results of its surgical treatment are gratifying and long lasting.     

Development of left ventricular--coronary sinus fistula following replacement of mitral valve prosthesis

The features and course of a patient in whom a fistula developed between the left ventricle and the coronary sinus following replacement of a faulty mitral valve prosthesis are described. The subsequent deterioration of her condition was associated with a pansystolic murmur and periprosthetic regurgitation. Replacement by a third prosthesis was complicated in addition by tricuspid regurgitation. The fistula was not suspected until the fourth operation, when the right atrium was opened for tricuspid annuloplasty. The fistula was repaired successfully, and the woman has made good progress postoperatively.     

An operative case of left ventricular-right atrial communication complicated with aortic valve stenosis and regurgitation

A 70-year-old woman suffering from aortic valve stenosis and regurgitation and tricuspid valve regurgitation was admitted to our hospital. At the operation, the right atrium and both ventricles were dilated, and a thrill was felt over the aortic root and right atrium. The aortic valve was bicuspid and heavily calcified. After aortic leaflets were resected, a defect was seen in the left ventricle. Then the right atrium was opened. The defect was located in the atrioventricular membranous septum just above the septal leaflet of the tricuspid valve. The defect was diagnosed to be a left ventricular-right atrial communication (group I according to Riemenschneider's classification). The defect was repaired directly and then the aortic valve was replaced with a prosthesis. After the operation, the heart murmur disappeared. Eleven days after the operation, the patient developed a transient complete AV block for a month.     

Surgical treatment of cardiac myxomas

Information of 40 patients operated on for cardiac myxoma was reviewed. The age ranges were as follows: nine patients, 20 to 30 years; seven patients, 31 to 40 years; 10 patients, 41 to 50 years; and 11 patients, over 50 years. One patient each was 14 months, 14 years, and 16 years old. Left atrial myxoma was diagnosed in 31 patients, left ventricular myxoma in one, right atrial myxoma in five, and right ventricular myxoma in two. Multiple calcified right atrial and ventricular myxomas were detected in one patient. Detailed clinical characteristics and diagnostic methods are presented. The hospital mortality rate was 7.5%. At 15 years' follow-up practically all of the patients had good late results. There were no recurrent myxomas. Rare left ventricular myxomas and multiple calcified right atrial and ventricular myxomas involving a tricuspid valve are also presented.     

Cardiac myxomas: 24 years of experience in 49 patients

Objectives: In this single-center study we reviewed our experience with a significant number of cardiac myxoma cases occurring over the past two decades. Patients and methods: Cardiac myxomas represented 86% of all surgically treated cardiac tumors at our center. Specifically, there were 49 consecutive patients, each with at least one myxoma. A detailed clinical, immunological, and echocardiographic long-term examination of 37 patients revealed one recurrent myxoma. Results: Most myxomas originated from the left atrium (87.7%), but also much less frequently from the mitral valve (6.1%), from the right atrium (4.1%), and from the left and right atria (2.0%). The myxomas produced a prolapse into the left ventricle in 40.8% of the patients, mitral stenosis in 10.2%, and threatened left ventricular outflow tract obstruction in 2.0%. Multiple myxomas were found in 20.4% of the patients. Cardiac signs appeared in 93.9% of the patients. Preoperative embolic events had occurred in 26.5%. Immunologic alterations were present in 87.5%. For resection, a bilateral atriotomy was used. An additional aortotomy was needed to expose one mitral valve myxoma. Postoperatively, 81.1% of the patients remained without cardiac symptoms. The early mortality rate was 2.0% and the late mortality rate was 6.1%. Long-term prognosis was excellent with an actuarial survival rate of 0.74. Specific immunologic alterations were found in 71.4% of the patients. The actuarial freedom from reoperation of the myxoma was 0.96. The rate of reoperations was low with 2.0% after 24 years. Conclusions: Myxomas were usually detected and operated on in symptomatic patients. A high index of suspicion seems important for early diagnosis. Immunologic findings may play an additional role in confirming the diagnosis and the recurrence of a myxoma. Immediate surgical treatment was indicated because of the high risk of embolization or of sudden cardiac death. Also, a familial genesis must be excluded in myxoma patients.