Heart myxoma. Surgical treatment

We reviewed all the patients who underwent surgical excision of cardiac myxomas at La Pitié during the last fifteen years. Fifty-one cases were found (32 female and 19 male) aging from 16 to 75 years (mean 51). Congestive heart failure was the primary symptom present in 28 patients. Thirteen patients presented peripheral embolization, four with syncope and 11 with tachyarrhythmias. The diagnosis was made either by echocardiography or angiography. All of them had correct preoperative diagnoses, and no tumors were found incidentally at operation. Forty-six myxomas were localized in the left atrium, four in the right atrium and one in the right ventricle. All the patients underwent open-heart operation and myxomas were successfully removed with excision of a portion of normal atrial septum or wall. Path reconstruction of the atrial septum was required thirty-six times. Mortality after surgical excision is very low. Only one death (1.96%) occurred as a result of a postoperative low output syndrome. Late recurrences have been reported in other series, but no recurrences were diagnosed in our patients up to the present. Although the recurrence rate is low, long-term clinical and echocardiographic follow-up is recommended.     

Primary cardiac tumours--operative treatment of 20 patients

This report describes a series of 20 patients operated on for a primary cardiac tumour. The majority of the tumours (16) were benign myxomas; 12 of them were located in the left atrium, two in the right atrium and two were biatrial. Two lipomas were found; one was epicardial and the other was located in the left atrium. The only intraventricular tumour was a malignant left ventricular myosarcoma. The propensity of intracardiac tumours to embolize was distinctive. Nine of the 16 myxomas presented with peripheral embolization, and in two patients surgery was complicated by fatal perioperative cerebral embolization of myxomatous tissue. Furthermore, in one patient embolization of a left atrial lipoma necessitated amputation of her left arm before cardiac surgery. Late postoperative recurrences were found in two patients with atrial myxomas. In one of them, reoperation showed that the tumour had grown at that site in the interatrial septum where the original pedicle had been excised. One patient developed severe mitral valve regurgitation and underwent replacement with a prosthetic valve at reoperation. Otherwise our late follow-up study showed that the results of surgery were usually excellent even though mild echocardiographic abnormalities were not uncommon. Our experience emphasizes the embolic potential of intracardiac myxomas and suggests, furthermore, that to avoid recurrences excisions with wide margins should be preferred. Echocardiography is an optimal method for the follow-up of these patients.     

Clinicopathologic study and DNA analysis of 37 cardiac myxomas: a 28-year experience

PURPOSE: This study was performed to identify morphologic features of cardiac myxomas related to embolism and to provide a better understanding of the biology of these tumors, mainly in relation to their interleukin (IL)-6 expression and DNA content. PATIENTS AND METHODS: A total of 37 cardiac myxomas were reviewed retrospectively in a clinicopathologic study that included the correlation of echocardiographic and pathologic findings in 25 cases, together with immunohistochemical evaluation of IL-6 expression and flow cytometric DNA analysis of 35 tumors. RESULTS: There were 24 female patients and 13 male patients. The mean (+/- SD) age was 52 +/- 15 years. Fifty-four percent of patients presented with dyspnea, 51% presented with increased erythrocyte sedimentation rate (ESR), and 27% presented with embolic episodes, which were significantly associated with villous surface tumors. Atrial fibrillation was registered in 19% of patients and was significantly associated with large left atrial myxomas. Echocardiography proved to be a reliable method for preoperative diagnosis and for predicting tumor size and morphology. There was no perioperative mortality or long-term recurrences. The frequency of early surgical complications was associated with a longer mean ischemic time. Seventeen percent of tumors had abnormal DNA content, and 74% of tumors showed immunohistochemical expression of IL-6. Neither of these factors showed a significant association with embolism or constitutional illness. CONCLUSIONS: Villous surface myxomas are related to embolism, and large left atrial tumors are related to atrial fibrillation. Echocardiography is a reliable method with which to predict tumor size and morphology. Myxoma cells usually express IL-6, and some tumors have abnormal cellular DNA content. Surgical excision of the tumor is a safe and effective treatment.     

The Challenge Presented by Right Atrial Myxoma

BACKGROUND AND PURPOSE: Right atrial myxoma can be heralded by nonspecific constitutional symptoms-i. e., remittent or lasting fever; weight loss; chronic anemia and general arthralgia-and may escape timely diagnosis until severe complications develop: i. e., pulmonary hypertension due to embolism from fragments originating from the tumor mass, or, by blocking the right atrioventricular ostium, a Budd-Chiari syndrome with acute abdominal pain. Myocardial tamponade and infected right atrial myxomas are reported casuistically. META-ANALYSIS: This study, covering 1,029 patients from 32 reports, represents the most extensive meta-analysis to date of recent publications on patients with intracardiac myxomas who had undergone surgery followed by histological confirmation. 83.0% of myxomas studied were located in the left and 12.7% in the right atrial cavity. Even more rarely, the tumor was present in the left (0.6%) or right (1.7%) ventricles; it was biatrial in 1.3% and multilocular in 0.8% of cases. The age of all reported individuals varied between 1 month and 81 years (mean value of reported mean ages: 50 years); 65% of the patients were female. In a subpopulation of 154 individuals, the fraction of familial cardiac myxomas was 4.5%. CASE STUDY: For a 68-year-old patient with recurrent fever of unknown origin, weight loss, exertional dyspnea, and tachycardia, the authors further describe epidemiology, clinical symptoms, prognosis, and the specific diagnostic and therapeutic challenges involved in the rare case of a right sided atrial myxoma.     

Morbidity and embolic potential of left atrial cardiac tumors

BACKGROUND: The aim of the study was to correlate cardiac tumors with implantation site, embolic potential and long-term evolution in patients undergoing surgery. METHODS: From 1981 to 2004, 78 patients underwent surgery for cardiac tumors. 64 % were female, with a mean age of 44 years. 14 % had embolic episodes (91 % CVA). The sizes of the tumors ranged from 0.4 cm to 10 cm. The most frequent tumors were myxomas (70 %), papillary fibroelastomas and fibromas. The preferential location was the left atrium (72 %). Data were analyzed using chi (2) and Student's T-tests, and the Kaplan-Meyer method and curves were compared with the log-rank test. RESULTS: Hospital mortality was 6 % and 8.2 % in the follow-up period. Myxoma was predominantly found in the left atrium ( P < 0.0001; OR = 12.8 and 4 - 41 95 % CI). Left atrial tumors showed less mortality ( P = 0.1; OR = 3 and 0.7 - 11.6 95 % CI), with a lower mortality for myxomas ( P < 0.001), fewer recurrences ( P = 0.03; OR = 8.7 and 0.8 - 89 95 % CI), and a greater embolic potential than in other locations ( P = 0.03 % OR = 0.7 and 0.6 - 0.8 95 % CI). CONCLUSION: Left atrial tumors showed greater embolic potential, fewer recurrences and less mortality.     

Left atrial myxoma: new perspectives in the diagnosis of murmur free cases.

No cardiological signs or symptoms were seen in three patients with left atrial myxomas. The diagnosis was established by cross sectional echocardiography. In two patients this investigation was performed to exclude a cardiac source of systemic emboli. The third patient had constitutional signs only. All three had raised erythrocyte sedimentation rates and C reactive protein concentrations. In two patients the myxomas were successfully excised; the third patient who had presented with massive peripheral and central embolisation died during emergency operation. Cross sectional echocardiography is the technique of choice for detecting atrial myxomas and the absence of the cardiological signs should not preclude referral for diagnostic echocardiography.     

Left atrial myxoma: new perspectives in the diagnosis of murmur free cases

No cardiological signs or symptoms were seen in three patients with left atrial myxomas. The diagnosis was established by cross sectional echocardiography. In two patients this investigation was performed to exclude a cardiac source of systemic emboli. The third patient had constitutional signs only. All three had raised erythrocyte sedimentation rates and C reactive protein concentrations. In two patients the myxomas were successfully excised; the third patient who had presented with massive peripheral and central embolisation died during emergency operation. Cross sectional echocardiography is the technique of choice for detecting atrial myxomas and the absence of the cardiological signs should not preclude referral for diagnostic echocardiography.     

Asymptomatic recurrence of a left auricular myxoma. Apropos of a case

A case of asymptomatic recurrence, in a 23-year old female patient, of a left atrial myxoma surgically removed 7 years previously is reported. The initial tumour, revealed by lipothymias and progressive heart failure, had been diagnosed by TM-mode echocardiography. The myxoma had been entirely removed, together with part of the atrial septum around its pedicle. The recurrent tumour was discovered by chance during routine control echocardiography. Post-surgical recurrent cardiac myxomas are uncommon (5% of the cases), and they predominate in the left atrium, like the initial tumours. They develop more rapidly and may recur several times in succession, becoming increasingly aggressive and multiple, though most of them remain benign. They are thought to result from the proliferation of myxomatous cells in clusters disseminated within the cardiac walls. Echocardiography is the key to the diagnosis; it is also used for post-surgical follow-up.     

Large left atrial myxoma with severe mitral regurgitation: the inverted T-shaped biatrial incision revisited

Cardiac myxomas are primary cardiac tumors. In some cases of atrial myxoma, the standard left atriotomy alone does not enable safe tumor resection and easy access to the mitral valve. We report the cases of 2 patients with huge left atrial myxomas associated with severe mitral valve regurgitation who underwent an inverted T-shaped biatrial incision for tumor excision and mitral repair. This approach reduces tumor fragmentation and permits good mitral valve exposure.     

Multiple recurrences of nonfamilial cardiac myxomas: a report of two cases

Cardiac myxomas are the most common primary cardiac tumors. Surgical resection usually provides definitive treatment; however, postoperative tumor recurrence has been reported, especially when myxomas occur as part of a familial pattern. Only a few cases of 2nd recurrence of nonfamilial cardiac myxoma have been reported. We report 2 cases of nonfamilial cardiac myxoma, with multiple recurrences after surgical resection. The possibility of repeated recurrence of cardiac myxomas demonstrates the importance of regular echocardiography after surgical resection in order to detect such recurrence. Future studies, including genetic analysis of patients with recurrent cardiac myxomas, are warranted to investigate the nature of these tumors.     

Four cardiac myxomas diagnosed three times in one patient.

A left atrial myxoma was surgically removed in a 58-year-old man following several embolic events. Five months later, a new myxoma was found in the right ventricular outflow tract and surgically removed. In this patient, we visualized two more recurrences 14 years later, one in the left atrium and the other in the left ventricle. A short review of the literature concerning recurrent cardiac myxomas is given.     

Bi-auricular myxoma associated with atrioventricular dissociation in an 18-year-old boy: a case report

Primary cardiac tumours are rarely found and have an incidence of 0.3% in all open-heart surgeries. Among those, approximately 70% are myxomas, most of them in the left atrium. The reported incidence of cardiac tumours in autopsy series is 0.001-0.28%. Right atrial myxomas are uncommon, but when present they often originate from the interatrial septum, and conduction disturbances are rarely noted as an accompanying feature in this condition. We report the case of an 18-year-old boy with a myxoma in both left and right atrium associated with atrioventricular dissociation.     

Diagnosis of noninfective cardiac mass lesions by two-dimensional echocardiography. Comparison of the transthoracic and transesophageal approaches

This study was conducted in 46 patients with cardiac thrombi, 15 patients with atrial myxomas, and 32 patients with other cardiac or paracardiac tumors. Diagnoses were subsequently proven by surgery, autopsy, computed tomography, magnetic resonance imaging, or angiography in all patients. All patients underwent precordial and transesophageal two-dimensional echocardiography to assess the various mass detection rates. Atrial myxomas and predominantly left-sided cardiac tumors were identified by both echocardiographic techniques with comparable detection rates. Left ventricular apical thrombi were detected more frequently by precordial echocardiography. In contrast, transesophageal echocardiography was superior in visualizing left atrial appendage thrombi, small and flat thrombi in the left atrial cavity, thrombi and tumors in the superior vena cava, and masses attached to the right heart and the descending thoracic aorta. These data indicate that transesophageal echocardiography leads to a clinically relevant improvement of the diagnostic potential in patients in whom cardiac masses are suspected or have to be excluded in order to ensure the safety of clinical procedures.     

64例心脏粘液瘤外科治疗分析

本文报道64例心脏粘液瘤的手术治疗。术中发现粘液瘤在左心房56例,右心房7例;左心室1例。左心房粘液瘤中有2例为恶性粘液瘤。64例肿瘤均在体外循环直视下切除。术前确诊依靠超声心动图和多普勒检查,手术后死亡3例,死亡率为4.7%。     

Myxoid leiomyosarcoma of the left atrium: a rare malignancy of the heart and its comparison with atrial myxoma

Primary tumours of the heart are rare. The majority of these tumours are benign, with myxomas located in the left atrium being the most common form. Almost all malignant tumours are sarcomas and occur preferentially in the right side of the heart. An exception to this rule is leiomyosarcoma, a rare form of primary cardiac sarcoma that occurs predominantly in the left atrium, as does cardiac myxoma. The case of a 53-year-old woman who presented with symptoms of mitral valve stenosis and pulmonary hypertension is reported. Cardiac catheterization, angiography and echocardiography revealed a left atrial mass that was interpreted as atrial myxoma. At the time of operation, the myxoid appearance of the tumour mass further supported this assumption. The tumour, including a wide rim of atrial septum, was removed with cautery. Histopathological examination unexpectedly showed that the tumour was not an atrial myxoma but rather a myxoid variant of a primary leiomyosarcoma. Immunohistochemistry and electron microscopy confirmed the diagnosis. Local radiotherapy was considered but deemed contraindicated in view of the longstanding pulmonary hypertension. Two months after excision, a repeat echocardiogram indicated recurrence of tumour in the left atrium, and the patient died a few days later. The preferential left atrial location and the frequently myxoid appearance of primary leiomyosarcomas of the heart make it particularly difficult to differentiate them preoperatively from atrial myxomas. The authors recommend resection of all atrial myxoid tumours with a wide (at least 1 cm) margin, combined with intraoperative frozen section diagnosis, because complete surgical resection appears to correlate with prolonged survival in the few reported cases of atrial leiomyosarcomas. In cases of incomplete initial resection or local recurrence in the absence of metastatic disease, heart transplantation may be a valid option in appropriately selected patients.     

163例心脏肿瘤临床分析

目的 :总结心脏肿瘤的临床特征 ,提高对心脏肿瘤的认识。方法 :回顾性分析了 163例心脏肿瘤的临床表现、肿瘤发生部位及肿瘤性质等。结果 :心脏肿瘤最常见临床表现是心悸和气短。心脏肿瘤可发生于心脏任何部位 ,左心房最常见 ,且多为粘液瘤 ,其次是右心房。右心肿瘤除粘液瘤外 ,多为恶性肿瘤。 163例心脏肿瘤中原发性肿瘤 15 9例 (97.5 % ) ,其中粘液瘤 12 8例(78.5 % ) ,非粘液良性肿瘤 17例 (10 .4% ) ,恶性肿瘤 18(11.0 % )。结论 :原发心脏肿瘤中左心房粘液瘤最多见 ,良性心脏肿瘤手术治疗效果好 ,恶性心脏肿瘤预后差。     

Right atrial trans-septal approach for left atrial myxomas--nine-year experience

OBJECTIVE: In this single-center study we reviewed our experience with left atrial myxomas occurring over the past 9 years. METHODS: Sixty-three patients underwent excision of cardiac myxomas between 1995 and 2004. Of these, 56 patients (32 females and 24 males) had left atrial (LA) myxoma. The mean age was 37.80+12.97 years (range 3.5-67 years). Echocardiography was the only diagnostic evaluation done. The preferred approach for resection was right atrial trans-septal. Annual echocardiographic evaluation was undertaken following surgery. Follow-up is current and available in all the survivors (range 4 months-9 years). RESULTS: Clinically 75% of the LA myxomas simulated mitral stenosis. The symptoms were present for 2-6 months before operation. Most (86%) LA myxomas were attached to the fossa ovalis. Few (14%) originated from the LA wall, mitral valve annulus and anterior mitral leaflet. One patient succumbed to low cardiac output and another died of massive embolic stroke following surgery. There were no late deaths. Two patients (3.7%) developed left hemiparesis after operation but recovered completely. There was one (1.9%) recurrence 3 years after surgery. Atrial fibrillation occurred in one patient. Mitral insufficiency which was seen in two (3.7%) patients prior to surgery subsided following excision of the tumor. Postoperatively 94% patients remained without symptoms. CONCLUSION: Owing to the risk of valvular obstruction or embolization early surgery is indicated. Right atrial trans-septal approach is safe and easy. Most patients are asymptomatic following surgery. A yearly follow-up is essential.     

The echocardiographic spectrum of atrial myxoma: a ten-year experience

We analyzed the echocardiograms of 28 patients with a left atrial myxoma and two with a right atrial myxoma. Our purpose was to evaluate the value of echocardiography for the diagnosis of these cardiac masses. Only 59% of the m-mode echocardiograms in patients with a left atrial myxoma showed the characteristic findings of multiple diastolic echoes within the mitral orifice as well as abnormal systolic echoes within the left atrium. M-mode echocardiograms were atypical for left atrial myxoma in the remaining patients, and a definitive diagnosis could not be established on the basis of this procedure in six of the patients (22%). Two-dimensional echocardiography showed the presence of a left atrial mass in all 16 patients who had the procedure, and aided in understanding the atypical m-mode recordings. There was a close relationship (r =.82) between two-dimensional echocardiographic measurements of the myxomas' size and pathologic measurements. Both right atrial myxomas could be identified on the m-mode echocardiogram. Our experience indicates that two-dimensional echocardiography is superior to the m-mode technique for the diagnosis and characterization of left atrial myxomas.     

Left atrial myxoma in transplanted heart

Primary cardiac tumours are discovered in about 1 in 2000 necropsies. Cardiac myxomas make up half of all benign intracardiac tumours. Seventy five per cent are located in the left atrium. The first left atrial myxoma arising from the left atrium of a transplanted heart is reported.     

A case of left atrial myxoma in the elderly (77-year-old)

We reported a case of a 77-year-old female, whose left atrial myxoma with a cyst, which had been identified by 2D echocardiography, was successfully resected. Apart from echocardiogram-revealed tumor hemorrhages in atrial myxomas, reports of echo-lucency within cardiac tumors are considerably rare. She was successfully operated on, and the section through the tumor specimen showed a cyst with a diameter of 10 mm, that corresponded to the echo-lucencies noted on the 2D echocardiogram. Even in an elderly patient, surgical treatment of left atrial myxoma offers excellent results with low morbidity and low mortality.