Bilateral atrial myxomas with multiple arterial aneurysms--a syndrome mimicking polyarteritis nodosa.

This patient, previously described as having polyarteritis nodosa with multiple aneurysms shown by angiography, was recently found to have bilateral atrial myxomas. The angiographic demonstration of multiple cerebral aneurysms has been described in a few cases of left atrial myxoma. This uncommon tumor is also accompanied by "constitutional" manifestations. Thus, a syndrome mimicking polyarteritis nodosa may be produced. Cardiac myxoma should be borne in mind in conditions with multifocal organic involvement, and they should be included in the list of conditions causing multiple arterial aneurysms.     

Clinical features and surgical treatment of cardiac myxoma: report of 18 cases

Myxomas are the most common benign primary cardiac tumors. As many patients with cardiac myxoma suffer from cerebral or systemic embolism, which are serious complications, diagnosis of the tumor is vital. Between 1990 and 2000, 18 patients (6 males, 12 females), aged 24 to 73 years (mean, 55.3 years), were operated on for cardiac myxoma. The most common location of the myxoma was the left atrium (78%), and the transseptal surgical approach was preferred (78%) as it allows total resection of the left atrial myxoma along with its pedicle. Carney complex, a familial autosomal dominant form of atrial myxoma, was not found in any of the patients. There had been no operative or postoperative mortality and morbidity. The mean postoperative follow-up period was 5 years (mean, 1 to 10 years). No recurrence had been seen. We believe that the transseptal approach, in allowing total resection of the myxoma, prevents recurrence.     

Left Atrial Myxoma Causing Coronary Steal: An Atypical Cause of Angina

Cardiac myxomas are rare primary cardiac tumors that usually present with dyspnea or manifestations of systemic embolization. Coronary steal is a rare phenomenon of unbalanced blood flow that is seen primarily in patients who have undergone coronary artery bypass grafting and have subclavian artery stenosis.We report the case of a 72-year-old woman who presented with fatigue, weakness, and exertional chest heaviness and had abnormal results on a cardiac stress test. The results of coronary angiography showed no obstructive coronary artery disease but revealed a large intracardiac left atrial mass that was supplied by 2 anomalous coronary arteries. The patient underwent successful ligation of the anomalous coronary arteries and resection of the mass, which was histologically an atrial myxoma. The patient''s symptoms resolved, and results of a repeat cardiac stress test were normal.To our knowledge, this is the first report of a highly vascularized atrial myxoma that caused coronary steal with objective evidence of ischemia, and with subsequent resolution after resection of the mass and ligation of the anomalous coronary arteries.     

Familial atrial myxoma.

Two siblings 14 years apart were found to have atrial myxomas. The brother's right atrial myxoma was found at age 21 and his sister's left atrial myxoma, with peripheral cerebral and coronary aneurysms, at age 30. Both tumors were excised successfully. These findings support previous reports suggesting that atrial myxoma may be familial. Echocardiography can be used effectively in the diagnosis of atrial myxoma, detection of its possible recurrence, and screening other members of the family.     

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.     

Right atrial cardiac myxoma and multiple pulmonary artery aneurysms: Is there a causal relationship?

Cardiac myxomas are rare occurrences in pediatric populations, as are pulmonary artery aneurysms. We report a 17‐year‐old adolescent with right atrial cardiac myxoma and concomitant multiple peripheral pulmonary artery aneurysms. Histological examination indicated infiltration of the pulmonary artery wall through the embolic cardiac myxoma cells, thereby weakening it. This report highlights the probable causal relationship between these two entities, proposes the possible pathomechanism and presents the diagnostic and therapeutic dilemmas in the management of this condition.     

Neovascularized Myxoma‐Causing Abnormal Blood Flow in the Left Atrium Diagnosed by Transesophageal Echocardiography

Myxomas are the most common primary cardiac tumor, and neovascularization of cardiac myxoma has been shown in previous case reports. However, it is seldom reported that abnormal blood flow spurted from a neovascularized myxoma into the cardiac chamber. In this unusual case report, we present a left atrial myxoma in a 44‐year‐old woman with atypical angina. Transesophageal echocardiography (TEE) revealed several small blood streams spurting from the surface of massively vascularized myxoma into the left atrium. Coronary angiography showed a feeding artery which arose from the right coronary artery and supplied the left atrial myxoma, with no evidence of coronary artery stenosis. This case demonstrated that TTE might be helpful for confirming the neovascularization of myxoma in the left atrium and finding the abnormal blood flow that spurts from the neovascularized myxoma. We speculate that the abnormal blood flow spurting from the neovascularized myxoma into the left atrium may have caused a coronary steal phenomenon thus resulting in myocardial ischemia.     

Cardiac Myxoma With Unusual Obstructive and Embolic Presentations: Concurrent Stroke and Angiography-Negative Myocardial Infarction—A Case Report

We present a case of cardiac myxoma with atypical presentations of concurrent stroke and angiography-negative myocardial infarction. The case emphasizes the importance of basic echocardiography and timely surgery in the management of cardiac myxoma.An elderly woman presented to the emergency department in an unconscious state. Electrocardiogram and elevated cardiac enzymes suggested acute myocardial infarction; however, immediate coronary angiography proved patency. Basic echocardiography revealed an oscillating left atrial myxoma obstructing inflow through the mitral valve. After regaining consciousness while in the intensive care unit, the patient developed respiratory distress and shock, and emergent en bloc resection was performed. Ataxia was noted in her postoperative course and multiple small cerebellar infarcts were found on magnetic resonance imaging. After a 1-month period of rehabilitation, the patient recovered well and continues to be followed as an outpatient.Cardiac myxoma requires timely management and may be missed if not included in the differential diagnoses. Basic echocardiography, also called focused cardiac ultrasound, may aid in the diagnosing of perplexing cardiac cases.     

Coronary angiography of cardiac myxoma

BACKGROUND: Myxoma usually presents with nonspecific symptoms. Preoperative coronary angiography is presently only considered if patients are at increased risk of coronary artery disease (CAD). The angiographic patterns of cardiac myxoma have not been fully described. HYPOTHESIS: The aim of our study was to investigate coronary angiograms as well as patterns of tumor vascularity in patients with cardiac myxoma. METHODS: From January 1990 to December 2003, 33 patients with cardiac myxoma, who had received surgical resection at our hospital, were enrolled; of these, 9 patients underwent preoperative coronary angiography. The severity and extent of coronary artery stenosis, as well as tumor angiographic patterns, were analyzed. Coronary artery disease is defined as a > 50% stenosis in diameter at any segment of the coronary artery viewed by two orthogonal views on cineangiogram. RESULTS: Four (44.4%) patients showed concomitant CAD. The presence of coronary risk factors was not different between patients with and without CAD. Contrast media-enhanced tumor vasculature was found in five (55.6%) patients. Four (80%) patients had multiple feeding arteries. There was the characteristic "sea anemone" appearance of the tumor vasculature composed of (1) basal vascular network, (2) vessel stem, (3) backbone branches, and (4) dye brushes. These findings were characteristic of cardiac myxoma. CONCLUSION: Coronary angiography can detect the concomitant coronary disease and the unique vascular appearances of cardiac myxoma. Familiarity with the "sea-anemone" angiographic findings may help in the diagnosis of cardiac myxoma.     

Coronary angiography in cardiac myxomas: Findings in 19 consecutive cases and review of the literature

We reviewed the coronary angiographic findings of 19 patients with a cardiac myxoma, who underwent cardiac catheterization before surgery. Seventeen myxomas were localized in the left atrium and seven had angiographically visible tumor vascularity emerging from atrial branches of the right coronary artery in four patients and the circumflex coronary artery in three. In one patient, we found significant coronary artery disease of the circumflex coronary artery and in another we saw a thrombus-like lesion in the proximal third of the left anterior descending coronary artery. Our results are compared with the findings in two smaller groups of patients with cardiac myxoma who underwent coronary angiography preoperatively. We conclude that the major importance of coronary angiography in patients with cardiac myxomas is to exclude concomitant coronary artery disease before surgery. In a very small minority of patients, a selective coronary angiography is the clue to the diagnosis of cardiac myxoma. © 1993 Wiley-Liss, Inc.     

Familial recurring cardiac myxoma

A 34-year-old woman presented with her fourth occurrence of an atrial myxoma and a right lower lung field mass. Her surgical history was extensive. In addition to resection of three previous atrial myxomas, she had undergone resection of her adrenal glands as a teenager because of Cushing's syndrome, and a hysterectomy at age 26 revealed a myxoid leiomyoma. Family history was remarkable as a maternal uncle and daughter died from embolic complications of left atrial myxomas and her sister previously had a left atrial myxoma resected. The patient underwent uncomplicated removal of the myxoma, and resection of the lung mass revealed a granuloma. A review of typical and atypical aspects of cardiac myxomas is provided including a rare and recently described syndrome of familial cardiac myxoma associated with Cushing's syndrome, spotty skin pigmentation and other myxoid tumors.     

Infected left atrial myxoma with bacteremia simulating infective endocarditis.

A 58-year-old man had intermittent fever of eight months' duration following a dental extraction. There were no abnormal cardiac auscultatory findings. Multiple blood cultures yielded Streptococcus mutans. Treatment for infective endocarditis was initiated; however, an echocardiogram suggested the presence of a left atrial myxoma. The diagnosis was confirmed by angiography and the infected tumor was removed successfully. Differentiating features between left atrial myxoma and mitral valve endocarditis may not be obvious clinically, and bacteremia does not preclude atrial myxoma as a diagnostic possibility. We therefore suggest that all cases of infective endocarditis be evaluated by echocardiography to elucidate lesions such as large vegetations or left atrial myxoma, both of which may require urgent operative intervention.     

Left atrial myxoma presenting with acute myocardial infarction--a case report

A 38-year-old man presented with an acute anterior myocardial infarction (MI) and was subjected to thrombolysis. Echocardiography and cardiac catheterization revealed a mass in the left atrium that was considered to be a myxoma. The coronary arteries appeared normal on angiography. The left atrial mass was removed surgically without complication. Histopathologic examination confirmed the diagnosis of the myxoma. Coronary embolism secondary to the myxoma was thought to be the cause of the MI.     

Left Atrial Myxoma with versus without Cerebral Embolism: Length of Symptoms,Morphologic Characteristics,and Outcomes

The aim of this study was to evaluate the embolic sequelae of left atrial myxomas and their influence on diagnosis, treatment, and prognosis.Seventy-eight patients were retrospectively investigated. According to their symptoms and neurologic-imaging findings, these patients were classified into 2 groups: embolism (15 patients, 19%) and nonembolism (63 patients, 81%). The time from the first onset of symptoms to diagnosis (that is, the duration of symptoms) was significantly longer in the embolism group than in the nonembolism group (105 ± 190 vs 23 ± 18 d; P <0.01). The myxomas were divided into 2 types on the basis of clinicopathologic findings: type 1, with an irregular or villous surface and a soft consistency, and type 2, with a smooth surface and a compact consistency. There were 42 patients with type 1 myxoma and 36 with type 2. Type 1 myxoma was more frequently found in the embolism group (12 patients, 29%) than was type 2 myxoma (3 patients, 8%). The difference was significant (P=0.04). There were 2 perioperative deaths in the nonembolism group. No recurrence of cardiac myxoma or death was recorded in either group during follow-up. In the embolism group, neurologic symptoms were relieved by surgery, and no subsequent neurologic event was reported.Because surgical resection is highly effective in left atrial myxoma, we should strive for early diagnosis in order to shorten the duration of symptoms and to avoid worse neurologic damage in patients in whom an embolic event is the initial manifestation.     

Varied manifestations of left atrial myxoma and the relationship of echocardiographic patterns of tumor size.

Four cases of left atrial myxoma with varied clinical manifestations were diagnosed with echocardiographic studies prior to cardiac catheterization and surgery. The unusual clinical features include Raynaud's phenomenon and intermittent claudication. The echocardiographic features of the atrial myxoma appear to be related to tumor size. Systematic echocardiographic evaluation of patients with mitral valvular disease and systemic and cerebral vascular insufficiency will undoubtedly increase the positive diagnostic results in patients with atrial myxomas. Removal of these tumors results in normalization of the echocardiographic pattern.     

An interleukin-6-producing cardiac myxoma associated with mediastinal lymphadenopathy

We report our experience with a patient whose mediastinal lymphadenopathy resolved after resection of a cardiac myxoma that secreted interleukin-6 (IL-6). The patient was a 68-year-old female who complained of nocturnal chest discomfort related to congestive heart failure. An echocardiogram demonstrated a large left atrial mass. A computed tomogram showed not only the left atrial mass but multiple enlarged mediastinal lymph nodes. The serum IL-6 level was markedly elevated at 13.7 pg/ml. After resection of the cardiac myxoma, serum IL-6 returned to the normal range. A repeat computed tomogram showed no mediastinal lymphadenopathy. We believe that overproduction of IL-6 by the cardiac myxoma was the cause of the mediastinal lymphadenopathy.     

The diagnosis and clinical importance of tumour neovascularisation from coronary artery to right atrial myxoma

Myxomas are the commonest cardiac tumours and are usually localised in the atria. Neovascularisation in cardiac myxomas has been shown in previous case reports. However, the clinical importance of neovascularisation in cardiac myxomas is not well understood. In our case report, we present a right atrial myxoma in a 46 year-old woman admitted to our hospital with exertional angina and dyspnea. Coronary angiography revealed the presence of tumour neovascularisation from the right coronary artery, with no evidence of coronary artery stenosis. We thus speculate that neovascularisation of myxoma may cause typical anginal symptoms as a result of coronary steal phenomenon. Coronary angiography might help in the evaluation of the neovascularisation process and also in indicating surgery.     

心脏粘液瘤误诊16例诊治的体会

目的:探讨心脏粘液瘤误诊的原因,提高其正确诊断率。方法:回顾性分析我院16例心脏粘液瘤误诊的情况。结果:16例心脏粘液瘤患者中误诊为:脑栓塞6例,癫痫2例,肺部感染2例,血栓栓塞性脉管炎1例,血液病2例,肺栓塞1例,急性心肌梗塞1例,眼底疾病1例;误诊时间3～72d,平均(12±17)d,均经B超确诊。本组1例猝死,15例手术切除粘液瘤,手术患者无死亡。结论:缺乏心脏粘液瘤的认识,未进行B超检查是其误诊的主要原因。其有效治疗为手术切除粘液瘤。     

Rapid Growth of Left Atrial Myxoma after Radiofrequency Ablation

Atrial myxoma is the most common benign tumor of the heart, but its appearance after radiofrequency ablation is very rare. We report a case in which an asymptomatic, rapidly growing cardiac myxoma arose in the left atrium after radiofrequency ablation. Two months after the procedure, cardiovascular magnetic resonance, performed to evaluate the right ventricular anatomy, revealed a 10 × 10-mm mass (assumed to be a thrombus) attached to the patient''s left atrial septum. Three months later, transthoracic echocardiography revealed a larger mass, and the patient was diagnosed with myxoma. Two days later, a 20 × 20-mm myxoma weighing 37 g was excised.To our knowledge, the appearance of an atrial myxoma after radiofrequency ablation has been reported only once before. Whether tumor development is related to such ablation or is merely a coincidence is uncertain, but myxomas have developed after other instances of cardiac trauma.Key words: Atrial fibrillation/prevention & control, catheter ablation/adverse effects, diagnosis, differential, echocardiography, transesophageal, echocardiography, transthoracic, heart neoplasms/ultrasonography, myxoma/diagnosis/surgery, pulmonary veinsRadiofrequency ablation (RFA) is an effective therapy for symptomatic patients with atrial fibrillation.¹ The reported complication rate after RFA is 3.9% to 22%,² including thromboembolism in 2% and an early mortality rate of 2% to 4% among all patients.³ Atrial myxoma is the most common cardiac tumor, but its appearance after RFA is very rare. We report a case in which an asymptomatic, rapidly growing cardiac myxoma arose in a patient''s left atrium after RFA.     

The surgical treatment of cardiac myxomas: 10 years of experience]

Over a ten-year period (September 1980-July 1990) 3172 patients underwent open heart surgery in our hospital. Twenty five patients (0.78%) had a cardiac myxoma. There were 18 female and 7 male patients, mean age of 32.3 +/- 21 years (4-61). Left atrial myxomas were diagnosed in 18 patients, left ventricular myxoma in three, right atrial myxoma in two, and one in right ventricle; there was a biatrial myxoma in one case, another had biatrial and left ventricular myxoma. The clinical manifestations of right sided myxomas was congestive heart failure. Patients with left myxoma frequently presented embolic episodes. Myxoma diagnosis was made by means of chest films, EKG and two-dimensional echocardiography which proved effective in twenty four of our patients; cardiac catheterization was performed in ten of them. Symptoms before diagnosis lasted an average of 16.3 months and mean time from diagnosis to operation was 6.6 days. Different surgical approaches are analyzed and discussed. Concomitant surgical procedures were performed in five patients: mitral annuloplasty in one, mitral valve replacement in four (two biological and two mechanical prosthesis). In eight patients the resulting septal defect due to resection of pedicle was closed with pericardial or dacron patches. There were two operative deaths due to pulmonary emboli in one and multiple organ failure in other. Cardiac myxoma is the more frequent primary neoplasm of the heart; its "benign" nature is doubtful; cure by surgical excision can be considered in vast majority of patients and its morbidity is reasonably low.