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      Eleven Years’ Experience with Korean Cardiac Myxoma Patients: Focus on Embolic Complications

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          Abstract

          Background: Cardiac myxomas are rare but are the most common cardiac tumors. This study is based on our clinical experience with cardiac myxomas over a period of 11 years at Sejong General Hospital. We focused on the embolic complications of patients with cardiac myxoma. Methods: We retrospectively reviewed the medical records of 59 consecutive patients with cardiac myxoma who were treated between January 2000 and March 2011. The myxomas were divided into two types: type 1, with an irregular or villous surface and a soft consistency, and type 2, with a smooth surface and a compact consistency. The 59 investigated patients were classified into the embolic group and the non-embolic group. Results: Cardiac obstructive symptoms, embolic events and constitutional symptoms were observed in 37 (62.7%), 13 (22.0%) and 10 (16.9%) patients, respectively. When the embolic and non-embolic groups were compared, there were no significant differences in vascular risk factors, the ejection fraction, the left atrial diameter or the tumor size. However, type 1 myxomas were significantly more frequent in the embolic group (p = 0.009 by Fisher’s exact test). A binary logistic regression analysis showed that type 1 pathology alone was independently associated with myxoma-related embolism (p = 0.008; odds ratio 10.056; 95% confidence interval 1.828–55.337). There were no operative deaths in any of the 59 patients studied. Among the 13 patients with embolism, 11 (84.6%) had brain infarcts. The main patterns of the lesions were multiple lesions (8 out of 11 patients, 72.7%) and lesions in the middle cerebral artery territories (7 out of 11 patients, 63.6%). The other 2 patients were found to have occlusion of the left central retinal artery and left external iliac artery. Additionally, incidental cerebral aneurysms were found in the latter case. There was no recurrence of myxoma or myxoma-related symptoms in the 53 patients receiving outpatient management during the follow-up period (range 2 months to 11 years). Conclusions: The embolic potential of myxoma was associated with an irregular surface pathology but not with vascular risk factors. Echocardiography should be performed in patients with embolic events, especially when cerebral infarcts with multiple territorial lesions are detected. Surgical resection is a relatively safe and curative procedure for cardiac myxoma.

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          The complex of myxomas, spotty pigmentation, and endocrine overactivity.

          Of 40 patients (16 males and 24 females), 29 had cardiac myxoma(s), 14 had skin pigmentation (lentigo and several types of nevi) which also commonly affected the lips, 6 had skin myxoma(s), and 12 had both pigmentation and myxoma(s); 18 had primary pigmented nodular adrenocortical disease (Cushing syndrome was present in 11); 10 had myxoid mammary fibroadenomas; 9 had testicular tumor(s) (large-cell calcifying Sertoli cell tumor, Leydig cell tumor, or adrenocortical rest tumor, or a combination); and 4 had pituitary adenoma with gigantism or acromegaly. The maximum number of conditions present together was five, occurring in two patients; each of the remaining patients had at least two of the conditions. The overlap, in this sizeable number of patients, of various combinations of the same rare or very rare conditions unlikely to occur together by chance with any degree of frequency is striking evidence for a unique syndrome. The patients were young (mean age at diagnosis of the first component, 18 years). Pathologic involvement tended to be multicentric (heart and skin) and bilateral in paired organs (adrenal, breast, and testis). Thirteen patients (32%) are alive and well. Twelve patients are alive but with complications of cardiac myxoma (in 8), testicular tumors (in 2), residual Cushing syndrome (in 1), or bilateral pulmonary nodules (in 1). Twelve patients are dead: 9 of cardiac myxoma, 1 of intracranial (nonpituitary) tumor, and 2 postoperatively. The status of three is unknown.
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            Central nervous system manifestations of cardiac myxoma.

            Neurologic complications can be the initial manifestation of atrial myxoma. Prompt diagnosis is of paramount significance to prevent recurrent complications. To identify patients with neurologic complications attributed to atrial myxoma. With institutional review board approval, we retrospectively reviewed the medical records of 74 consecutive patients with pathologically confirmed cardiac myxoma at the Mayo Clinic from January 1, 1993, through December 31, 2004. Discharge and follow-up modified Rankin score. Nine of the 74 patients with cardiac myxoma (12%) presented with neurologic manifestations in the setting of atrial myxoma. Mean age was 48.5 years (range, 17-70 years). There were 6 females and 3 males. Among patients with myxoma and neurologic symptoms, ischemic cerebral infarct was the most common neurologic manifestation (8 patients [89%]). No patients had concomitant cardiac symptoms. The size of the atrial myxoma was variable, with a mean diameter of 2.7 (range, 0.4-6.5) cm. Most of the atrial myxomas causing neurologic symptoms demonstrated a mobile component on transesophageal echocardiography (8 patients [89%]). Two patients (22%) had pathologic evidence of systemic myxomatous emboli. One patient with intracerebral hemorrhage had pathologically confirmed intracranial metastatic myxoma and myxoma-induced aneurysmal dilatation. Neurologic complications are associated with cardiac myxoma in some patients with myxoma and, when they occur, frequently present with cerebral infarction. The mobility, not the size, of the myxoma appears to be related to embolic potential. Potential delayed neurologic complications relevant to patients with tumor embolization include myxoma-induced cerebral aneurysm and myxomatous metastasis, which can mimic the clinical picture of central nervous system vasculitis or infective endocarditis.
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              Cardiac myxoma: 40 years' experience in 63 patients.

              Cardiac myxomas were resected in 63 patients, including the first successful procedure in the world. Patient data and data obtained during follow-up of the survivors were reviewed. Preoperatively, valve obstruction occurred in 56% and emboli in 32% of the patients. The myxomas were located in the left atrium in 88%, right atrium in 10%, and both atria in 1 patient (1.6%). One patient died early, and 5 died late. The 20-year survival rate was 85%. At a median of 13 years (range, 1 month to 42 years) after resection, 26% of the survivors reported having various cardiopulmonary symptoms, 5% had had thromboembolic events, and 69% were asymptomatic. After 2 years, there was a recurrence in a young woman who had had a multifocal myxoma at the time of the primary operation. No other evidence of recurrence was found at autopsy or during repeat echocardiography in the remaining patients during an observation period of 787 patient-years. Surgical resection of a single myxoma is a safe and effective treatment, with a low risk of recurrence. After uncomplicated resection, the frequency with which postoperative echocardiography is performed should be limited.
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                Author and article information

                Journal
                CED
                Cerebrovasc Dis
                10.1159/issn.1015-9770
                Cerebrovascular Diseases
                S. Karger AG
                1015-9770
                1421-9786
                2012
                May 2012
                19 April 2012
                : 33
                : 5
                : 471-479
                Affiliations
                Departments of aNeurology and bThoracic and Cardiovascular Surgery, Sejong General Hospital, Bucheon, Republic of Korea
                Author notes
                *Dr. Seung-Jae Lee, Department of Neurology, Sejong General Hospital, 91–121 Sosabon2-dong, Sosa-gu Bucheon-si, Gyeonggi-do 422-711 (Republic of Korea), Tel. +82 32 340 1136, E-Mail neurosj@catholic.ac.kr
                Article
                335830 Cerebrovasc Dis 2012;33:471–479
                10.1159/000335830
                22517375
                262de4d8-75c6-48e0-a762-ebaaf7e26f3f
                © 2012 S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                : 27 June 2011
                : 12 December 2011
                Page count
                Figures: 2, Tables: 4, Pages: 9
                Categories
                Original Paper

                Geriatric medicine,Neurology,Cardiovascular Medicine,Neurosciences,Clinical Psychology & Psychiatry,Public health
                Embolic complication,Ischemic stroke,Myxoma

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