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      Cardiac Myxoma and Cerebrovascular Events: A Retrospective Cohort Study

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          Abstract

          Background: Cardiac myxoma (CM) is the most frequent, cardiac benign tumor and is associated with enhanced risk for cerebrovascular events (CVE). Although surgical CM excision is the only curative treatment to prevent CVE recurrence, in recent reports conservative treatment with antiplatelet or anticoagulant agents in high-risk patients with CM-related CVE has been discussed.

          Methods: Case records at the University Hospital of Tübingen between 2005 and 2017 were screened to identify patients with CM-related CVE. Clinical features, brain and cardiac imaging findings, histological reports, applied treatments and long-term neurological outcomes were assessed.

          Results: 52 patients with CM were identified and among them, 13 patients with transient ischemic attack, ischemic stroke or retinal ischemia were included to the (to our knowledge) largest reported retrospective study of CM-related CVE. In all identified patients, CVE was the first manifestation of CM; 61% suffered ischemic stroke, 23% transient ischemic attack and 15% retinal ischemia. In 46% of the patients, CVE occurred under antiplatelet or anticoagulation treatment, while 23% of the patients developed recurrent CVE under bridging-antithrombotic-therapy prior to CM surgical excision. Prolonged time interval between CVE and CM-surgery was significantly associated with CVE recurrence ( p = 0.021). One patient underwent i.v. thrombolysis, followed by thrombectomy, with good post-interventional outcome and no signs of hemorrhagic transformation.

          Discussion: Our results suggest that antiplatelet or anticoagulation treatment is no alternative to cardiac surgery in patients presenting with CM-related CVE. We found significantly prolonged time-intervals between CVE and CM surgery in patients with recurrent CVE. Therefore, we suggest that the waiting- or bridging-interval with antithrombotic therapy until curative CM excision should be kept as short as possible. Based on our data and review of the literature, we suggest that in patients with CM-related CVE, i.v. thrombolysis and/or endovascular interventions may present safe and efficacious acute treatments.

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          Most cited references38

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          Cardiac myxomas.

          K Reynen (1995)
          Although cardiac myxomas are histologically benign, they may be lethal because of their strategic position. They can mimic not only every cardiac disease but also infective, immunologic, and malignant processes. Myxomas must therefore be included in the differential diagnosis of valvular heart disease, cardiac insufficiency, cardiomegaly, bacterial endocarditis, disturbances of ventricular and supraventricular rhythm, syncope, and systemic or pulmonary embolism. The symptoms depend on the size, mobility, and location of the tumor. Echocardiography, including the transesophageal approach, is the most important means of diagnosis; CT and MRI may also be helpful. Coronary arteriography in patients over 40 years of age is generally required to rule out concomitant coronary artery disease. Surgical removal of the tumor should be performed as soon as possible; the long-term prognosis is excellent, and recurrences are rare. In follow-up examinations as well, echocardiography is essential.
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            Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases.

            We analyzed a series of 112 consecutive cases of left atrial myxoma diagnosed in a single French hospital (72 women and 40 men; age range, 5-84 yr) over 40 years, from 1959 to 1998. Symptoms of mitral valve obstruction, the first arm of the classic triad of myxoma presentation, were present in 75 patients (67%), with mostly cardiac failure or malaise. Symptoms of embolism, the second frequent presentation in the classic triad, were observed in 33 cases (29%) with 1 or several locations, essentially cerebral emboli with stroke. Males are statistically at greater risk than females of developing embolic complications. The third arm of the classic triad consists of constitutional symptoms (34%) with fever, weight loss, or symptoms resembling connective tissue disease, due to cytokine (interleukin-6) secretion. Younger and male patients have more neurologic symptoms, and female patients have more systemic symptoms. Seventy-two patients (64%) had cardiac auscultation abnormalities, essentially pseudo-mitral valve disease (53.5%) and more rarely the suggestive tumor plop (15%). The most frequent electrocardiographic sign was left atrial hypertrophy (35%), whereas arrhythmias were uncommon. The greater number of myxoma patients (98) diagnosed preoperatively after 1977 reflects the introduction of echocardiography as a noninvasive diagnostic procedure. However, there was no significant reduction in the average time from onset of symptoms to operation between patients seen in the periods before and after 1977. The tumor diameter ranged from 1 to 15 cm with a weight of between 15 and 180 g (mean, 37 g). The myxoma surface was friable or villous in 35% of the cases, and smooth in the other 65% cases. Myxomas in patients presenting with embolism have a friable surface; those in patients with cardiac symptoms, pseudo-mitral auscultation signs, tumor plop, and electrocardiogram or radiologic signs of left atrium hypertrophy and dilatation are significantly the larger tumors. The long-term prognosis is excellent, and only 4 deaths occurred among our 112 cases over a median follow-up of 3 years. The recurrence rate is low (5%), but long-term follow-up and serial echocardiography are advisable especially for young patients.
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              Cardiac myxomas: 24 years of experience in 49 patients.

              In this single-center study we reviewed our experience with a significant number of cardiac myxoma cases occurring over the past two decades. 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. 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. 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.
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                Author and article information

                Contributors
                Journal
                Front Neurol
                Front Neurol
                Front. Neurol.
                Frontiers in Neurology
                Frontiers Media S.A.
                1664-2295
                03 October 2018
                2018
                : 9
                : 823
                Affiliations
                [1] 1Department of Neurology and Stroke, and Hertie-Institute for Clinical Brain Research, Eberhard-Karls University of Tübingen , Tübingen, Germany
                [2] 2Department of Cardiology, Eberhard-Karls University of Tübingen , Tübingen, Germany
                [3] 3Department of Diagnostic and Interventional Neuroradiology, Eberhard-Karls University of Tübingen , Tübingen, Germany
                [4] 4Department of Thoracic and Cardiovascular Surgery, Eberhard-Karls University of Tübingen , Tübingen, Germany
                Author notes

                Edited by: Yannick Béjot, Centre Hospitalier Regional Universitaire De Dijon, France

                Reviewed by: Sebastian Kelle, Deutsches Herzzentrum Berlin, Germany; Wolfgang Boehmerle, Charité Medizinische Universität Berlin, Germany

                *Correspondence: Annerose M. Mengel annerose.mengel@ 123456med.uni-Tübingen.de

                This article was submitted to Stroke, a section of the journal Frontiers in Neurology

                Article
                10.3389/fneur.2018.00823
                6178925
                30337904
                a077216b-57ca-4c53-a7f8-1e6a2fdbc7d7
                Copyright © 2018 Stefanou, Rath, Stadler, Richter, Hennersdorf, Lausberg, Lescan, Greulich, Poli, Gawaz, Ziemann and Mengel.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

                History
                : 13 July 2018
                : 11 September 2018
                Page count
                Figures: 6, Tables: 1, Equations: 0, References: 41, Pages: 10, Words: 6533
                Categories
                Neurology
                Original Research

                Neurology
                cardiac myxoma,cerebrovascular events,stroke,cerebral ischemia,cardiac surgery
                Neurology
                cardiac myxoma, cerebrovascular events, stroke, cerebral ischemia, cardiac surgery

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