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Multiple cerebral infarction as the initial manifestation of left atrial myxoma: a case report and literature review Dujuan Sha, Guofeng Fan & Jun Zhang To cite this article: Dujuan Sha, Guofeng Fan & Jun Zhang (2014) Multiple cerebral infarction as the initial manifestation of left atrial myxoma: a case report and literature review, Acta Cardiologica, 69:2, 189-192, DOI: 10.1080/AC.69.2.3017301 To link to this article: https://doi.org/10.1080/AC.69.2.3017301
Published online: 23 May 2017.
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Date: 12 January 2018, At: 09:56
Acta Cardiol 2014; 69(2): 189-192
Multiple cerebral infarction as the initial manifestation of left atrial myxoma: a case report and literature review Dujuan SHA, MD, PhD; Guofeng FAN, MM; Jun ZHANG, MD Downloaded by [Gothenburg University Library] at 09:56 12 January 2018
Dept. of Emergency, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, Jiangsu Province, China.
Abstract Left atrial myxoma is a rare cause of cardioembolic stroke in adults and when stroke occurs, it is mainly due to arterial embolism in cerebral vessels. Cerebral infarction is the ﬁrst clinical manifestation in one-third of cases. We report a 24-year-old female patient with left atrial myxoma, and no other conventional vascular risk factors such as hypertension, diabetes or hyperlipidaemia. The patient presented with multiple infarctions as the ﬁrst clinical manifestation and was diagnosed with a left atrial myxoma. The literature on similar cases was also reviewed.
Keywords Left atrium – myxoma – embolism – cerebral infarction.
INTRODUCTION Cardiac myxoma accounts for 40% of benign cardiac tumours with approximately 75-90% occurring in the left atrium1. Although the majority are benign lesions, myxoma can cause severe haemodynamic complications. The main complications of left atrial myxoma include tumour tissue necrosis and shedding, resulting in the formation of systemic emboli to the central nervous system, retinal arteries, upper and lower limb arteries and coronary arteries. Among these, the most serious is cerebrovascular embolism2. This paper reports a case of left atrial myxoma complicated with multiple cerebral emboli and a review of the related literature.
CASE REPORT A 24-year-old female developed symptoms during exertion, including right upper extremity weakness and numbness, which did not improve with rest. Because the neurological symptoms persisted, the patient presented to
Address for correspondence: Zhang Jun, Department of Emergency, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, 210008, Jiangsu Province, China. E-mail: [email protected]
Received 17 May 2013; revision accepted for publication 27 November 2013.
a local hospital four days after the onset of symptoms. Brain MRI revealed cerebral infarcts scattered throughout the left hemisphere (figure 1). The patient was not treated with medication in the local hospital and was transferred to our hospital. The patient was hospitalized and physical examination showed: length 1.55 m, weight 52 kg, blood pressure 120/75 mm Hg, regular cardiac rhythm and no heart murmur. Neurological examination revealed that the patient was clear-headed with clear speech, responsive and cooperative; bilateral eye movements were symmetrical; pupils were bilaterally symmetrical and equal in size and diameter (3 mm); light reflex was normal; the right nasolabial groove was slightly shallow, with normal tongue and pharyngeal reflexes; cervical movement was soft without neck resistance; strength in the right upper limb was graded as level 4, and level 5 in the right lower limb. The muscle strength of the left side was level 5 (normal) and physiological reflexes were present with a positive Babinski sign on the right side. There was no history of diabetes mellitus, hypertension, hyperlipidaemia, or smoking. After admission, the patient was treated with antiplatelet aspirin (100 mg, once daily), low-molecular-weight heparin (nadroparin calcium, 4100 IU, subcutaneous injection, every 12 hours) anticoagulation and activating blood circulation (Mailuoning3, a traditional Chinese drug, 20 ml, intravenously guttae, once daily). Laboratory examination revealed: blood sedimentation rate, 50 mm/h; total protein, 89.0 g/L; globulin, 40.1 g/L. Normal values occurred for white blood cells, neutrophilic granulocytes, red blood cells, platelets, haemoglobin, C-reactive protein, D-dimer,
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Fig. 1 Brain MRI showing multiple intracranial cerebral lesions (arrows): T1WI (A); T2WI (B); DWI (C).
IgA, IgG, IgM, blood lipids, homocysteine, anticardiolipin antibodies, autoantibodies, antineutrophil cytoplasmic antibodies, blood coagulation, thyroid function, and tumour indicators. Chest radiography showed no significant abnormalities; electrocardiography revealed sinus tachycardia with a heart rate of 102 bpm, and ST-T segment depression in leads II, III, aVF, and V4–V6. Transthoracic echocardiography revealed that the internal diameter of the left atrium and left ventricle were normal but a round, space-occupying mass was visible in the left atrium, with a pedicle in the atrial-septal space with marked motion. The tumour size was approximately 26 mm × 25 mm and the surface area was approximately 6.95 cm2; mild mitral valve regurgitation was observed. The ultrasound diagnosis was a left atrial space-occupying mass with a high possibility of left atrial myxoma (figure 2.A). Four days after admission, the patient underwent cardiac tumour resection. Intraoperative findings revealed that the tumour was located in the left atrium, with a narrow pedicle in the atrial-septum space. The tumour, sized 25 × 20 × 12 mm3, was completely removed. The surface of the tumour had a light-yellow colour. A large quantity of mucus was seen microscopically within the tumour tissue, with floating star-shaped cells (figure 2.C). The pathological findings confirmed cardiac myxoma. No tumour tissue was found in the incisal margin of the specimen. Six days postoperatively, an echocardiogram showed mild mitral valve regurgitation and a small amount of pericardial effusion (figure 2.B). The patient was discharged 8 days postoperatively without medication.
DISCUSSION Cardiac myxoma is the most common clinical primary cardiac tumour, and approximately 75% of cases are benign. Myxoma is sporadic and can occur at any age, but it is most frequent in middle age, from 30-60 years of age with an average age of 43 years. Women are more susceptible than men with a reported
male to female incidence ratio of 2:3. Several epidemiological studies demonstrated that at least 7% of affected cases are familial. Kirschner et al. found that myxoma may also be an autosomal dominant disease, caused by PRKAR1 α gene mutation on the long arm of chromosome 17 (region 17q22-24) 4. Cardiac myxoma was first reported in 1845. Approximately 10% of atrial myxoma patients show no clinical signs. The symptoms are complex and the pathogenesis involves three main aspects: systemic symptoms, mitral valve obstruction and embolism (including peripheral or intracranial)5. About 30% of atrial myxomas presented systemic symptoms, such as fatigue, fever, arthralgia, erythema and weight loss. Dislodgement of the myxoma near the valve orifice is a cause of obstruction. The association of myxoma and mitral stenosis is extremely rare. When the obstruction occurs near the mitral valve, it leads to mitral stenosis. Mitral stenosis usually presents with significant dyspnoea and can be associated with atrial fibrillation. Arterial embolism is the main clinical manifestation, with a prevalence of 30-40%, especially with left atrial myxoma. It can cause acute abdomen, pain and necrosis of the limbs. Pulmonary embolism occurs in approximately 10% of the patients with myxoma in the right atrium, manifesting as chest pain, dyspnoea and other symptoms. Arterial embolism is the initial sign of left atrial myxoma, owing to a lack of heart symptoms in one-third of patients6. Approximately 83% of arterial embolism occurs in cerebral vessels and 41% of patients have concurrent multiple cerebral emboli, manifesting as coma, hemiplegia, aphasia and other symptoms. In 1971, Yarnell et al. reported two cases of left atrial myxoma causing cerebral embolism, and found no cardiac symptoms. Pinede et al. found the presence of single or multiple cerebral emboli in 33 cases (29%) out of 112 left atrial myxoma patients from 1959 to 1998. Lee and colleagues5 reported nine cases of atrial myxoma presented with cerebral embolism and three cases (33%) developed multiple embolism. Recently, Konagai et al.7 reported a 63-year-old male patient with left atrial myxoma and
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Multiple cerebral infarction and left atrial myxoma
Fig. 2 (A) Pre-operative transthoracic echocardiogram showing that the internal diameter of the left atrium and left ventricle were normal but a round, space-occupying mass was visible in the left atrium, with a pedicle in the atrial-septal space with marked motion (arrow). (B) Post-operative transthoracic echocardiogram showing mild mitral valve regurgitation (white arrow) and a small amount of pericardial effusion (red arrow). LV: left ventricle; LA: left atrium, RV: right ventricle, RA: right atrium. (C) Histological examination of the surgically removed cardiac mass showing (haematoxylin-eosin staining, scale bars: ×200) a large quantity of mucus was seen microscopically within the tumour tissue (red arrow), with floating star-shaped cells (black arrow).
concurrent multiple cerebral embolism and acute myocardial infarction. Most myxomas occur in the left atrium, with smaller percentages in the right atrium (4%-28%), 2 or more locations (2.5%), and less than 2.5% in both atria of the same patient. In a study by Lee et al.5, 74 patients with cardiac myxoma were recruited from the Mayo Clinic between 1993 and 2004. Eighty-one percent of the patients (60/73) had left atrial myxoma; 11% (8/73) had right atrial myxoma; and 7% (5/73) had ventricular myxoma. The biatrial myxoma is the most uncommon of all cardiac myxomas. Recently, Choi et al. presented a rare case of giant biatrial myxoma nearly obstructing the orifice of the inferior vena cava. Ibrahim et al. reported one case of biatrial myxoma presenting with cerebral ischaemia. Histological studies demonstrated that in 41% of cardiac myxomas, the surface forms thrombi and systemic embolization could result5. Atrial myxoma complicated with cerebral embolism is related to tumour activity and tumour brittleness, rather than tumour size. Tumour fragments may enter the blood flow leading to arterial embolization because the polypoid and grape-like parts of the myxoma can easily fall off. In this study,
multiple cerebral embolism was the initial sign, without prior cardiac or systemic symptoms. Rapid multiple embolism caused multiple infarctions in the deep white matter and cortex of the left hemisphere, resulting in neurological impairment. Cerebral artery embolization leads to a variety of neurological complications. In left atrial myxoma patients, approximately 12% develop intracerebral haemorrhage and 5% have subarachnoid haemorrhage because of aneurysm formation during early embolization. Multiple intracranial aneurysms secondary to left atrial myxoma are induced by myxoma embolic infarction, but the exact mechanism is still poorly understood. A recent study found that myxoma embolic-infarcted arteries nourishing the peripheral vessels allowed myxoma cells to proliferate on the vascular walls. This triggered thinning of the endothelium and reduced its elasticity, ultimately leading to aneurysm formation8. The local inflammatory response and interleukin-6 produced by myxoma cells also play a key role in aneurysm formation. Multiple intracranial aneurysms are delayed-onset neurological complications, appearing in most patients from 2 months to 19 years
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D. Sha et al.
after cardiac surgery. Since this problem was first reported by Marchand in 1894, approximately 40 cases have been reported. Sabolek et al. summarized the data from 34 patients with left atrial myxoma complicated with intracranial aneurysm, and found that aneurysms are usually multiple and that 91% of the aneurysms showed fusiform dilatation and the rest showed cystic dilatation. Aneurysms are commonly visible in the distal branches of bilateral middle cerebral arteries, but are rarely seen in the basilar artery. Fusiform and saccular aneurysms rarely coexist. Li et al.9 reported a 27-year-old female patient with left atrial myxoma presenting with multiple cerebral aneurysms of different shapes and sizes. Digital subtraction angiography is the gold standard for the diagnosis of aneurysm, but this patient did not receive this assessment because of a lack of understanding of the condition. At present, the emergency treatment for cerebral embolism caused by left atrial myxoma is controversial. Myxoma emboli consist of tumour emboli and thrombus emboli9 and because of the different myxoma emboli components, the effect of anticoagulant and antiplatelet drugs is unpredictable10. These therapies may be effective for thromboembolism or mixed emboli, but ineffective for tumour emboli. At the same time, delayed multiple aneurysm formation after embolism also has an impact on thrombolytic efficacy and safety. To date, 10 reported cases of left atrial myxoma complicated by cerebral embolism have received arterial or venous thrombolytic therapy using urokinase or recombinant tissue-type plasminogen activator10,11. The treatment was effective in six cases and invalid in four cases; only one case suffered intracerebral haemorrhage. These findings indicate that thrombolytic therapy is effective in some patients. However, the number of cases is too small to provide reliable evidence for the efficacy
and safety of thrombolytic therapy in the treatment of myxoma patients complicated with cerebral embolism. As more cases occur, the technical ability to distinguish tumour emboli and thrombus emboli will improve, providing a basis for selection of thrombolytic therapy. Our patient was admitted to the hospital on day 5 after the onset of symptoms, received no venous or arterial thrombolysis, and was treated only preoperatively with anticoagulation and antiplatelet therapy, so the effect was not assessable. Echocardiography is the most convenient and reliable diagnostic method for cardiac myxoma, with a sensitivity of approximately 95% 10. The sensitivity of transoesophageal ultrasonography, especially real-time three-dimensional transoesophageal echocardiography, reaches 100% 12. In this patient, the initial symptoms were neurological and echocardiographic and histopathological findings confirmed the left atrial myxoma. Although the long operation time for left atrial myxoma is controversial, surgical resection is still the primary means of treatment and prevention of stroke9. It is widely accepted that surgical treatment should be performed immediately upon the diagnosis of left atrial myxoma, especially for patients with a history of embolization and syncope. Cardiac myxoma is a rare cause of stroke but commonly leads to cerebral infarction or transient ischaemic attack in young adults. Therefore, echocardiography is an indispensable examination for young patients with cerebral infarction and asymptomatic cerebral infarction. The recurrence rate for myxoma is low after surgical resection, with a rate of 1-3% in sporadic cases, but such patients still need regular echocardiography to detect recurrence or relapse.
CONFLICT OF INTEREST: none.
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