RESUMO

Takotsubo syndrome (TTS) is a rare cardiomyopathy, but its prevalence is increasing due to the greater availability of diagnostic tools, whose pathophysiology is unknown; however, the evidence points to an excess of catecholamines that ends up generating cardiac stunning. The cause of excessive sympathetic discharge is multifactorial, and some tumors may be related to their origin. In this case report, we present a female patient with TTS whose only identified triggering factor was an atrial myxoma, which generated an unusual clinical presentation. Current multimodal diagnostic tools together with the multidisciplinary evaluation of the HeartTeam allowed an accurate diagnosis and an adequate management of the clinical picture.

RESUMO

Las neoplasias cardíacas se dividen en tumores primarios y secundarios; estos últimos son 30 veces más frecuentes. La mayoría de las neoplasias cardíacas son benignas y dentro de este grupo destaca el mixoma auricular. La presentación clínica es muy variable en relación con su ubicación, tamaño y movilidad, y los hallazgos a nivel de las pruebas complementarias son inespecíficos. Dado que su historia natural muchas veces pasa inadvertida, el mixoma puede culminar en la obstrucción auriculoventricular, obstrucción del tracto de salida ventricular o incluso embolias sistémicas; de esta forma, es el causante de sintomatología severa como síncope, insuficiencia cardíaca y muerte súbita cardíaca. Su manifestación clínica como un síncope es bastante frecuente. Se expone el caso de un paciente de sexo masculino de 81 años que consultó en el servicio de urgencias por un síncope. Durante su valoración, se identificó el mixoma auricular como etiología de este.

Cardiac neoplasms are divided into primary and secondary tumors, the latter are 30 times more frequent. Most are benign and within this group the atrial myxoma stands out. The clinical presentation is very variable in relation to its location, size and mobility, and the findings in the complementary tests are nonspecific. Since its natural history often goes unnoticed, the myxoma can culminate in atrial ventricular obstruction, ventricular outflow tract obstruction or even systemic embolism, causing severe symptoms such as syncope, heart failure and sudden cardiac death. Its clinical manifestation as syncope is quite common. The case of an 81-year-old male patient who consulted at the emergency department for syncope is presented, in which the atrial myxoma was subsequently identified as the etiology of the event during the further evaluation.

As neoplasias cardíacas dividem-se em tumores primários e secundários, estes últimos são 30 vezes mais frequentes. A maioria são benignas e dentro deste grupo destaca-se o mixoma auricular. A apresentação clínica é muito variável em relação com sua localização, tamanho e mobilidade, e os achados a nível dos testes complementares são inespecíficos. Dado que a sua história natural muitas vezes passa despercebida, o mixoma pode culminar na obstrução aurículo-ventricular, obstrução do tracto de saída ventricular ou mesmo embolias sistémicas, sendo assim o causador de sintomatologia severa como síncope, insuficiência cardíaca e morte súbita cardíaca. Sua manifestação clínica como síncope é bastante comum. Apresenta-se o caso de um paciente de sexo masculino de 81 anos que consultou no serviço de urgências para um síncope. Durante a sua avaliação, se identificou mixoma auricular como etiologia deste

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Atrial myxomas are the most common primary cardiac tumors and may manifest with neurological symptoms in 30% of cases. Cerebral ischemia, aneurysmal formation, and extravascular metastases are mechanisms that lead to these neurological manifestations. Perilesional changes on computed tomography (CT) and magnetic resonance imaging (MRI) may help in the diagnosis of myxomatous aneurysms, which are usually located in the distal middle cerebral artery (MCA) and in the posterior cerebral artery (PCA) circulation territories. Careful resection of the cardiac lesion is essential for preventing embolism. However, treatment of myxomatous aneurysms is controversial due to the limited understanding of the natural history of this condition. Treatment may include clinical observation in asymptomatic patients, surgical resection, endovascular approaches, adjuvant chemotherapy, and low-dose radiation therapy. We present one case of a female patient with myxomatous aneurysm secondary to an atrial myxoma who presented with neurological symptoms and another case of a female patient who developed neurological symptoms after initial surgical treatment of the primary lesion. Lesion growth rate, topography, morphology, and the patient's clinical condition must be considered when choosing a therapeutical method. Further clinical studies are needed to achieve a better understanding and treatment of this disease.

Mixomas atriais são os tumores primários cardíacos mais comuns. Podem levar a manifestações neurológicas em cerca de 30% dos pacientes devido a: isquemia cerebral, formação de aneurismas e metástases extravasculares. Alterações perilesionais encontradas tanto nas de tomografia (TC) ou ressonância magnética (RM) podem ajudar no diagnóstico de aneurismas mixomatosos, que geralmente envolvem ramos distais das artérias cerebral média e posterior. Ressecção cirôrgica cuidadosa da lesão cardíaca é essencial para prevenção de eventos embólicos. Entretanto, por sua história natural mal conhecida devida à raridade da condição, o tratamento dos aneurismas mixomatosos é controverso, incluindo conduta conservadora em pacientes assintomáticos, abordagem cirôrgica, endovascular, quimioterapia adjuvante e radioterapia em baixas doses. Discutiremos dois casos clínicos de pacientes femininas com aneurismas mixomatosos, tendo o primeiro caso o acometimento neurológico como manifestação inicial e o segundo com sintomas posteriores ao tratamento cirôrgico do tumor primário. Determinação da melhor escolha do tratamento deve levar em consideração taxa de crescimento da lesão, sua topografia, morfologia e clínica do paciente. Mais estudos clínicos são necessários para melhor compreensão e definição de conduta desses pacientes.

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Cardiac tumors are rare and myxomas are the most prevalent among them. Although it presents benign histology, they may be the cause of severe consequences due to intracardiac location. Nonspecific manifestations may hinder the diagnosis, which can lead to complications, such as changes in cardiac dynamics, thromboembolic events and death. This paper presents a case report of a patient in the emergency department of a high complexity hospital in Minas Gerais State, Brazil, with a clinical history of angina and 7 previous Transient Ischemic Attack (TIA), besides aphasia, right hemiparesis, dysarthria, and mouth deviation. Submitted to transesophageal echocardiography, she was diagnosed with left atrial myxoma. For the treatment of myxoma, a surgical approach was used to resect the myxoma in the left atrium. The standard surgical approach was through a median sternotomy. Cardiopulmonary bypass (CPB) with aortic and bicaval cannulation and moderate hypothermia was used. Myocardial protection was achieved by cold antegrade blood cardioplegia. The patient had notable characteristics for the diagnosis, especially the previous occurrence of seven episodes of TIA. The importance of correct clinical investigation (anamnesis, general and specific physical examination and imaging tests) is notable and a fundamental tool for the diagnosis and early treatment of neoplasia, providing good clinical outcomes.

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Systemic embolization has been reported in up to 40% of patients with left atrial myxoma, half of them with cerebral involvement. However, development of intracerebral embolization associated with parenchymal seeding of the myxoma emboli is an extremely rare complication, with only 36 histologically diagnosed cases reported in the published literature. We describe a 69-year-old woman who arrived at the emergency service with hemiparesis associated with drug-resistant epilepsy and a medical history of resection of a left atrial myxoma 10 months previously. Cranial computed tomography revealed multiple large lesions of heterogeneous density and cystic components in the occipital lobes and posterior fossa parenchyma. Histopathological analyses after stereotactic biopsy of the occipital lesion revealed infiltrative myxoma cells with benign histological findings and uniform expression of calretinin similar to that of the primary cardiac myxoma. Additional immunohistochemical studies confirmed brain parenchymal seeding of the myxoma cells with strong expression of interleukin-6 (IL-6) and focal expression of matrix metalloproteinases-2 (MMP-2). Here, we discuss the clinicopathological features of intracerebral embolization of left atrial myxomas associated with progressive parenchymal seeding of the tumor emboli and the potential pathogenic role of IL-6 and MMPs.

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Cardiac myxomas are the most frequently encountered tumors of the heart. However, they are unusual to be newly diagnosed in the geriatric population. Myxomas are among the great mimickers, with a myriad of clinical presentations related to heart failure, embolic events, and constitutional symptoms. We describe a rare case of a giant atrial myxoma in a nonagenarian presenting with heart failure, which was medically managed.

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RESUMEN El mixoma auricular es un tumor cardíaco benigno de origen primario que se localiza mayormente en la aurícula izquierda. El presente trabajo tiene como objetivo describir las formas clínicas de presentación de un mixoma de aurícula derecha, a través de la presentación de un caso con el diagnóstico de síndrome de hipertensión pulmonar secundaria al mencionado tumor cardíaco, en una paciente de 54 años de edad. Las características clínicas de los mixomas están determinadas por su localización, tamaño y movilidad. En este caso, la obstrucción intracardíaca producida por el mixoma, con movimiento paradójico del septum, dió lugar a alteraciones en el llenado ventricular, signos de bajo gasto e hipertensión pulmonar grave. El abordaje clínico y quirúrgico inmediato tras el diagnóstico evitó el desarrollo de complicaciones mayores. Este caso enfatiza la vital importancia del juicio clínico y el uso de diversas técnicas de imagen cardíaca en la toma de decisiones.

ABSTRACT Atrial myxoma is a benign primary cardiac tumor that is mostly found in the left atrium. This paper aims to describe the clinical presentation of a right atrial myxoma through the case report of a 54-year-old patient diagnosed with pulmonary hypertension syndrome secondary to the above-mentioned cardiac tumor. The clinical features of myxomas are determined by their location, size and mobility. In this case, intracardiac obstruction produced by the myxoma, with paradoxical movement of the septum, resulted in ventricular filling alterations, low cardiac output signs and severe pulmonary hypertension. Immediate clinical and surgical management after diagnosis successfully prevented the development of major complications. This case highlights the paramount importance of clinical judgment and the use of various cardiac imaging techniques in decision making.

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RESUMEN La asociación de dos tumores de diferente origen en un mismo paciente es cada vez más frecuente en la práctica clínica actual. Se presenta un caso con una rara asociación de mixoma cardíaco e hipernefroma, previamente tratado. Ante la presencia de manifestaciones neurológicas, inicialmente se planteó el diagnóstico de metástasis cerebral del tumor renal; luego, con el hallazgo ecocardiográfico de una masa intracardíaca, se pensó en la posibilidad de trombo, por lo que se decidió practicar cirugía cardíaca con carácter urgente para resecarla. El estudio anatomopatológico confirmó la existencia de un mixoma cardíaco, por lo que se trata de un enfermo con dos enfermedades tumorales cuya coincidencia ha sido pocas veces descrita.

ABSTRACT The association of two tumors of different origin in the same patient is becoming more frequent in the current clinical practice. Here is presented a case with a rare association of cardiac myxoma and hypernephroma, previously treated. Due to the presence of neurological symptoms, there was initially set a diagnosis of brain metastasis from the renal tumor; then, with the echocardiographic finding of an intracardiac mass, the possibility of thrombus was considered, therefore a cardiac surgery was decided to be performed urgently in order to resect it. The pathologic examination confirmed the existence of a cardiac myxoma, then, we have a patient with two tumor diseases whose coincidence has been rarely described.

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Abstract Atrial myxoma is a benign tumor of the heart that occurs primarily in the left atrium. Floating or large left atrial myxomas frequently cause functional mitral stenosis, may also affect mitral valve structure and flow, and lead to mitral regurgitation. Systemic embolization occurs in around 30% of cases either from tumor fragmentation or complete tumor detachment hence it should be removed as soon as it is detected. Intraoperative transesophageal echocardiography has a vital importance in the surgery. After resection of myxoma, intraoperative transesophageal echocardiography must be performed to rule out residual mass. The case here reported is of a 48-year old female, who presented with giant and floating left atrial myxoma. Residue mass was detected with intraoperative transesophageal echocardiography in the left ventricle after the resection of myxoma. Subsequently, the residue mass was successfully removed. Complete resection must be required to prevent possible complications such as recurrence, embolization in atrial myxomas. Transesophageal echocardiography performed intraoperatively is vital importance to confirm that the myxoma is completely resected.

Resumo Mixoma atrial é um tumor benigno do coração que ocorre principalmente no átrio esquerdo. Os mixomas flutuantes ou grandes em átrio esquerdo com frequência causam estenose mitral funcional, podendo também afetar a estrutura e o fluxo da válvula mitral e levar à insuficiência mitral. A embolização sistêmica ocorre em cerca de 30% dos casos, quer pela fragmentação do tumor ou pelo desprendimento total do tumor; portanto, o tumor deve ser removido assim que detectado. A ecocardiografia transesofágica intraoperatória tem uma importância vital na cirurgia. Após a ressecção do mixoma, a ecocardiografia transesofágica intraoperatória deve ser feita para excluir a massa residual. O caso aqui relatado é o de uma paciente de 48 anos que apresentou um mixoma de átrio esquerdo gigante e flutuante. A massa residual foi detectada com ecocardiografia transesofágica intraoperatória no ventrículo esquerdo após a ressecção do mixoma. Posteriormente, a massa residual foi removida com sucesso. A ressecção completa é necessária para evitar possíveis complicações, como recorrência e embolização em mixomas atriais. A ecocardiografia transesofágica realizada no intraoperatório é de vital importância para confirmar a ressecção completa do mixoma.

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Cardiac myxomas are frequently located in the left or right atria, with multiple locations being rare. We report a 59-year-old healthy female with 5 months of cough and exertional dyspnea. A transthoracic echocardiography (TTE) exhibits a 9 × 5 cm nonpedunculated tumor arising from the interatrial septum (IAS) and inhabiting both atria, but was unable to depict the relation with the IAS. Transesophageal echocardiography exposes a single tumor crossing the IAS through an ostium secundum atrial septal defect (ASD) causing right heart functional impairment. Uneventful cardiac surgery allowed complete resection of the lesion and ASD closure. Pathology reported a myxoma.

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Atrial myxoma is a benign tumor of the heart that occurs primarily in the left atrium. Floating or large left atrial myxomas frequently cause functional mitral stenosis, may also affect mitral valve structure and flow, and lead to mitral regurgitation. Systemic embolization occurs in around 30% of cases either from tumor fragmentation or complete tumor detachment hence it should be removed as soon as it is detected. Intraoperative transesophageal echocardiography has a vital importance in the surgery. After resection of myxoma, intraoperative transesophageal echocardiography must be performed to rule out residual mass. The case here reported is of a 48-year old female, who presented with giant and floating left atrial myxoma. Residue mass was detected with intraoperative transesophageal echocardiography in the left ventricle after the resection of myxoma. Subsequently, the residue mass was successfully removed. Complete resection must be required to prevent possible complications such as recurrence, embolization in atrial myxomas. Transesophageal echocardiography performed intraoperatively is vital importance to confirm that the myxoma is completely resected.

RESUMO

Se presenta un caso de mixoma auricular izquierdo que se manifiesta clínicamente por episodios sincopales de corta duración relacionados con la posición postural. El diagnóstico fue confirmado con la ecocardiografía transtorácica y transesofágica y complementado con un cateterismo cardiaco en donde se descubrió una lesión severa de la arteria descendente anterior. La cirugía cardiaca con circulación extracorpórea fue el tratamiento lográndose exéresis completa del tumor en el sitio de implante de su pedículo.

We present a case of left atrial myxoma that is manifested clinically by syncopal episodes of short duration related to the postural position. The diagnosis was confirmed by transthoracic and transesophageal echocardiography and complemented by cardiac catheterization in which a severe lesion of the anterior descending artery was discovered. Cardiac surgery with extracorporeal circulation was the treatment achieving complete excision of the tumor at the site of implantation of its pedicle.

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RESUMEN Los tumores cardíacos son parte del diagnóstico diferencial en el estudio de las masas intracardíacas, junto con vegetaciones, trombos, calcificaciones valvulares, entre otros. Su localización, tamaño y movilidad pueden dar lugar a importantes implicaciones funcionales y anatómicas que determinan la presentación clínica. Describimos el caso de una mujer de 51 años de edad, quien se presentó con cuadro sugestivo de un síndrome coronario agudo, pero gracias a los hallazgos ecocardiográficos se le diagnosticó un tromboembolismo pulmonar originado por un mixoma en la aurícula derecha con posterior verificación histopatológica..

Cardiac tumors are part of the differential diagnosis in the study of intra-cardiac masses along with vegetations, thrombi, valvular calcifications among others. Their localization, size and mobility can lead to major anatomical and functional implications determining the clinical presentation. We describe the case of a 51 year-old female, who presented with complains suggestive of an acute coronary syndrome, in which an echocardiographic showed it a pulmonary embolism caused by a right atrial myxoma with histopathological verification was made.

RESUMO

Los tumores primarios del corazón son raros, con una incidencia estimada de 0,0017-0,19 porciento.Los mixomas cardíacos son los más frecuentes (50 porciento). La localización en la aurícula derecha es inusual y ocurre solo en el 15-20 porciento de los casos. Su presentación clínica puede ser asintomática, con presencia de síntomas constitucionales o dependientes de una obstrucción cardíaca o un embolismo pulmonar. Presentamos el caso de un paciente masculino de 36 años de edad con historia de astenia, palpitaciones y disminución de la capacidad funcional. La ecocardiografía transtorácica mostró una gran masa en la aurícula derecha, prolapsando al interior del ventrículo derecho y obstruyendo la válvula tricúspide. El paciente fue sometido a cirugía de bypass cardiopulmonar y la masa (9 Î 9 cm) fue extirpada sin complicaciones. El examen histológico confirmó el diagnóstico de mixoma

Primary tumors of the heart are rare, their estimated incidence ranging between 0.0017-0.19 percent, and among them cardiac myxomas are the most common (50 percent). Right atrial localization is uncommon, occurring only in 15-20 percent of cases. Their clinical presentation ranges from an asymptomatic status to the presence of constitutional symptoms or symptoms pointing to the presence of cardiac obstruction or pulmonary embolism. A case is presented of a 36-year-old male patient with a history of asthenia, palpitations and reduced functional capacity. Transthoracic echocardiography revealed a large mass in the right atrium which prolapsed into the right ventricle and obstructed the tricuspid valve. The patient underwent cardiopulmonary bypass surgery and the mass (9x9 cm) was removed without any complication. Histological examination confirmed the diagnosis of myxoma

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