Acompanhamento a longo prazo de paciente do sexo masculino com fibroma cardíaco assintomático / Long-Term follow-up of an asymptomatic man with a cardiac fibroma

Descreve-se o caso de um homem de 19 anos assintomático com fibroma de ventrículo esquerdo em acompanhamento por 15 anos, sem tratamento.(AU)

Here we describe a case of a 19-year-old asymptomatic man with a left ventricular fibroma on follow-up for 15 years with no treatment required.(AU)

Insuficiencia cardíaca como presentación de sarcoma cardíaco: caso clínico y revisión de la literatura / Cardiac sarcoma associated to heart failure: case report and review

RESUMEN: Los tumores cardíacos primarios malignos son infrecuentes en la práctica clínica. Suelen ser oligosintomáticos y de diagnóstico tardío lo que tiene como consecuencia una alta mortalidad. El caso clínico que se presenta a continuación es un ejemplo de esta patología, que debe ser especialmente considerada cuando la insuficiencia cardíaca no tiene una etiología clara. Se describen los hallazgos clínicos y de laboratorio, como también los resultados de estudios de imágenes y la histología. Se ilustra los hallazgos en la operación y la evolución clínica del paciente. Se incluye una revisión de la literatura.

ABSTRACT: Malignant primary cardiac tumors are infrequent in clinical practice. They are usually oligosymptomatic and a late diagnosis leads to a high mortality rate. The clinical case of a patient presenting with heart failure with unclear etiology is presented. Clinical and laboratory findings are described along with the results of imaging studies. Findings at the time of surgery and histopathological characteristics are illustrated. A review of the literature is included.

Mixoma auricular izquierdo gigante que causa obstrucción valvular mitral, hipertensión pulmonar y tromboembolia pulmonar / Giant atrial myxoma causing mitral valve obstruction, pulmonary hypertension and pulmonary thromboembolism

Resumen: Los tumores cardíacos pueden ser primarios o, más frecuentemente secundarios o metastásicos. Entre los tumores primarios es más frecuente el mixoma, cuya ubicación más común es en la aurícula izquierda. Las manifestaciones clínicas son diversas, producidas principalmente por obstrucción mecánica, embolizaciones, y manifestaciones constitucionales. Se comunica el caso de un paciente de 32 años, con cuadro clínico de insuficiencia cardíaca, hipertensión pulmonar severa y tromboembolismo pulmonar bilateral. Se hizo el diagnóstico de mixoma auricular izquierdo. Se resecó el tumor y se manejó la hipertensión pulmonar desde el ingreso al hospital con inhibidores de la fosfodiesterasa asociado a anticoagulación. Se discute el tema dando énfasis a aspectos fisiopatológicos involucrados tanto en la hipertensión pulmonar como en la presencia de tromboembolia pulmonar.

Abstract: Cardiac tumors may be primary or, more frequently secondary or associated to metastasis. Atril myxoma es the most frequent primary tumor, usually located in the left atrium. Clinical manifestations include those due to mitral valve occlusión, emboli and general non spedific symptoms and signs. Herein we report the clinical case of a 32 year old patient with severe pulmonary hypertension and bilateral pulmonary embolism. The tumor was extirpated, and he received phosphoro-diesterase inhiborts and anticoagulants. Subsequent clinical course was satisfactory. A brief discussion of this condicion is included.

Paraganglioma Cardíaco: reporte de un caso / Cardiac Paraganglioma: case report

Abstract: A 49-year-old woman presented with dyspnea and palpitations, leading to Functional Class III.An echocardiogram showed a heterogeneous mass adhered to the right heart cavities. This was confirmed by NMR. A large right coronary artery was occluded in relation to the tumor, which was hyper vascularized. Resection of the tumor was performed; the right ventricular wall was sutured, and an atrial defect was closed using pericardial tissue. Post operative course was uneventful and she was asymptomatic 4 years after surgery.

Accidente cerebrovascular secundario a mixoma auricular izquierdo: una causa infrecuente de embolismo cerebral / Stroke due to a auricular myxoma: a infrequent cause of cerebral embolism

BACKGROUND: Atrial myxomas are very infrequent primary bening cardiac neoplasms, being considered a rare but highly fatal cause of cerebral embolism. Objectives: We describe the case of an ischemic stroke (CVA) with hemorrhagic conversion secondary to atrila myxoma as an embolic source, and its subsequent early surgical resolution. CLINICAL CASE: A 63-year-old male has a clinical episode compatible with ischemic stroke, receiving thrombolytics treatment with subsequent hemorrhagic conversion. Embolic source study show a mass compatible with cardiac myxoma in the left atrium, performing surgical resection via transeptal approach at 12 days of evolution, with repair of the interatrial defect with autologous pericardium patch. DISCUSSION: Atrial myxoma is a silent pathology and little diagnosed at its early stage, associated with events of systemic repercussion of high mortality and uncertain prognosis. Hemorrhagic cerebrovascular events constitute contraindication for anticoagulation prior to 21 days of evolution. In this case, due to the high embolic risk of myxoma, the inactivy of the bleeding was demonstrated by performing the surgery successfully on the twelfth day of evolution.

Accidente cerebrovascular embólico secundario a fibroelastoma papilar de válvula mitral / Embolic stroke secondary to a mitral valve fibroelastoma: case report

Embolic stroke secondary to cardiac tumors is uncommon. However, 25-30% of cardiac tumors may cause systemic emboli. We report a 29-year-old male consulting for a sudden episode of aphasia and right hemiparesis, compatible with infarct of the left middle cerebral artery territory. Transthoracic echocardiography reported an ovoid tumor of 8 × 7 × 7 mm in relation to the sub valvular apparatus of the mitral valve. After neurologic stabilization, surgical treatment was performed. Approached by median sternotomy and in cardiopulmonary bypass, the mitral valve was explored. A macroscopic tumor consistent with a papillary fibroelastoma curled in sub valvular chordae was found. It was deployed and resected from its base, while the anterior mitral leaflet was preserved intact. Histopathological examination confirmed the intraoperative macroscopic diagnosis. The patient recovered uneventfully postoperatively and was discharged on the fifth day after surgery. He currently is in functional capacity I without cardiovascular symptoms at five years follow-up.

Multiple hepatic metastases of cardiac angiosarcoma

The differential diagnosis of hepatic focal lesions is challenging because the etiology can be inflammatory, infectious, and even neoplastic. A rare cause of metastatic liver nodules is cardiac angiosarcoma. We report a case of this tumor, which was diagnosed only after autopsy. A 26-year-old Caucasian man was admitted for progressive dyspnea and cough over the past 3 weeks. Physical examination showed only hypophonetic heart sounds. Laboratory analysis demonstrated anemia and elevated inflammatory markers, despite normal biochemical parameters and liver function. Transthoracic echocardiography revealed massive pericardial effusion. Abdomen computed tomography (CT) showed multiple hepatic nodules, the largest of which measured 3 cm, but the percutaneous biopsy revealed only lobular necrosis and perisinusoidal fibrosis without granulomas or neoplastic cells. During hospitalization, the patient had fever and night sweats with weight loss, and empiric treatment for extrapulmonary tuberculosis associated with corticosteroids was initiated. The outpatient follow-up revealed complete improvement of the pericardial effusion, but maintenance of the liver lesions. After 2 months of hospital discharge, the patient was readmitted with hemorrhagic shock due to bleeding liver lesions, which were evidenced by CT. Embolization of the right hepatic artery was performed, but the patient soon died. The autopsy revealed a primary cardiac angiosarcoma with multiple hepatic metastases, rupture of the Glisson's capsule and laceration of the liver. The case shows how important and difficult the diagnosis of focal liver lesions is, since it may result in an unexpected fatal outcome.

Upper vena cava syndrome secondary to giant atrial myxoma

SUMMARY Cardiac myxoma is a benign neoplasm, which corresponds to the most common primary heart tumour, responsible for about 50% of the cases. In general, 75-80% of myxomas are located in the left atrium, 18% in the right atrium, and more rarely in the ventricles or multicentric. Right atrial myxoma, in particular, can obstruct the tricuspid valve, causing symptoms of right heart failure, peripheral oedema, hepatic congestion, and syncope. Systemic embolization occurs in 30% of cases, by either tumour fragmentation or total tumour detachment. In the present report, we present a case of a symptomatic patient, who showed a large right intra-atrial lesion, with consequent superior vena cava syndrome, and then underwent surgical resection at admission.

RESUMO O mixoma cardíaco é uma neoplasia benigna, que corresponde ao tumor primário mais comum do coração, responsável por cerca de 50% dos casos. De modo geral, 75 a 80% dos mixomas estão localizados no átrio esquerdo, 18% no átrio direito, e mais raramente, nos ventrículos ou multicêntricos. O mixoma atrial direito, em particular, pode obstruir a válvula tricúspide, causando sintomas de insuficiência cardíaca direita, edema periférico, congestão hepática e síncope. A embolização sistêmica ocorre em 30% dos casos, quer pela fragmentação do tumor ou pelo desprendimento total do mesmo. No presente relato, apresentamos um caso de uma paciente sintomática, que evidenciou grande lesão intra-atrial direita, com consequente síndrome da veia cava superior, sendo, então, submetida a ressecção cirúrgica na internação.

A rare cause of abdominal pain in childhood: cardiac angiosarcoma

Abstract Cardiac angiosarcomas are extremely rare in childhood, they are rapidly progressive tumours that often present themselves as diagnostic dilemmas, resulting in delayed diagnosis. Also, extracardiac manifestations, including abdominal pain, are extremely rare in patients with intracardiac tumors. We herein present the case of a 15-year-old girl who presented with abdominal pain. Echocardiography and thoracic computed tomography showed right atrial mass. The patient underwent surgery, chemotherapy, and radiotherapy. Eight months after treatment, abdominal recurrence was detected. The abdominal mass was resected, and radiotherapy and new chemotherapy protocol were given. The present case illustrates a rare case of primary cardiac angiosarcoma posing a diagnostic dilemma in an adolescent girl.

Diagnóstico prenatal de rabdomioma fetal asociado a esclerosis tuberosa: a propósito de un caso / Prenatal diagnosis of fetal rhabdomyoma associated with tuberous sclerosis

Los rabdomiomas son los tumores benignos cardíacos más frecuentes encontrados en vida fetal y postnatal, asociándose frecuentemente a esclerosis tuberosa. A propósito de un caso manejado en nuestra maternidad el año 2014 se revisó la literatura existente sobre su diagnóstico, manejo, implicancias pronósticas. Se trata de una embarazada de 23 años que fue referida a las 25 semanas a nuestro servicio, el estudio ecográfico revela tres imágenes cardiacas concordantes con rabdomiomas en las paredes ventriculares y el septum. No se detectan otras anormalidades y se controla en forma seriada con ultrasonido. Se realiza a las 33 semanas resonancia nuclear magnética fetal con el objetivo de evidenciar signos de esclerosis tuberosa los cuales se evidencian en el examen y se confirman en exámenes postnatales. Se comenta el diagnóstico diferencial con otros neoplasias benignas cardiacas como teratomas, fibromas y hemangiomas. Las posibles complicaciones de los rabdomiomas dependerán de su ubicación y tamaño, produciendo alteraciones en flujo o bien menos frecuentemente arritmias. La asociación más importante de esta patología es con esclerosis tuberosa de manera que se debe realizar una cuidadosa anamnesis familiar, estudios de imágenes que deben abarcar el SNC, corazón y riñones. Finalmente en ese contexto realizar la mejor consejería a los padres.

Rhabdomyomas are the most common benign cardiac tumors found in fetal and postnatal life, frequently being associated with tuberous sclerosis. In relation to a case handled in our maternity unit in 2014, existing literature on cardiac rhabdomyomas diagnosis, management and prognostic implications, was revised. The case concerned a 25 week pregnant 23 year old patient, who was referred to our facility; ultrasound examination reveals three cardiac images consistent with rhabdomyomas in the ventricular walls and the septum. No other abnormalities are detected and the patient is monitored with a series of ultrasound. At 33 weeks a fetal magnetic resonance imaging is performed in order to show signs of tuberous sclerosis which are noted in the examination and confirmed in post natal tests. Differential diagnosis with other benign cardiac neoplasms such as teratomas, fibromas and hemangiomas is discussed. Possible complications of the rhabdomyomas will depend on its location and size, producing changes in flow or less frequent arrhythmias. The most important association of this pathology is with tuberous sclerosis, therefore the family medical history must be carefully evaluated, and imaging controls that should include the CNS, heart and kidneys. Finally in this context give the best counseling to the parents.

Síncope secundario a mixoma gigante del anillo mitral: reporte de un caso / Large mitral annulus myxoma presenting with syncope: report of one case

We report a 23-year-old woman, with three recent exertional syncopes. Transthoracic (TTE) and transesophageal (TEE) echocardiography found a large heterogeneous mass (38 x 35 mm) arising from the posterior mitral annulus, protruding in systole through the left ventricular outflow tract (LVOT). Heart MRI confirmed the echocardiography findings, suggesting a cardiac myxoma. Cardiac surgery accomplished the complete resection of the lesion, confirming a mass arising from the posterior mitral annulus and preserving mitral anatomy and function. Pathology was positive for a myxoma. Uneventful evolution allowed the discharge of the patient at the fifth postoperative day. Control TTE discarded any complication.

Stroke of a cardiac myxoma origin / Acidente vascular cerebral com origem em mixoma cardíaco

Abstract Objective: The clinical features of cardiac myxoma stroke have not been sufficiently described. Debates remain concerning the options and timing of treatment and the clinical outcomes are unknown. This article aims to highlight the pertinent aspects of this rare condition. Methods: Data source of the present study came from a comprehensive literature collection of cardiac myxoma stroke in PubMed, Google search engine and Highwire Press for the year range 2000-2014. Results: Young adults, female predominance, single cerebral vessel (mostly the middle cerebral artery), multiple territory involvements and solitary left atrial myxoma constituted the outstanding characteristics of this patient setting. The most common affected cerebral vessel (the middle cerebral artery) and areas (the basal ganglion, cerebellum and parietal and temporal regions) corresponded well to the common manifestations of this patient setting, such as conscious alteration, ataxia, hemiparesis and hemiplegia, aphasia and dysarthria. Initial computed tomography scan carried a higher false negative rate for the diagnosis of cerebral infarction than magnetic resonance imaging did. A delayed surgical resection of cardiac myxoma was associated with an increased risk of potential consequences in particular otherwise arterial embolism. The mortality rate of this patient population was 15.3%. Conclusion: Cardiac myxoma stroke is rare. Often does it affect young females. For an improved diagnostic accuracy, magnetic resonance imaging of the brain and echocardiography are imperative for young stroke patients in identifying the cerebral infarct and determining the stroke of a cardiac origin. Immediate thrombolytic therapy may completely resolve the cerebral stroke and improve the neurologic function of the patients. An early surgical resection of cardiac myxoma is recommended in patients with not large territory cerebral infarct. .

Resumo Objetivo: As características clínicas do acidente vascular cerebral causado por mixoma cardíaco não foram descritas suficientemente. Debates permanecem sobre as opções e o momento de tratamento e os resultados clínicos são desconhecidos. Este artigo tem como objetivo destacar os aspectos pertinentes desta condição rara. Métodos: Os dados do presente estudo foram levantados em uma revisão abrangente de literatura sobre acidente vascular cerebral causado por mixoma cardíaco no PubMed, no sistema de buscas do Google e no Highwire Press, abrangendo ao anos entre 2000 e 2014. Resultados: Adultos jovens, predominância do sexo feminino, vaso cerebral único (principalmente a artéria cerebral mediana), envolvimentos de territórios múltiplos e mixoma atrial esquerdo solitário são características marcantes destes pacientes. O vaso cerebral afetado mais comum (artéria cerebral média) e áreas (o gânglio basal, cerebelo e regiões parietais e temporais) corresponderam bem com as manifestações comuns destes pacientes, como alteração da consciência, ataxia, hemiparesia e hemiplegia, afasia e disartria. Tomografia computadorizada inicial mostrou taxa de falso negativo mais alta para o diagnóstico de acidente vascular cerebral do que a imagem por ressonância magnética. A ressecção cirúrgica tardia de mixoma cardíaco foi associada com risco aumentado de potenciais consequências, em particular, de outra forma de embolia arterial. A taxa de mortalidade dessa população de pacientes foi de 15,3%. Conclusão: Acidente vascular cerebral causado por mixoma cardíaco é raro. Frequentemente, afeta mulheres jovens. Para um diagnóstico mais preciso, exames de ressonância magnética e ecocardiográficos são imperativos para pacientes jovens com acidente vascular cerebral para determinar a localização do enfarte cerebral e se houve origem cardíaca. Terapia trombolítica imediata pode resolver completamente o acidente vascular cerebral e melhorar a função neurológica ...

Mortality and Embolic Potential of Cardiac Tumors / Mortalidade e Potencial Embólico dos Tumores Cardíacos

Background: Cardiac tumors are rare, mostly benign with high embolic potential. Objectives: To correlate the histological type of cardiac masses with their embolic potential, implantation site and long term follow up in patients undergoing surgery. Methods: Between January 1986 and December 2011, we retrospectively analyzed 185 consecutive patients who underwent excision of intracardiac mass (119 females, mean age 48±20 years). In 145 patients, the left atrium was the origin site. 72% were asymptomatic and prior embolization was often observed (19.8%). The diagnosis was established by echocardiography, magnetic resonance and histological examination. Results: Most tumors were located in the left side of the heart. Myxoma was the most common (72.6%), followed by fibromas (6.9%), thrombi (6.4%) and sarcomas (6.4%). Ranging from 0.6cm to 15cm (mean 4.6 ± 2.5cm) 37 (19.8%) patients had prior embolization, stroke 10.2%, coronary 4.8%, peripheral 4.3% 5.4% of hospital death, with a predominance of malignant tumors (40% p < 0.0001). The histological type was a predictor of mortality (rhabdomyomas and sarcomas p = 0.002) and embolic event (sarcoma, lipoma and fibroelastoma p = 0.006), but not recurrence. Tumor size, atrial fibrillation, cavity and valve impairment were not associated with the embolic event. During follow-up (mean 80±63 months), there were 2 deaths (1.1%) and two recurrences 1 and 11 years after the operation, to the same cavity. Conclusion: Most tumors were located in the left side of the heart. The histological type was predictor of death and preoperative embolic event, while the implantation site carries no relation with mortality or to embolic event. .

Fundamento: Os tumores do coração são infrequentes, em sua maioria benignos e com alto potencial embólico. Objetivo: Correlacionar o tipo histológico do tumor cardíaco com seu potencial embólico, com o sítio de implantação e analisar a evolução tardia destes pacientes submetidos à cirurgia. Métodos: No período de dezembro de 1986 a setembro de 2011 foram retrospectivamente analisados 186 pacientes operados (119 do sexo feminino e idade média de 48 ± 20 anos). Foram 145 tumores de átrio esquerdo (77%), 72% dos pacientes assintomáticos e 19,8% com embolização prévia. O diagnóstico foi confirmado por ecocardiograma, ressonância magnética e exame histológico. Resultados: A maioria dos tumores situava-se nas câmaras esquerdas. O mixoma foi o mais frequente (72,6%), seguido dos fibromas (6,9%), trombos (6,4%) e sarcomas (6,4%). Seus tamanhos variaram de 0,6cm a 15 cm (média de 4,6 ± 2,5cm). Houve 37 embolizações prévias à operação (10,2% AVC, 4,8% IAM e 4,3% periférica). Foram 5,4% de óbito hospitalar, com predomínio nos tumores malignos (40% p < 0,0001). O tipo histológico foi preditor de mortalidade (rabdomioma e sarcomas p = 0,002) e de evento embólico (sarcomas, fibroelastoma e lipoma p = 0,006), porém não de recidiva. O tamanho tumoral, a fibrilação atrial, a cavidade e valva acometida não apresentaram relação com o evento embólico. Durante o seguimento (média de 80 ± 63 meses), houve 2 óbitos (1,1%) e duas recidivas tumorais 1 e 11 anos após a operação, ambas para a mesma cavidade. Conclusão: O tipo histológico foi preditor de óbito e de evento embólico pré-operatório, enquanto o sítio de implantação não. .

Stroke associated with left atrial mass: Association of cerebral aneurysm with left atrial myxoma.

Association of LA myxoma with cerebral aneurysm is rare. We describe a patient who had LA mass and cerebral aneurysm and developed stroke. The patient underwent clipping of the cerebral aneurysm. We discuss the pathology of the association and the anesthetic management.

Trombocitopenia severa como manifestación de un tumor testicular de células germinales con metástasis cardíaca intracavitaria / Testicular tumor with intracardiac metastasis presenting as severe thrombocytopenia

Se describe el caso de un hombre de 24 años que se presenta con Púrpura trombocitopénico, cuyo estudio ecocardiográfico y tomografía computada demostró masa tumoral ocupando las cavidades derechas. Se evidenció posteriormente un tumor testicular izquierdo con componentes de seminoma y teratoma. Se resecó el tumor testicular y posteriormente el tumor intracardíaco, con normalización del recuento plaquetario.

A 24 year old man presented with severe thrombocytopenia. An intracardiac mass was shown to be a metastasis from a malignant testicular tumor. Resection of the primary tumor and the intracardiac metastasis led to full recovery of thrombocytopenia.

Infarto do miocárdio e mixoma em átrio esquerdo / Myocardial infarction and myxoma in the left atrium / Infarto del Miocardio y Mixoma en Aurícula Izquierda

Os mixomas são os tumores cardíacos primários benignos mais comuns, sendo que a grande maioria localiza-se no átrio esquerdo; 80 por cento têm sua origem no septo interatrial e 5 por cento deles são biatrial. Clinicamente, apresentam-se, quase sempre, com sinais e sintomas de doença valvular mitral ou de eventos tromboembólicos. Este relato ilustra um caso de mixoma em átrio esquerdo, projetando-se através da valva mitral para o ventrículo esquerdo e simulando uma estenose mitral, que evoluiu com acidente vascular cerebral isquêmico (AVCi) e infarto agudo do miocárdio (IAM), como complicações tromboembólicas. O ecocardiograma continua a ser uma ferramenta valiosa no diagnóstico e o tratamento cirúrgico imediato é necessário para evitar desfechos fatais.

Myxomas are the most common benign primary cardiac tumors, the vast majority located in the left atrium and 80 percent originates in the atrial septum and 5 percent of them are biatrial.Usually present, often with signs and symptoms of mitral valve disease or thromboembolic events. This report illustrates a case of myxoma in left atrium protrudind through the mitral valve into the left ventricle, simulating stenotic mitral valve disease, which evolved with ischemic stroke (AIS) and acute myocardial infarction (MI) as thromboembolic events. Echocardiography continues to be a valuable tool in the diagnosis and surgical treatment is immediately necessary to prevent fatal outcomes.

Los mixomas son los tumores cardíacos primarios benignos más comunes, siendo que la gran mayoría se localiza en la aurícula izquierda; el 80% tiene su origen en el septo interauricular y el 5% de ellos son biauriculares. Clínicamente, se presentan, casi siempre, con señales y síntomas de enfermedad valvular mitral o de eventos tromboembólicos. Este relato ilustra un caso de mixoma en aurícula izquierda, proyectándose a través de la válvula mitral hacia el ventrículo izquierdo y simulando una estenosis mitral, que evolucionó con accidente vascular cerebral isquémico (AVCi) e infarto agudo del miocardio (IAM), con complicaciones tromboembólicas. El ecocardiograma continua siendo una herramienta valiosa en el diagnóstico y el tratamiento quirúrgico inmediato es necesario para evitar desenlaces fatales.

Multimodalidade em imagem cardiovascular na caracterização de lesão valvar aórtica pós radioterapia: relato de quatro casos / Multimodality in cardiovascular image in aortic valvar lesion after radiotherapy: report of four case

A eficácia do tratamento de tumores como linfoma Hodgkin e o câncer de mama com o uso da radioterapia reduziu, significativamente, a morbidade e mortalidade desses pacientes. Todavia, com o aumento da sobrevida, as complicações cardiovasculares, entre elas, as lesões valvares começaram a surgir, anos ou até décadas após a radioterapia. O acompanhamento com o ecodopplercardiograma, após a radioterapia, é indispensável como medida de detecção precoce e acompanhamento evolutivo, devido ao seu baixo custo e fácil acesso. O objetivo deste estudo é relatar quatro casos de pacientes que desenvolveram lesão valvar aórtica após radioterapia.

Treatment efficacy of tumors like Hodgkin’s Lymphoma and breast cancer with the use of radiotherapy has significantly reduced morbidity and mortality of these patients. However, with the increase of survival, cardiovascular complication, e.g., valvar lesions has arisen years to decades after the end of radiotherapy. The following with echocardiography after radiotherapy is an essential step in early detection and following, because of low cost and easy access. The aim of this paper is to report four cases of patients that developed aortic valvar lesion after radiotherapy.