Mixoma cardiaco como causa de isquemia en niños. Reporte de casos

El mixoma cardiaco constituye uno de los tumores benignos más frecuentes entre los tumores cardiacos. El mismo se caracteriza por la obstrucción del flujo sanguíneo por trombosis o émbolo, resultando en causa de insuficiencia cardiaca con disfunción ventricular, síncope ortostático, isquemia de diferentes órganos de acuerdo a la arteria afectada, e incluso muerte súbita. Presentamos 2 casos en pacientes pediátricos: el primer caso, una adolescente con ACV isquémico y el segundo caso se trata de un adolescente con isquemia a nivel de miembros inferiores por una tromboembolia en la Aorta distal. En ambos casos se realizó la exéresis del tumor, con éxito.

Cardiac myxoma is one of the most frequent benign tumors among cardiac tumors. It is characterized by the obstruction of blood flow due to thrombosis or embolus, resulting in heart failure with ventricular dysfunction, orthostatic syncope, ischemia of different organs depending on the affected artery, and even sudden death. We present 2 cases in pediatric patients: the first case, an adolescent with ischemic stroke and the second case is an adolescent with ischemia in the lower limbs due to a thromboembolism in the distal aorta. In both cases, the exeresis of the tumor was performed successfully.

Tumores cardíacos benignos operados en un Centro de Cirugía Cardiovascular Regional 2015 - 2021

Introducción: Los tumores cardíacos primarios se caracterizan por su baja prevalencia, son principalmente mixomas y se presentan frecuentemente de forma asintomática. Objetivos: Identificar el tipo histológico más común, edad de presentación, tipo de cirugías y sobrevida de un grupo de pacientes tratados por Tumores Cardíacos Primarios (TCP) en el Hospital Regional de Temuco. Métodos: Revisión de fichas clínicas de 14 pacientes portadores de TCP entre marzo 2015 y diciembre 2021. Resultados: El tipo histológico más común fue el mixoma (85,7%), seguido por el fibroelastomas papilar (14,3%). La edad promedio fue 62 años (39-85), 9 fueron mujeres y 5 hombres. Los antecedentes mórbidos más comunes fueron: Insuficiencia Cardíaca Congestiva (ICC), Hipertensión Arterial (HTA) y Accidente Vascular Encefálico (AVE). La localización anatómica más común fue la Aurícula izquierda (92%). El tratamiento en el 92% de los casos fue resección aislada y en el 7% restante resección y reparación con parche. Conclusiones: Nuestros resultados son concordantes con la literatura.

Background: primary cardiac tumors are characterized by a low prevalence. Most of them are myxomas and asymptomatic. Aim: To describe the most common histological type, the age of presentation, type of surgery performed and survival of a group of patients operated on for Primary Cardiac Tumors (PCT) in the Hospital Regional de Temuco (Chile). Methods: Review of clinical records of 14 patients with PCT operated on between March 2015 and December 2021. Results: By far the most common histological type was a myxoma (85.7%), followed by a papillary fibroelastoma (14.3%). Mean age was 62 years (39-85), 9 were women and 5 men. The most common associated medical conditions were Congestive Heart Failure (CHF), Arterial Hypertension and Stroke The usual anatomical location was the left atrium (92%). Surgical treatment was isolated resection in 92% of cases and along with a patch repair in the remaining patient. Conclusion: good results were obtained, similar to those described in the literature.

Cisto Hidático Cardíaco: Uma Causa Incomum de Bloqueio Atrioventricular Total / Cardiac Hydatid Cyst: An Uncommon Cause of Complete Atrioventricular Block

Resumo A hidatidose é uma zoonose causada pelo Echinococcus granulosus, levando à formação de cistos nos órgãos acometidos. O envolvimento cardíaco é raro e pode causar diversas complicações secundárias à ruptura, embolização ou compressão. Seu diagnóstico é desafiador, sendo confirmado por meio de dados relacionados a manifestações clínicas, exposição ambiental, exames laboratoriais e de imagem. A ressecção cirúrgica é necessária na maioria dos casos, sendo indicada a associação com terapia antiparasitária. No presente artigo, descreve-se um caso de cisto hidático cardíaco associado a bloqueio atrioventricular total em paciente jovem, com necessidade de implante de marcapasso, uma apresentação atípica e pouco relatada na literatura.

Abstract Hydatidosis is a zoonosis caused by Echinococcus granulosus, leading to the formation of cysts on involved organs. Cardiac involvement is rare and can cause a wide range of complications secondary to rupture, embolization, or compression. Its diagnosis is challenging, and is generally confirmed through data related to clinical manifestations, environmental exposure, and laboratory and imaging exams. Surgical removal is necessary in most cases, in which an association with antiparasite therapy is recommended. The present article describes a case of a cardiac hydatid cyst associated with a complete atrioventricular block (AVB) in a young adult patient, with the need for a pacemaker implant, an atypical presentation, and scarce reports in the literature.

Lipoma cardiaco / Cardiac masses

Resumen Las masas cardiacas son entidades raras que cursan con un espectro muy variado de manifestaciones clínicas que van desde cuadros asintomáticos hasta compromiso hemodinámico severo. Entre las lesiones benignas, los lipomas cardiacos son los segundos en frecuencia. Corresponden principalmente a lesiones neoplásicas benignas; no obstante, se pueden presentar otras patologías como trombos, vegetaciones y variantes de la normalidad. Gracias a la disponibilidad de técnicas de imagen de alta definición, como la ecocardiografía, la TC y la RM, ha aumentado su detección y tratamiento temprano. En el ámbito terapéutico se ofrece manejo quirúrgico, pues las imágenes no permiten la caracterización y diferenciación fidedigna de la naturaleza de las masas cardiacas. Se describe el caso de una paciente sin antecedentes cardiovasculares, con historial de disnea crónica, en quien se identificó, a través de estudios imagenológicos, masa cardiaca adosada al ventrículo con deformación leve de cavidades derechas. Fue llevada a resección quirúrgica y por histopatología se confirmó lipoma. Este hallazgo es el más infrecuente de todos los tumores cardíacos benignos. Se resalta la importancia del conocimiento de esta enfermedad para dar tratamiento eficaz y oportuno en aras de evitar complicaciones que impacten en morbimortalidad.

Abstract Cardiac masses are rare entities that present with a very varied spectrum of clinical manifestations that go from asymptomatic to pictures with severe hemodynamic compromise, within these, cardiac lipomas are the second in frequency within benign lesions. They mainly correspond to benign neoplastic lesions, however, other pathologies such as thrombi, vegetations and variants of normality can occur. Thanks to the availability of high-definition imaging techniques, echocardiography, CT and MRI have increased early detection and treatment. In the therapeutic field, surgical management is offered, since the images do not allow the characterization and reliable differentiation of the nature of cardia masses. It is described the case of a patient with no cardiovascular history, with chronic dyspnea, in whom the presence of CM attached to the ventricle with slight deformation of the right cavities is identified by imaging studies. Was taken to surgical resection confirming the presence of lipoma by histopathology, being this finding the rarest of all benign cardiac tumors. We highlight the importance of knowing this pathology to provide effective and timely treatment to avoid complications that impact morbidity and mortality.

Síncope por mixoma auricular: a propósito de un caso clínico / Syncope due to atrial myxoma: presentation of a clinical case / Síncope por mixoma atrial: a propósito de um caso clínico

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

Tumores cardíacos pediátricos: características de imagens ecocardiográficas / Pediatric cardiac tumors: echocardiographic imaging features

Tumores cardíacos (TC) em crianças são lesões muito raras, mas seu diagnóstico é fundamental para a conduta adotada para o paciente. A ecocardiografia é a modalidade de imagem cardiovascular mais utilizada na prática clínica para o diagnóstico inicial de TC em pacientes pediátricos. Conhecer as características ecocardiográficas das TCs pode possibilitar um diagnóstico cada vez mais precoce e a identificação de sua etiologia mais provável. Os TCs primários e benignos são os mais frequentes na população pediátrica. Entre os TCs benignos, os mais frequentes em fetos e neonatos são rabdomiomas e teratomas. Em crianças e adolescentes, rabdomiomas e fibromas são os mais comuns. Neste artigo, descrevemos as características ecocardiográficas dos principais TCs em idades pediátricas.(AU)

Cardiac tumors (CTs) in children are very rare, but their diagnosis is crucial for patient management. Echocardiography is the most commonly used cardiovascular imaging modality in clinical practice for the initial diagnosis of CTs in pediatric patients. Knowing the echocardiographic characteristics of CTs can enable an increasingly early diagnosis and the identification of its most likely etiology. Primary and benign CTs are the most frequent types in the pediatric population. Among benign CTs, the most frequent in fetuses and neonates are rhabdomyomas and teratomas. In children and adolescents, rhabdomyomas and fibromas are more common. Here we describe the echocardiographic characteristics of the most common CTs in pediatric patients.(AU)

Tumor cardíaco en edad pediátrica

RESUMEN Introducción: Los tumores cardíacos no son frecuentes en pediatría. Su incidencia estimada es del 0,001 % en estudios necrópsicos y de hasta 0,3 % en estudios ecocardiográficos. Objetivo: Mostrar la localización infrecuente de un mixoma en el ventrículo derecho. Presentación de caso: Lactante de 30 días de edad que comienza con dificultad respiratoria leve y al examen clínico se constata la presencia de soplo cardiaco; se admite en hospital Juan B. Viñas en Palma Soriano provincia de Santiago de Cuba, con la sospecha de cardiopatía congénita. Se realizan exámenes complementarios, el estudio radiológico muestra cardiomegalia, en la ecocardiografía en modo B se observa tumoración en ventrículo derecho y derrame pericárdico; se traslada al Cardiocentro Nacional Pediátrico "William Soler" donde se realiza la exptirpación parcial de la tumoración. El estudio histopatológico concluye mixoma del ventrículo derecho. Conclusiones: La ecocardiografía en modo B es una herramienta muy importante para el diagnóstico de las tumoraciones intracardiacas, brinda la localización, dimensiones y estimación del grado de repercusión de la masa intracardiaca. La indicación quirúrgica depende más del efecto hemodinámico de la tumoración que de su origen anatomopatológico.

ABSTRACT Introduction: Cardiac tumors are not frequent in pediatrics. Its estimated incidence is 0.001% in autopsy studies and up to 0.3% in echocardiographic studies. Objective: To show the rare location of a myxoma in the right ventricle. Case report: A 30-day-old infant started to have mild respiratory distress and the clinical examination revealed the presence of a heart murmur. He was admitted to Juan B. Viñas Hospital in Palma Soriano, Santiago de Cuba province under the suspicion of congenital heart disease. Complementary examinations were performed, the radiological study showed cardiomegaly, B-mode echocardiography revealed a tumor in the right ventricle and pericardial effusion. He was transferred to William Soler National Pediatric Cardio Institute, to the partial removal of the tumor. The histopathological study revealed a myxoma of the right ventricle. Conclusions: B-mode echocardiography is a very important tool for the diagnosis of intracardiac tumors, it provides the location, dimensions and estimation of the degree of repercussion of the intracardiac mass. The surgical indication depends more on the hemodynamic effect of the tumor than on its pathological origin.

Rabdomioma cardiaco en relación con tetralogía de Fallot y esclerosis tuberosa / Cardiac rhabdomyoma associated with tetralogy of Fallot and tuberous sclerosis

Resumen Los tumores cardiacos son inhabituales en la edad pediátrica; de ellos, el rabdomioma es el más prevalente. Su curso suele ser benigno. Los casos sintomáticos obedecen a su efecto mecánico obstructivo o a la presencia de arritmias. No es común su asociación con cardiopatías congénitas. Se presenta el caso de un lactante con rabdomiomas múltiples dentro del complejo de esclerosis tuberosa y portador de tetralogía de Fallot con estenosis infundibulovalvular grave, quien presentó crisis de hipoxia que requirió valvuloplastia pulmonar percutánea, la cual se complicó por arritmia supraventricular y tuvo un desenlace fatal.

Abstract Cardiac tumors are rare in pediatrics, and when they occur, rhabdomyomas are the most prevalent. They are generally benign; symptomatic cases are due to mechanical obstruction or arrhythmias. They are not commonly associated with congenital heart disease. We present the case of an infant with multiple rhabdomyomas as part of tuberous sclerosis complex, and tetralogy of Fallot with severe infundibular and valvular stenosis, who presented a hypoxic crisis requiring percutaneous pulmonary valvuloplasty which was complicated by supraventricular arrythmia and had a fatal outcome.

Tromboembolismo pulmonar como forma de presentación de neurofibrosarcoma cardíaco

RESUMEN Los tumores cardíacos pueden provocar síncope, falla cardíaca, fenómenos embólicos y muerte. Se requiere un elevado índice de sospecha debido a la similitud clínica con otras enfermedades sistémicas. Se describe el caso de una mujer de 26 años que empezó con dificultad respiratoria, tos, expectoración hemoptoica, hipotensión arterial y manifestaciones de insuficiencia cardíaca a predominio derecho. La ecocardiografía mostró una masa tumoral en aurícula derecha y múltiples imágenes de trombos a nivel del tronco de la arteria pulmonar, con signos de disfunción ventricular derecha e hipertensión pulmonar grave. Fue intervenida quirúrgicamente, pero el tumor era irresecable, pues infiltraba el pericardio y la vena cava inferior. La paciente falleció un día después de la operación. El estudio histológico confirmó que el tumor era un neurofibrosarcoma.

ABSTRACT Cardiac tumors can cause syncope, heart failure, embolic events, and death. A high index of suspicion is required due to the clinical similarity with other systemic diseases. Here is described the case of a 26-year-old woman who began with respiratory distress, cough, hemoptoic expectoration, low blood pressure and manifestations of heart failure predominantly on the right. The echocardiography showed a tumor mass in the right atrium and multiple images of thrombi at the level of the pulmonary artery trunk, with signs of right ventricular dysfunction and severe pulmonary hypertension. She underwent surgery, but the tumor was unresectable, as it infiltrated the pericardium and the inferior vena cava. The patient died one day after surgery. The histological study confirmed that the tumor was a neurofibrosarcoma.

Multidisciplinary approach to right ventricular myxoma

Abstract Right ventricular (RV) myxoma that obstructs the RV outflow tract is rare. Multimodality imaging is crucial due to the curved and triangular shape of the RV anatomy. Incomplete resection by the right atrial approach in cardiac myxomas may be prevented by preoperative imaging with echocardiography, computed tomography and magnetic resonance imaging to provide detailed visualization. Right ventriculotomy may be an alternative approach to the isolated atrial approach to get complete resection of RV myxoma in suitable patients. The preferred surgical treatment is not well defined for ventricular myxomas and careful preoperative planning is essential. Surgical resection should be performed as soon as possible to avoid outflow tract obstruction, which might result in sudden death. The collaboration between cardiologist and heart surgeon and the effective use of imaging tools are essential for successful treatment. In this article, diagnosis and treatment and the heart team approach to RV myxoma are discussed with a demonstrative patient.

Linfoma pericárdico primario a células T/NK: reporte de un caso / Primary pericardial T/NK cell lymphoma: case report / Linfoma pericárdico primário de células T/NK: relato de um caso

Los linfomas cardíacos primarios son un subtipo muy poco frecuente de tumor en los cuales la lesión primaria se encuentra en el corazón. Los tumores suelen ser infiltrantes y se localizan en la aurícula derecha, seguidos del pericardio. Su mortalidad es notablemente alta y el diagnóstico tardío es el principal factor para su mal pronóstico. Describimos el caso de un paciente que presentó shock obstructivo por derrame pericárdico profuso causado por un tipo raro de tumor cardíaco primario, un linfoma pericárdico de células T/NK.

Primary cardiac lymphomas are a rare subtype of lymphomas in which the primary lesion is in the heart. The tumors are usually located in the right atria, followed by the pericardium and are frequently infiltrative. Mortality is remarkably high in this group and the delayed diagnosis is the main factor for its poor prognosis. We describe the case of a patient that presented with obstructive shock due to profuse pericardial effuse caused by a rare kind of primary cardiac tumor, a T/NK cell pericardial lymphoma.

Os linfomas cardíacos primários são um subtipo de tumor muito raro, no qual a lesão primária está no coração. Os tumores geralmente são infiltrativos e localizam-se no átrio direito, seguidos pelo pericárdio. Sua mortalidade é notavelmente alta e o diagnóstico tardio é o principal fator que produz seu mau prognóstico. Descrevemos o caso de um paciente que apresentou choque obstrutivo devido a um derrame pericárdico profuso causado por um tipo raro de tumor cardíaco primário, um linfoma pericárdico de células T/NK.

Mixoma auricular izquierdo gigante como causa de obstrucción de la válvula mitral e hipertensión pulmonar severa

Resumen: Los tumores cardíacos primarios son raros, representan el 0.0017-0.03% en grandes series de autopsias, ocurren con mayor frecuencia entre mujeres en la quinta o sexta década de la vida y generalmente se desarrollan en las aurículas. Se presenta el caso clínico de una paciente de sexo femenino de 74 años que consulta a urgencias con sospecha de síndrome coronario agudo sin elevación del segmento ST y troponinas positivas. El ecocardiograma transtorácico evidenció la presencia de un mixoma auricular gigante con área de 11 cm2, que protruye a través de la válvula mitral, una presión sistólica de la arteria pulmonar de 110 mmHg y un resultado de dímero D positivo dando como diagnóstico un mixoma auricular izquierdo gigante que causa obstrucción mitral e hipertensión pulmonar, el cual fue remitido a otra institución para manejo quirúrgico.

Abstract: Primary cardiac tumors are rare, accounting for 0.0017-0.03% in autopsy series, occur more frequently among women between the fifth and sixth decade of life. A 74-year-old female patient presented with symptoms of acute coronary syndrome without ST segment elevation and positive troponins. The transthoracic echocardiogram showed the presence of a giant atrial myxoma with an area of 11 cm2, protruding through the mitral valve, a systolic pressure of the pulmonary artery of 110 mmHg and a positive D-dimer result, yielding a diagnosis of a giant left atrial myxoma that causes mitral obstruction and pulmonary hypertension, being referred to another institution for surgical management.

Resumo: Os tumores cardíacos primários são raros, representam o 0,0017-0,03% em grandes séries de autópsias, ocorrem mais frequentemente entre mulheres na quinta ou sexta década de vida e geralmente se formam nos átrios. Apresentamos o caso de uma paciente do sexo feminino de 74 anos, que apresentou sintomas de síndrome coronário agudo sem elevação do segmento ST e troponinas positivas. O ecocardiograma transtorácico revelou a presença de um mixoma atrial gigante com área de 11 cm2 projetando-se através da válvula mitral, uma pressão sistólica de artéria pulmonar de 110 mmHg e um resultado de D-dímero positivo, dando o diagnóstico de um mixoma atrial esquerdo gigante causando obstrução mitral e hipertensão pulmonar, que foi encaminhado para outra instituição para manejo cirúrgico.

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.

Actualización en enfermedad de kawasaki: diagnóstico, estudio y tratamiento / Kawasaki disease update: diagnosis, study and treatment

La enfermedad de Kawasaki (EK) es una vasculitis aguda de vaso mediano que afecta principalmente a niños menores de 5 años, que de no ser tratada, se asocia al desarrollo de aneurismas de las arterias coronarias en aproximadamente el 25% de los casos. Típicamente la EK se presenta con fiebre, cambios mucocutáneos y linfadenopatía. Sin embargo, EK es una enfermedad excepcional en la que las formas incompletas de la enfermedad son muy comunes y a menudo asociación sintomatologías atípicas. Éstas pueden crear un desafío diagnóstico para los tratantes y retrasar el inicio de la terapia. No existe un gold standard para el diagnóstico de EK pero hay estudios de laboratorio y hallazgos ecocardiográficos que permiten apoyar el diagnóstico en casos incompletos. El estudio con ecocardiograma debe realizarse lo más pronto posible cuando se sospecha el diagnóstico, pero no debe retrasar el inicio de tratamiento. El objetivo de la terapia en EK es disminuir el riesgo de desarrollar aneurismas de las arterias coronarias y de esta forma, la morbimortalidad asociada a dicha condición. El propósito de esta revisión es conocer las características clínicas y las posibles formas de presentación de esta patología, además del tratamiento actual.

Kawasaki disease (KD) is an acute vasculitis of the medium vessel that mainly affects children under 5 years old, which if it's not treated, is associated with the development of coronary artery aneurysms in approximately 25% of all cases. Typically, KD presents with fever, mucocutaneous changes and lymphadenopathy. However, EK is an exceptional disease in which incomplete forms of the disease are very common and atypical presentations often occur. These presentations may create a diagnostic puzzle for pediatricians and may delay the start of therapy. There is no specific study for the diagnosis of KD but there are laboratory studies and findings in ECG that support the diagnosis in atypical cases. The echocardiogram study should be performed as soon as possible when the diagnosis is suspected, but should not delay the treatment. The goal of treatment in KD is to reduce the risk of developing aneurysms of the coronary arteries and thereby decrease the morbidity and mortality associated with this condition.

Surgical Treatment of Cardiac Tumors: A Single Institutional Experience Over 12 Years

Introduction: Tumors of the heart represent an exceedinglyrare entity in cardiac surgery and literature regardingmanagement and outcome is less in comparison to other fieldsof cardiac surgery. 12 years of our experience in both diagnosisand optimal surgical treatment of this small but rare collectionof patients was formed into a detailed analysis of patientprognosis, mean survival and risk of tumor relapse matchedto the corresponding pathology. The overall objective of thepresent study was a thorough characterization of both primarycardiac tumor or tumor like mass and secondary malignanttumor mass in cardiac chambers, their nature as well as ageand gender distribution and management.Material and methods: 17 patients with cardiac tumors,who underwent open-heart surgery at Medical College andHospitals, Kolkata, for tumor excision between 2007 and2019 were analyzed retrospectively. Mean follow-up wasfrom 11 to 1 years.Results: There were 2 males and 15 female patients rangingin age from 7 years to 60 years, median age being 47 years.12 of these tumors were primary left atrial myxoma, 2 wereright atrial myxoma, 1 was right ventricular fibroma, 1 wasintravenous extension into right atrium of renal cell carcinomaand 1 was multiple inflammatory pseudo tumors in leftventricle. Overall operative survival was 88.3%. Operativemortality was 11.7%.Conclusion: Cardiac tumors remain challenging in theclinical setting. Early operation is recommended afterechocardiographic diagnosis as such patients can have suddendeath or severe cardiac failure during preoperative waitingperiod. Follow up should be maintained based upon thehistopathological diagnosis.

Tumor cardiaco fetal. Reporte de un caso / Fetal cardiac tumor. Report of a case

Resumen: ANTECEDENTES: Los tumores cardiacos fetales son excepcionales y se asocian con complicaciones que ponen en riesgo la vida del feto. Se diagnostican a partir del segundo trimestre y pueden provocar hidrops fetal no inmunitario, arritmias, compresión de los conductos de salida y muerte súbita. Es importante el seguimiento durante la gestación para detectar posibles complicaciones y establecer un plan de nacimiento. CASO CLÍNICO: Paciente de 35 años, multigesta, enviada a la unidad materno-fetal para valoración por embarazo de 24.2 semanas y feto con tumor cardiaco único, localizado en el ápex, de gran tamaño. No se identificó afectación de la función cardiaca, por lo que solo ameritó vigilancia prenatal. Al nacimiento, el recién nacido recibió tratamiento con everolimus, con reacción satisfactoria. CONCLUSION: El tratamiento y seguimiento de fetos con tumor cardiaco es de suma importancia para detectar complicaciones prenatales y establecer el plan de nacimiento en la unidad de tercer nivel de atención médica.

Abstract: BACKGROUND: Fetal cardiac tumors are rare, with a very low incidence, however; when they do occur, they are associated with life-threatening complications of the fetus. They are diagnosed from the second trimester and can cause non-immune fetal hydrops, arrhythmias, compression of outflow tracts, and sudden fetal death. Follow-up during pregnancy is important to detect possible complications and establish a birth plan. CLINICAL CASE: A 35-year-old multigest patient, sent to the fetal maternal unit by his treating physician for evaluation for 24.2-week pregnancy and fetus with a single cardiac tumor, located on the apex, of large size; and without compromise in cardiac function, so only prenatal surveillance was warranted. At birth, the newborn received everolimus treatment, with a good response. CONCLUSION: The case of a patient with a single pregnancy and fetus with a prenatal diagnosis of a large cardiac tumor is presented with a family history of hemangiomas. In this case, a follow-up approach to detect prenatal complications and establish a birth plan in a third level of medical care is critical for a good practice.

Cardiac myxoma with cartilaginous differentiation-An uncommon variant presented as mitral stenosis

The estimated incidence of primary cardiac tumors is extremely rare. Among it, cardiac myxoma represents the most common benign cardiac tumor constituting about 80% of cases. We are presenting a 30–year-old female with large left atrial myxoma. She was presented with severe dyspnea, palpitations, and systolic murmurs. On 2D echocardiography, left atrial mass obstructing mitral flow was noted. On cardiac magnetic resonance imaging, a single, large, mobile pedunculated mass lesion in left atrium attached to inferior interatrial septum with heterogeneous enhancement was noted, which was likely represented to be myxoma. The mass was surgically excised and valve repairing was done. We received large, solid, lobulated, gray white, soft-to-firm mass measuring 4.5 × 4.1 × 2.5 cm, and on microscopy showed cardiac myxoma with cartilaginous differentiation. We are presenting this case for its clinical, imaging, and uncommon histological features.

Linfoma pericárdico primario con compromiso hemodinámico a propósito de un caso / Primary pericardial lymphoma with hemodynamic disorder apropos of a case

RESUMEN El linfoma cardíaco primario es una enfermedad extremadamente rara y fatal del corazón. Es definido como un linfoma que compromete el corazón y el pericardio. Tiene un pronóstico pobre debido a la dificultad en su diagnóstico por lo inaccesible de su localización. Se presenta el caso de un hombre de 84 años de edad con antecedentes de salud que acudió con síntomas y signos de insuficiencia cardíaca. Se sospechó cardiopatía dilatada, pero en el ecocardiograma transtorácico se observó derrame pericárdico severo con masa adherida a la pared lateral de las cavidades derechas, sin dilatación de cavidades. Se realizó ventana pericárdica para drenar el derrame, sin poder estudiar el líquido extraído. Finalmente se realizó resonancia magnética nuclear que diagnosticó linfoma cardíaco, y teniendo en cuenta la ausencia de otras alteraciones en el resto del cuerpo, se definió como primario. No se pudo realizar diagnóstico histológico por negativa del paciente y familiares.

ABSTRACT Primary cardiac lymphoma is an extremely rare and fatal entity of the heart. It is defined as a lymphoma that compromises the heart as well as the pericardium. It has a poor prognosis because of the difficulty in its diagnosis, due to the inaccessibility of its location. Here is presented the case of an 84-year-old man with healthy history that came to our hospital with symptoms and signs of heart failure. The first suspicion was dilated cardiomyopathy, but in the transthoracic echocardiography was observed a large pericardial effusion with a mass attached to the side wall of the right chambers, without chamber dilation. A pericardial window was performed to drain the effusion, without being able to study the extracted fluid. Finally, a magnetic resonance imaging was performed resulting in a cardiac lymphoma diagnosis, that, taking into account the absence of other changes in the rest of the body, was defined as primary. No histological diagnosis could be developed due to refusal of the patient and his relatives.

Síndrome de hipertensión pulmonar secundario a mixoma auricular derecho, a propósito de un caso / Pulmonary hypertension syndrome secondary to right atrial myxoma apropos of a case

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.

Surgical resection of a cardiac lipoma of the right ventricle

Cardiac lipomas are rare, benign, encapsulated tumors which predominantly appear outside of the heart and less frequently inside the cardiac chambers. We describe a case of a right ventricular cardiac lipoma in a 72-year-old female patient who presented with palpitations. Surgical considerations, diagnostic modalities, and up-to-date literature are also presented and discussed.