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Background: Cardiac myxomas are the most common primary benign tumour of the heart. Most of them occur between the 4th and 6th decade of life, are most frequent in the woman, and most frequently localized in the left atrium. Case summary: We present a case of a 41-year-old female who presented with a history of left-sided heart failure. A left atrial mass of 87 × 88 × 65â mm was documented by cardiac magnetic resonance. She was taken to surgical resection of the mass. Histopathologic findings were diagnostic of cardiac myxoma. Generally, myxomas that are bigger than 6â cm are associated with the worst prognosis. Discussion: Primary cardiac tumours are mostly benign, being in 50% of the cases a cardiac myxoma. The rest of them correspond to papillary fibroelastoma (26%), fibromas (6%), lipomas (4%), and others including calcified tumours, haemangiomas, teratomas, cysts, and rhabdomyomas. Our clinical case illustrates an unusual and rare presentation of cardiac myxoma with a double mitral lesion.
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Resumen Hasta hace algunas décadas los tumores cardiacos tenían solo un interés académico dada su baja tasa de presentación; sin embargo, con el advenimiento del baipás cardiopulmonar, el manejo quirúrgico comienza a ser posible y, más recientemente, junto con la investigación farmacológica, contribuyen al desarrollo de nuevas estrategias de tratamiento para estos pacientes. La incidencia estimada de los tumores cardiacos es baja y tiene un comportamiento generalmente benigno y manifestaciones clínicas inespecíficas. Estos tumores en la edad pediátrica se asocian a esclerosis tuberosa, una enfermedad de patrón de herencia autosómica dominante que se caracteriza por una alteración en la diferenciación y multiplicación celular (hamartomas) en diferentes sistemas. Desde el punto de vista cardiaco, el grado de morbilidad está condicionado por el tamaño y la localización tumoral dentro de la cavidad, y tiene riesgo de obstruir los tractos de salida ventriculares, alterar la función valvular o producir trastornos de conducción. Algunos pacientes solo requieren seguimiento clínico durante el transcurso de su vida, pero existe la posibilidad de regresión tumoral; otros se benefician de una resección quirúrgica por su impacto hemodinámico o alteraciones del ritmo cardiaco. Sin embargo, no todos son candidatos quirúrgicos, en cuyo caso el manejo con inhibidores m-TOR ha surgido como una alternativa terapéutica. Se presenta el caso de tres neonatos con esclerosis tuberosa y rabdomioma cardiaco, diagnosticados en etapa prenatal, a quienes se les realizó un abordaje terapéutico individualizado, basado en las opciones actuales disponibles para este grupo de pacientes.
Abstract Up to a few decades ago, cardiac tumours were only of academic interest given their low presentation rate. However, with the advances in cardiopulmonary, surgical management began to be possible, and more recently, they contribute to the development of new treatment strategies for these patients. The estimated incidence of cardiac tumours is low and are generally benign with non-specific clinical signs and symptoms. In paediatrics, these tumours are associated with tuberous sclerosis, a disease with a dominant autosomal inheritance pattern, which is characterised by an alteration in cell differentiation and multiplication (hamartomas) in different systems. From a cardiac point of view, the morbidity level is determined by the size and location of the tumour within the cavity, the risk of obstructing ventricular outflow tracts, alterations in valvular function, and conduction disorders. Some patients only require clinical follow-up all their lives, as there is the risk of tumour regression. Others may benefit from surgical resection due to its haemodynamic impact or changes in cardiac rhythm. However, not everyone is a candidate for surgery, in which case, management with m-TOR inhibitors has emerged as a therapeutic alternative. The case is presented on three neonates with tuberous sclerosis and a cardiac rhabdomyoma, diagnosed in the prenatal stage. An individualised therapeutic approach was made based on the current options available for this group of patients.
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Humanos , Masculino , Feminino , Recém-Nascido , Neoplasias Cardíacas , Arritmias Cardíacas , Rabdomioma , Recém-Nascido , EverolimoRESUMO
Benign primary cardiac tumours are rare, with lipomas accounting for <9% of them. Their presentation varies depending on the size and location of the tumour, with the majority of the cases being asymptomatic. We are presenting a case of an extremely rare primary heart-tumour infiltrating the right ventricle (RV) compromising its function. RV lipomas are so unusual that there are no clear treatment guidelines. In this case, we decided to treat the patient with surgical resection of the tumour. Although a total resection was not possible, due to the tumoural proximity to vital structures, a great portion of the tumour was removed, alleviating the patient's symptoms.
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Procedimentos Cirúrgicos Cardíacos/métodos , Neoplasias Cardíacas/cirurgia , Lipoma/cirurgia , Feminino , Neoplasias Cardíacas/diagnóstico , Ventrículos do Coração , Humanos , Lipoma/diagnóstico , Adulto JovemRESUMO
BACKGROUND: Primary cardiac tumours are rare in children. Against this backdrop, Doppler echocardiogram is the main diagnostic procedure, while electrocardiogram (ECG) usually plays a secondary role, by detecting tumoural consequences as cardiac arrhythmias and chambers overload. We describe a case where an electrocardiographic sign was the cornerstone to diagnosis and surveillance of an infant with a cardiac rhabdomyoma. CASE SUMMARY: A female infant was referred for cardiac evaluation to elucidate an electrocardiographic abnormality, detected during investigation of seizures. She had recently been diagnosed with epilepsy and was under three different anticonvulsants for appropriate control. Cardiovascular symptoms were absent. Skin inspection revealed hypochromic macules. Respiratory and cardiovascular examinations were normal, as well as laboratorial tests and chest radiography. Electrocardiogram (ECG) showed dome-shaped ST-segment elevation in V2 and V3. Transthoracic echocardiogram unveiled a single hyper-echogenic node (0.4 cm2) in the interventricular septum. Cardiac chambers had normal size and function and Doppler analysis was also normal. No specific medication was used to treat the tumour. During follow-up, she remained free of cardiac symptoms. Eighteen months after her first visit to the cardiologist, routine clinical assessment, ECG, and transthoracic Doppler echocardiogram normal results stated the spontaneous and complete involution of the tumoural lesion. DISCUSSION: Convex ST-segment elevation, generally related to myocardial injury, is unusual in paediatric patients. Once it occurs in asymptomatic individuals within this age bracket, exclusion of cardiac tumours is mandatory. However, data regarding the accuracy of such electrocardiographic marker in this clinical setting are still to be defined.
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Resumen Introducción: Las masas cardiacas son una entidad heterogénea, que incluye lesiones neoplásicas y no neoplásicas y se clasifican en primarias y secundarias o metastásicas. Métodos: Estudio observacional analítico de tipo cohorte retrospectivo. Resultados: Se incluyeron un total de 73 individuos de los cuales se analizaron los datos de 66. Las masas cardiacas se diagnosticaron con mayor frecuencia en mujeres (53%). El síntoma más frecuente fue disnea (35%), seguido por dolor torácico (28%); otras manifestaciones comunes fueron fiebre (22%), pérdida de peso (22%) y focalización neurológica (22%). 44 pacientes fueron llevados a intervención quirúrgica o toma de biopsia de lesiones primarias encontrando como principal diagnóstico mixoma en 18 de ellos (27%); el resto de neoplasias primarias cardiacas fueron raras, fibroelastoma 2 casos (3%), al igual que rabdomioma y rabdomiosarcoma con solo un caso; el compromiso secundario fue frecuente con 18 casos (27%) representados principalmente por linfomas con 5 casos (8%), tumores primarios renales con 4 casos (6%) y cáncer de pulmón con 3 casos (5%). Dentro del seguimiento, el 77% estaban vivos en los siguientes seis meses del diagnóstico; la principal causa de muerte fue la progresión de la enfermedad oncológica (8 pacientes), seguida por ataque cerebrovascular (3 pacientes) y choque postoperatorio (2 pacientes). Conclusiones: Los pacientes con masas cardíacas con frecuencia tienen síntomas inespecíficos incluidos en tres ejes principales: insuficiencia cardíaca, síntomas generales y embolia sistémica. La frecuencia de mixoma auricular y las neoplasias secundarias fue similar.
Abstract Introduction: Cardiac masses are a heterogeneous condition, and include neoplastic and non-neoplastic lesions that can be classified as primary, secondary, or metastatic. Methods: An analytical observational study of a retrospective cohort. Results: A total of 74 subjects were included, of which the data of 66 of them were analysed. Cardiac masses were diagnosed more often in women (53%). The most frequent symptom was dyspnoea (35%), followed by chest pain (28%). Other common signs were fever (22%), weight loss (22%), and a neurological focus (22%). A total of 44 patients had surgery or a biopsy taken of the primary lesions found. The primary diagnosis was a myxoma in 18 (27%). The rest of the primary cardiac tumours were rare, with a fibroelastoma in 2 cases (3%), rhabdomyoma 2 cases (3%), and only one case of rhabdomyosarcoma. There was secondary involvement in 18 cases (27%), mainly as lymphomas in 5 cases (8%), primary renal tumours in 4 cases (6%), and lung cancer in 3 cases. During follow-up, 77% were alive in the six months following the diagnosis. The main cause of death was progression of the oncological disease (8 patients), followed a cerebrovascular attack (3 patients), and post-surgical shock (2 patients). Conclusions: Patients with cardiac masses of have non-specific symptoms included in three main groups: heart failure, general symptoms, and systemic embolisms. The frequency of atrial myxoma and secondary tumours was similar.