Реферат
Resumen Introducción: Los rabdomiomas son los tumores cardiacos benignos más prevalentes en la etapa fetal y durante la infancia. Objetivo: Nuestro objetivo es dar a conocer nuestra experiencia durante 39 años en pacientes pediátricos con rabdomioma en nuestra institución, así como enfatizar la importancia de su detección, estudio y control por la asociación que tiene con esclerosis tuberosa (ET). Material y métodos: Realizamos un estudio retrospectivo y descriptivo, de enero de 1980 a marzo del 2018. Veinticuatro cumplieron con nuestros criterios, recogimos información respecto a la clínica y estudios de gabinete, así como su evolución y el tratamiento dado. Se les llamó vía telefónica para conocer su evolución y estado actual. Resultados: Encontramos 51 pacientes con diagnóstico de tumor cardiaco, de los cuales 24 eran rabdomiomas. El diagnóstico se hizo prenatal en 8 pacientes, 5 al nacimiento y en 11 durante el primer año de vida. Las manifestaciones clínicas más frecuentes fueron la presencia de soplo, arritmias, cianosis, disnea y diaforesis. En 17 de ellos se hizo diagnóstico de ET. La mitad tuvo seguimiento por neurología, 10 por dermatología, 8 por oftalmología y 4 con genética. La mitad se dejó en vigilancia, a 7 se les dio tratamiento médico y 5 requirieron cirugía. En cuanto a la evolución, 17 pacientes se mantuvieron estables, 5 presentaron regresión espontánea y 2 fallecieron. Conclusiones: El rabdomioma es un tumor benigno poco frecuente, su evolución puede ser maligna y asociado a ET ensombrece el pronóstico.
Abstract Introduction: Rhabdomyomas are the most prevalent benign heart tumors in the fetal stage and during childhood. Objective: Our objective is to make known our experience over 39 years in pediatric patients with rhabdomyoma in our institution, as well as to emphasize the importance of its detection, study and control due to the association it has with tuberous sclerosis (TS). Material and methods: We conducted a retrospective, descriptive and cross-sectional study, from January 1980 to March 2018. Twenty-four met our criteria, we collected information regarding the clinic and cabinet studies, as well as their evolution and the treatment given. They were called by telephone to know their evolution and current status. Results: We found 51 patients with a diagnosis of cardiac tumor, of which 24 were rhabdomyomas. The diagnosis was made prenatal in 8 patients, 5 at birth and in 11 during the first year of life. The most frequent clinical manifestations were the presence of murmur, arrhythmias, cyanosis, dyspnea, and diaphoresis. In 17 of them a diagnosis of TS was made. Half had follow-up by neurology, 10 by dermatology, 8 by ophthalmology and 4 with genetics. Half were left under surveillance, 7 were given medical treatment and 5 required surgery. Regarding the clinical evolution 17 patients remained stable, 5 presented spontaneous regression and 2 died. Conclusions: Rhabdomyoma is a rare benign tumor, its evolution can be malignant and associated with TS, it darkens the prognosis.
Тема - темы
Humans , Male , Female , Child , Rhabdomyoma/diagnosis , Rhabdomyoma/therapy , Heart Neoplasms/diagnosis , Heart Neoplasms/therapy , Retrospective StudiesРеферат
Los tumores cardiacos primarios fetales representan una entidad de baja incidencia en la práctica clínica. La gran mayoría corresponde a lesiones benignas, siendo el rabdomioma el tipo más común. Si bien, es frecuente la regresión espontánea de este tipo de lesiones durante el embarazo, los tumores cardiacos fetales pueden asociarse a complicaciones como obstrucción del flujo cardíaco, insuficiencia valvular, arritmias, insuficiencia cardíaca e hidrops fetal, pudiendo conducir incluso a la muerte fetal. El mayor desarrollo de técnicas imagenológicas ha permitido un aumento en el número y precisión de los diagnósticos prenatales de tumores cardiacos, generando al mismo tiempo, nuevos desafíos y alternativas en relación al abordaje terapéutico. El objetivo del presente artículo de revisión es exponer la evidencia actual en relación al diagnóstico prenatal, manejo, complicaciones y condiciones asociadas de los tumores cardiacos fetales más frecuentes.
Fetal primary cardiac tumors represent a low incidence entity in clinical practice. The vast majority corresponds to benign lesions, with rhabdomyoma being the most common type. Although spontaneous regression of this type of lesions during pregnancy is frequent, fetal cardiac tumors can be associated with complications such as obstruction of cardiac flow, valvular insufficiency, arrhythmias, heart failure and fetal hydrops, which can even lead to fetal death. The greater development of imaging techniques has allowed an increase in the number and precision of prenatal diagnoses of cardiac tumors, generating at the same time, new challenges and alternatives in relation to the therapeutic approach. The objective of this review article is to present the current evidence regarding the prenatal diagnosis, management, complications and associated condition s of the most frequent fetal cardiac tumors.
Тема - темы
Humans , Female , Pregnancy , Fetus/diagnostic imaging , Heart Neoplasms/diagnostic imaging , Rhabdomyoma/diagnostic imaging , Teratoma/diagnostic imaging , Ultrasonography, Prenatal , Fibroma/diagnostic imaging , Heart Neoplasms/therapy , Myxoma/diagnostic imagingТема - темы
Humans , Female , Aged , Calcinosis/diagnosis , Calcinosis/therapy , Diagnostic Imaging/methods , Mitral Valve/surgery , Echocardiography/methods , Magnetic Resonance Spectroscopy/methods , Diagnosis, Differential , Multidetector Computed Tomography/methods , Heart Neoplasms/complications , Heart Neoplasms/therapyРеферат
AbstractObjective:Cardiac myxoma in pregnancy is rare and the clinical characteristics of this entity have been insufficiently elucidated. This article aims to describe the treatment options and the risk factors responsible for the maternal and feto-neonatal prognoses.Methods:A comprehensive search of the literature of cardiac myxoma in pregnancy was conducted and 44 articles with 51 patients were included in the present review.Results:Transthoracic echocardiography was the most common diagnostic tool for the diagnosis of cardiac myxoma during pregnancy. Cardiac myxoma resection was performed in 95.9% (47/49); while no surgical resection was performed in 4.1% (2/49) patients (P=0.000). More patients had an isolated cardiac myxoma resection in comparison to those with a concurrent or staged additional cardiac operation [87.2% (41/47) vs. 12.8% (6/47), P=0.000]. A voluntary termination of the pregnancy was done in 7 (13.7%) cases. In the remaining 31 (60.8%) pregnant patients, cesarean section was the most common delivery mode representing 61.3% and vaginal delivery was more common accounting for 19.4%. Cardiac surgery was performed in the first, second and third trimester in 5 (13.9%), 14 (38.9%) and 17 (47.2%) patients, respectively. No patients died. In the delivery group, 20 (76.9%) neonates were event-free survivals, 4 (15.4%) were complicated and 2 (7.7%) died. Neonatal prognoses did not differ between the delivery modes, treatment options, timing of cardiac surgery and sequence of cardiac myxoma resection in relation to delivery.Conclusion:The diagnosis of cardiac myxoma in pregnancy is important. Surgical treatment of cardiac myxoma in the pregnant patients has brought about favorable maternal and feto-neonatal outcomes in the delivery group, which might be attributable to the shorter operation duration and non-emergency nature of the surgical intervention. Proper timing of cardiac surgery and improved cardiopulmonary bypass conditions may result in even better maternal and feto-neonatal survivals.
ResumoObjetivo:Mixoma cardíaco durante a gravidez é raro e as características clínicas dessa entidade não foram suficientemente esclarecidas. Este artigo tem como objetivo descrever as opções de tratamento e os fatores de risco responsáveis pelo prognóstico materno e fetal-neonatal.Métodos:Foi realizada uma pesquisa abrangente na literatura sobre mixoma cardíaco durante a gravidez e 44 artigos com 51 pacientes foram incluídos na presente revisão.Resultados:Ecocardiografia transtorácica foi a ferramenta de diagnóstico mais comum para o diagnóstico de mixoma cardíaco durante a gravidez. Ressecção do mixoma cardíaco foi realizada em 95,9% (47/49); enquanto não foi realizada ressecção cirúrgica em 4,1% (2/49) dos pacientes (P=0,000). Mais pacientes tiveram ressecção isolada do mixoma cardíaco em comparação com aqueles com operação cardíaca concomitante ou adicional [87,2% (41/47) vs. 12,8% (6/47), P=0,000]. A interrupção voluntária da gravidez foi feita em 7 (13,7%) casos. Nas restantes 31 (60,8%) pacientes grávidas, a cesariana foi o modo de parto mais comum, representando 61,3% e parto vaginal contabilizou 19,4%. A cirurgia cardíaca foi realizada no primeiro, segundo e terceiro trimestre em 5 (13,9%), 14 (38,9%) e 17 (47,2%) pacientes, respectivamente. Nenhuma paciente morreu. No grupo de parto, 20 (76,9%) recém-nascidos sobreviveram livres de eventos, 4 (15,4%) tiveram complicações e 2 (7,7%) morreram. Os prognósticos neonatais não diferiram entre os modos de parto, opções de tratamento, tempo de cirurgia cardíaca e sequência de ressecção mixoma cardíaco em relação ao parto.Conclusão:O diagnóstico de mixoma cardíaco durante a gravidez é importante. Tratamento cirúrgico de mixoma cardíaco em pacientes grávidas trouxe resultados favoráveis para as mães e os neonatos no grupo de parto, o que pode ser atribuído à duração mais curta da operação e à natureza não emergencial da intervenção cirúrgica. O momento adequado da cirurgia cardíaca e melhoria das condições de circulação extracorpórea podem resultar em sobrevivência materna e do feto-neonato ainda melhor.
Тема - темы
Female , Humans , Pregnancy , Heart Neoplasms/therapy , Myxoma , Pregnancy Complications, Neoplastic , Delivery, Obstetric , Fetal Death , Heart Atria , Heart Neoplasms/diagnosis , Myxoma/diagnosis , Myxoma/therapy , Prognosis , Pregnancy Complications, Cardiovascular/diagnosis , Pregnancy Complications, Cardiovascular/therapy , Pregnancy Complications, Neoplastic/diagnosis , Pregnancy Complications, Neoplastic/therapyРеферат
Primary cardiac tumors are rare with myxoma being the most common benign cardiac tumor. They are usually sporadic, affecting left atrium and frequently occur in women. They are known to cause valvular obstruction, thromboembolism and arrhythmias. We present a case of right atrial myxoma complicated by pulmonary embolism. The atrial myxoma was diagnosed on autopsy.
Тема - темы
Female , Heart Atria , Heart Neoplasms/diagnosis , Heart Neoplasms/therapy , Humans , Myxoma/diagnosis , Myxoma/therapy , Pulmonary Embolism/complications , Pulmonary Embolism/diagnosis , Pulmonary Embolism/therapy , Review Literature as Topic , Young AdultРеферат
Objetivos: Analizar el diagnóstico ecográfico, la evolución y el tratamiento de los tumores cardiacos fetales diagnosticados en el Hospital La Paz de Madrid, entre los años 1995 y 2010. Método: Se realizó un estudio retrospectivo descriptivo recogiendo los principales datos ecográficos, de la historia clínica materna, neonatal y pediátrica. Resultados: En el periodo de estudio se diagnosticaron ecográficamente 28 tumores cardiacos fetales, 21 casos fueron catalogados como rabdomiomas. Once de ellos tuvieron una regresión prácticamente completa, con ocho casos diagnosticados hasta el momento de esclerosis tuberosa. Hubo 2 muertes intraútero, 1 interrupción legal del embarazo y 4 diagnósticos ecográficos de rabdomiomas, no se confirmaron al nacimiento. Se diagnosticaron prenatalmente dos fibromas; de ellos una gestante optó por la interrupción legal del embarazo y en el otro caso se produjo la muerte neonatal. Dos neonatos fueron sometidos a cirugía con una resección completa del tumor, con resultado anatomopatológico de heman-gioma capilar en uno y teratoma en el otro. En un caso se realizó una biopsia que confirmó la presencia de un hemangiopericitoma auricular que se redujo posteriormente con quimioterapia. Conclusiones: Los tumores cardiacos son una patología poco frecuente. La mayor parte de ellos son rabdomiomas, cuya sin-tomatología y evolución depende de su localización. Estos suelen regresar espontáneamente, pero pueden asociarse al diagnóstico de esclerosis tuberosa, lo que empeora su pronóstico.
Aims: To analize the diagnosis, clinical course and management of fetal cardiac tumors diagnosed at La Paz Hospital (Madrid) between 1995 and 2010. Methods: We performed a retrospective descriptive study collecting the main ultrasound dates of the maternal, newborn and pediatric history. Results: During the study period, 28 fetal cardiac tumors were dignosed. Rhabdomyomas were diagnosed in 21 fetuses; 11 rhabdom-yomas returned almost completely. Eight of them were diagnosed of tuberous sclerosis up to the moment. Other two cases died in utero. One pregnant decided to be practised a miscarriage and four rhabdomyomas which were diagnosed by ultrasound, were not found in the newborns. Two fibroms were diagnosed by ultrasound; one of the pregnant woman decided to be practised a miscarriage and the other fetus died when he was born. Two newborns were operated, with the pathological anatomy result of a hemangioma and a teratoma. A biopsy was made that confirmed the presence of an atrial hemangiopericitoma which was treated by quimiotherapy. Conclusion: Fetal cardiac tumors are a rare disease. Most of them are rhabdomyomas which syntomatology and clinical course depend on its location. They usually regret spontaneously, but they can be associated with tuberous sclerosis, and this aggravates their prognosis.
Тема - темы
Humans , Adolescent , Adult , Female , Pregnancy , Middle Aged , Fetal Heart , Heart Neoplasms , Rhabdomyoma , Clinical Evolution , Fetal Diseases , Tuberous Sclerosis , Fibroma/epidemiology , Heart Neoplasms/epidemiology , Heart Neoplasms/therapy , Retrospective Studies , Risk Factors , Rhabdomyoma/epidemiology , Spain , Teratoma/epidemiology , Ultrasonography, PrenatalРеферат
OBJETIVO: analisar o diagnóstico, evolução e conduta terapêutica em cinco casos de tumores cardíacos primários diagnosticados no período pré-natal. MÉTODOS: no período de Janeiro 1997 a Dezembro 2008, 7.989 gestantes foram submetidas à avaliação ultrassonográfica morfológica fetal devido à presença de fatores de risco para malformações fetais. Foram selecionados os casos com massas hipercogênicas intracardíaca maiores que 1 mm diagnosticados na avaliação ultrassonográfica do coração fetal. O diagnóstico diferencial dos tumores foi realizado segundo as características ultrassonográficas das massas. RESULTADOS: em cinco fetos foram diagnosticadas massas hiperecogênicas intracardíaca correspondendo a uma taxa de prevalência de 0,06 por cento. A idade gestacional variou entre a 28ª e a 36ª semanas (média=31) e a idade materna variou de 23 a 45 anos (média=34,2). A localização mais frequente das massas foi o ventrículo esquerdo (100 por cento). Ecograficamente, todas as massas eram hipercogênicas, homogêneas, únicas ou múltiplas e bem delimitadas, compatíveis com diagnóstico de um rabdomioma. Nos casos em que os diâmetros das massas foram menores que 20 mm, a conduta foi expectante e não houve complicações no período pré-natal. Um caso com tumor de grande volume apresentou arritmia e insuficiência cardíaca na 35ª semana de gestação, sendo indicada a interrupção da gestação. Em 80 por cento dos casos foi observada associação com esclerose tuberosa no seguimento pós-natal. A regressão dos tumores cardíacos ocorreu em três casos (60 por cento) durante um seguimento médio de três anos. Todos os recém-nascidos eram do sexo masculino e sem antecedentes familiares de esclerose tuberosa. CONCLUSÕES: a avaliação ultrassonográfica morfológica fetal é a principal forma de detecção precoce dos tumores cardíacos primários. A avaliação cardíaca fetal é fundamental para a caracterização morfológica diferencial das massas cardíacas e para avaliação funcional cardíaca...
PURPOSE: to analyze the differential diagnosis, follow-up and therapeutic approach in five cases of primary cardiac tumors diagnosed during the prenatal period. METHODS: during the period from January 1997 to December 2008, 7989 pregnant women were submitted to morphological ultrasound due to the presence of risk factors for fetal malformations. Fetuses with hyperechogenic intracardiac masses larger than 1 mm diagnosed by ultrasound evaluation of the fetal heart, were selected for study. The differential diagnosis between the different tumor types was made on the basis of the ultrasound characteristics of the masses. RESULTS: five fetuses with hiperechogenic intracardiac masses were diagnosed, corresponding to a 0.06 percent prevalence rate. Gestational age ranged from 28 to 36 weeks (mean: 31), and maternal age ranged from 23 to 45 years (mean: 34,2). The most frequent location of the masses was the left ventricle (100 percent). Echographically, all masses were single or multiple, hyperechogenic, homogeneous and well delimited, compatible with a diagnosis of rhabomyoma. In cases in which the diameters of the masses were less than 20 mm, an expectant conduct was followed and no complications occurred during the prenatal period. One case with a huge tumor presented arrhythmia and cardiac insufficiency during the 35 gestational weeks, and the interruption of pregnancy was indicated. Tuberous sclerosis was associated in four cases (80 percent) and the diagnosis was confirmed during the postnatal follow-up. CONCLUSIONS: fetal morphological ultrasonography is the main form of early detection of primary cardiac tumors. The fetal cardiac evaluation is of fundamental importance for the differential morphological characterization of cardiac masses and for the evaluation of cardiac function. Rhabdomyomas are the most common type of fetal tumor. An expectant pre and postnatal conduct is followed, with a low risk of complications and with the possibility of spontaneous...
Тема - темы
Female , Humans , Infant, Newborn , Male , Pregnancy , Fetal Diseases , Heart Neoplasms , Rhabdomyoma , Fetal Diseases/therapy , Fetal Diseases , Heart Neoplasms/therapy , Heart Neoplasms , Rhabdomyoma/therapy , Rhabdomyoma , Ultrasonography, PrenatalТема - темы
Adult , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Combined Modality Therapy , Heart Neoplasms/pathology , Heart Neoplasms/therapy , Humans , Lymphoma, Large B-Cell, Diffuse/pathology , Lymphoma, Large B-Cell, Diffuse/therapy , Magnetic Resonance Imaging , Male , Neoplasm Recurrence, Local/pathology , Neoplasm Recurrence, Local/therapy , Radiotherapy Dosage , Stem Cell Transplantation , Tomography, X-Ray Computed , Treatment OutcomeРеферат
Primary cardiac lymphoma is extremely rare. In this care report, we present the case of a 46-year-old man with primary cardiac lymphoma involving left atrium and interatrial septum, presenting as dyspnea palpitation and irregular heart beat. The diagnosis was obtained by transthoracic echocardiography and surgical biopsy with subxiphoid approach which revealed diffuse large B-cell non-Hodgkin's lymphoma, CD 20+. After 8 courses of chemotherapy, the patient achieved complete remission. After 4 months, however, he developed exertional dyspnea. Right atrial recurrent lymphoma extension was diagnosed. He is currently under external radiotherapy treatment. Patients with primary cardiac lymphoma have a very poor prognosis
Тема - темы
Humans , Male , Heart Neoplasms/therapy , /therapy , Echocardiography, Transesophageal , Biopsy , Antineoplastic Combined Chemotherapy Protocols , Radiotherapy , Lymphoma/diagnosis , Heart Atria , DyspneaРеферат
Primary malignant cardiac tumors are extremely rare, but their associated mortality is very high. The clinical presentation is oñen variable and nonspecific; by the time symptoms appear, the tumor has usually evolved to a large intracardiac and paracardiac mass causing considerable hemodynamic involvement, regional invasión and distant dissemination. We report a 26 year-old female with a right atrium angiosarcoma with a surprisingly favorable outcome at 2 years follow-up after treatment with a combination of neoadjuvant chemotherapy and surgical resection. She presented with chest tightness, shortness of breath at effort, atypical chest pain, palpitations, asthenia, weight loss and profuse perspiration. Physical examination and irnaging diagnostic procedures, identified a pericardial effusion and a pathologic cardiac mass. The diagnosis of a malignant angiosarcoma was suggested afier computed tomography transthoracic and transesophageal echocardiography It was confirmed at surgery by a biopsy followed by histopathology and immunohistochemistry.
Los tumores cardíacos malignos son extremadamente raros y su mortalidad es muy alta. El cuadro clínico es variable e inespecífico: frecuentemente cuando aparecen síntomas ya existe una gran masa intracardíacayparacardíaca que causa compromiso hemodinámico grave, además de invasión locorregionaly diseminación a distancia. Comunicamos un caso especial de una mujerjoven que tuvo una evolución sorprendentemente favorable después de un tratamiento combinado con quimioterapia neoadyuvante y resección quirúrgica. La paciente presentó opresión precordial, disnea de esfuerzo, dolor torácico atípico, palpitaciones, astenia, baja de peso y sudoración profusa. El examen físico y exámenes complementarios, especialmente de imágenes, identificaron un derrame pericárdico y una masa cardíaca patológica. La tomografía computada y la ecocardiografía, particularmente la transesofágica, sugirieron el diagnóstico de angiosarcoma maligno, que se confirmó en una intervención quirúrgica que incluyó una biopsia con estudios histopatológicos e inmunohistoquímicos. Se discuten las alternativas del diagnóstico y del tratamiento, y se revisan la epidemiología y los recursos terapéuticos actuales en la literatura. Este caso ilustra la utilidad de la ecocardiografía transesofágica, para el diagnóstico de estos tumores y se plantea que el uso de nuevas alternativas quimioterapias asociadas a la extirpación quirúrgica pueden mejorar la sobrevida que, en nuestra paciente, alcanza dos años libre de síntomas.
Тема - темы
Adult , Female , Humans , Heart Neoplasms/therapy , Hemangiosarcoma/therapy , Biopsy , Combined Modality Therapy , Echocardiography, Transesophageal , Heart Atria , Heart Neoplasms/pathology , Heart Neoplasms , Hemangiosarcoma/pathology , Hemangiosarcoma , Pericardial EffusionРеферат
The most common tumours of the heart are metastatic in origin, but the most common primary cardiac tumour in adults is a myxoma. We present a recent classic case of myxoma and review the topic of cardiac tumours as a whole
Тема - темы
Humans , Female , Myxoma/diagnosis , Heart Neoplasms/pathology , Heart Neoplasms/therapy , Echocardiography , Tomography, X-Ray Computed , Magnetic Resonance Imaging , Cardiac CatheterizationРеферат
A 44-year-old woman had a transient ischemic stroke, fibroelastoma of the mitral valve being the source of the embolus. The patient evolved with neutropenia induced by ticlopidine after 10 days of treatment. We report the major clinical features, therapeutical options, and medicamentous toxicity resulting from the use of antiplatelet drugs
Тема - темы
Humans , Female , Adult , Fibroma/complications , Heart Neoplasms/complications , Ischemic Attack, Transient/etiology , Mitral Valve , Fibroma/diagnosis , Fibroma/therapy , Heart Neoplasms/diagnosis , Heart Neoplasms/therapy , Heart Valve Diseases/complications , Heart Valve Diseases/diagnosis , Heart Valve Diseases/therapy , Neutropenia/chemically induced , Platelet Aggregation Inhibitors/adverse effects , Ticlopidine/adverse effectsРеферат
The term myxosarcoma is currently not used in standard classification for soft tissue tumors, but restricted to cardiac tumors. Primary cardiac myxosarcoma is a very rare disease and is difficult to differentiate from myxoma clinically and pathologically. We report a case of left atrial myxosarcoma with widespread systemic metastasis in a 21-yr-old male. The patient presented with sudden onset of intermittent dyspnea and orthopnea. Echocardiography showed a mobile, pedunculated tumor, 7.5x5x2 cm in size, at left atrium. Histologically, the excised tumor showed an amorphous finely fibrillar and mucinous stroma, in which irregular cords and clusters of lepidic cells and large stellate cells with plump vesicular nuclei resembled the usual type of cardiac myxoma were noted. And it showed focally cellular area with great nuclear pleomorphism and frequent mitoses. The patient received combination chemotherapy, peripheral blood stem cell collection transplantation and operations for systemic metastases in the brain, skeletal muscle and lung. He is alive at present 37 months after initial diagnosis and has no more new metastatic lesion.
Тема - темы
Adult , Humans , Male , Heart Neoplasms/therapy , Heart Neoplasms/pathology , Myxosarcoma/therapy , Myxosarcoma/secondary , Myxosarcoma/pathologyРеферат
OBJECTIVE: Heart transplantation has become available as a possible treatment for patients with malignancies. Primary cardiac malignant tumors are extremely rare but several patients have been treated with this modality. Whether survival is improved over the direct removal of tumor or heart transplantation is not known. We compiled data regarding malignant primary heart tumors that have been treated at various transplant centers in order to determine if early diagnosis and treatment with cardiac transplantation has resulted in an improved mortality. METHODS: A total of 173 questionnaires were sent to the heart transplant centers across the United States and Canada inquiring about those patients who were found to have a primary cardiac malignant tumor and who underwent heart transplantation. Cases reported in the literature, which had undergone transplantation, were also reviewed by a search in MEDLINE. RESULTS: Twenty-four cases were collected. The overall survival time was from 1 month to 66 months. The actuarial survival was 54% at 12 months, 45% at 24 months and 35% at 36, 48 and 60 months respectively. Metastases were present in 10 out of the 14 deceased patients, possibly being one of the major factors affecting survival. Only 1 living patient developed metastases. CONCLUSION: Survival rates of patients with primary cardiac malignancies treated with resection, radiation, chemotherapy, or a combination of them (conventional therapy) versus heart transplantation are similar. Early diagnosis and resection are the most important factors for a better outcome, however these factors will not guarantee success since the presence or development of metastasis is a major contributor to death in both groups of patients
Тема - темы
Humans , Male , Female , Child , Adolescent , Adult , Middle Aged , Heart Neoplasms/surgery , Heart Transplantation , Canada/epidemiology , United States/epidemiology , Health Care Surveys , Heart Neoplasms/mortality , Heart Neoplasms/therapy , Survival Rate , Heart Transplantation/mortalityРеферат
Primary cardiac tumors are rare entities with a frequency between 0.0017 to 0.28. We report a 53 year old male with a primary cardiac myofibroblastic sarcoma that presented with a hemorrhagic pericardial effusion. A review of the literature is presented with a brief discussion of the clinical presentation diagnosis and treatment of benign and malignant primary cardiac tumors
Тема - темы
Humans , Male , Middle Aged , Heart Neoplasms , Sarcoma , Diagnosis, Differential , Pericardial Effusion/etiology , Heart Atria , Heart Neoplasms/diagnosis , Heart Neoplasms/therapy , Sarcoma/diagnosis , Sarcoma/therapyТема - темы
Humans , Heart Neoplasms/diagnosis , Heart Neoplasms/classification , Heart Neoplasms/therapyРеферат
Homem de 48 anos, submetido à excisäo completa de leiomiossarcoma do átrio esquerdo. Portador de um nódulo próximo à glândula supra-renal direita, o tumor cardíaco foi achado casualmente durante exame ecocardiográfico, como parte da investigaçäo cardiológica pré-operatória da cirurgia abdominal, uma vez que apresentava dispnéia aos esforços. Os leiomiossarcomas cardíacos säo muito raros e, na maioria dos casos, constituem-se em achados necroscópicos casuais. Neste relato säo relatados os aspectos diagnósticos, com especial ênfase ao exame ecocardiográfico, bem como os aspectos terapêuticos e prognósticos com base em extensa revisäo da literatura
Тема - темы
Humans , Male , Middle Aged , Leiomyosarcoma/diagnosis , Heart Neoplasms/diagnosis , Retroperitoneal Neoplasms/secondary , Angiocardiography , Echocardiography, Transesophageal , Heart Atria/surgery , Heart Atria/pathology , Leiomyosarcoma/therapy , Leiomyosarcoma/epidemiology , Heart Neoplasms/therapy , PrognosisРеферат
En nuestro estudio revisamos los expedientes de 20 pacientes sometidos a pericardietomía en el Instituto Nacional de Cardiología "Ignacio Chávez" en un periodo entre los meses de agosto de 1987 y septiembre de 1992. Se trata de 9 hombres y 11 mujeres, con edades comprendidas entre 18 y 57 años (promedio 35.8). Las causas incluyeron: derrame recidivante 5 casos, pericarditis constrictiva 4, urémica 2, síndrome pospericardiectomía 2 y otras con menor frecuencia: neoplasia 1, postraumática 1 y secundaria a radioterapia 1. El diagnóstico se realizó en base a: cuadro clínico, radiografía torácica, ecocardiografía bidimensional y cateterismo cardiaco. Todos los pacientes se sometieron a esternotomía y las resección del pericardio fue ampliada hasta el borde anterior de ambos nervios frénicos. La monitorización incluyó catéter venoso central y línea arterial en todos los casos y en 12 (60 por ciento) catéter de flotación pulmonar. Todas las intervenciones contaron con equipo de circulación extracorpórea en standby. La mortalidad operatoria fue de 1 caso, como consecuencia de bajo gasto cardiaco progresivo y falla multiorgánica en un paciente urémico. La mortalidad a 1 año se incrementó a 2 casos (10 por ciento) con la inclusión de un paciente con neoplasia cardiaca que correspondía a un rabdomiosarcoma del ventrículo izquierdo. Las complicaciones postoperatorias incluyeron: arritmias supraventriculares (10 por ciento), bloqueo incompleto de la rama derecha del haz de His 1 caso (5 por ciento), sangrado postoperatorio 1 caso (5 por ciento) y mediastinitis 1 caso (5 por ciento). El estudio anatomopatológico de 17 piezas quirúrgicas demostró: pericarditis crónica inespecífica en 6 casos (35 por ciento), fibrosis e infiltración cálcica en 3 (17 por ciento), perivasculitis crónica en 3 (17 por ciento), granulomatosis crónica en 2 (11 por ciento), pericarditis fibrinopurulenta en 2 (11 por ciento) y fibrosis hialina en uno (5 por ciento). El seguimiento a 25 meses, promedio, demostró una mejoría en la clase funcional: clase I 72 por ciento y clase II 27 por ciento. Los resultados de este estudio se correlacionan bien con los reportados en otras series. La pericarditis más común en nuestro medio sigue siendo la idiopática