RESUMO
INTRODUÇÃO: A agenesia do pericárdio é uma patologia rara, com prevalência de 1:14.000 casos pela literatura. Seu diagnóstico é habitualmente incidental, visto que a maioria dos casos são assintomáticos. Pode ocorrer de forma total ou parcial, sendo três vezes mais prevalente entre indivíduos do sexo masculino. DESCRIÇÃO DO CASO: Paciente do sexo feminino, 28 anos de idade com história de derrame pleural à esquerda recorrente desde 2012 associada a dor ventilatório-dependente em hemitórax esquerdo. Ao exame físico apresentava desvio de ictus para linha axilar posterior, sem outros achados. Eletrocardiograma com desvio extremo do eixo para a direita e distúrbio de condução pelo ramo direito, Holter 24h com ausência de ectopias ou pausas, Ecocardiograma com desvio extremo do ápice cardíaco (ambos os ventrículos) para região axilar posterior e hipermobilidade cardíaca durante o exame, além de Ressonância Magnética Cardíaca evidenciando levoposição excessiva do coração, hipermobilidade ventricular e interposição de parênquima pulmonar entre o tronco pulmonar e a aorta ascendente, todos achados sugestivos de agenesia de pericárdio. DISCUSSÃO: A agenesia de pericárdio, embora frequentemente assintomática, pode manifestar-se com sintomas variados como dor torácica, dispneia, tonturas ou síncope, decorrentes de um defeito congênito no desenvolvimento da membrana pleuropericárdica esquerda. Em casos de defeitos parciais, onde os sintomas são mais prevalentes, pode ocorrer herniação e encarceramento do apêndice atrial esquerdo ou do ventrículo esquerdo, situações que demandam reparo cirúrgico urgente utilizando materiais sintéticos ou pericárdio bovino. No caso relatado, os achados de desvio extremo do ápice cardíaco e interposição de parênquima pulmonar entre o tronco pulmonar e a aorta ascendente indicam agenesia total. A decisão contra a intervenção cirúrgica foi baseada na ausência de risco iminente de morte súbita, sublinhando a importância de uma avaliação criteriosa dos riscos versus benefícios da cirurgia em casos de agenesia completa.
Assuntos
Humanos , Feminino , Adulto , Pericárdio/anormalidades , Derrame Pleural , EcocardiografiaRESUMO
A pericardite constritiva (PC) é uma condição na qual a cicatrização e perda de elasticidade do pericárdio resultam em enchimento ventricular prejudicado, disfunção diastólica e insuficiência cardíaca direita. O diagnóstico dessa patologia é desafiador, sendo frequente a necessidade de técnicas de imagem multimodal, dentre as quais a ecocardiografia representa a modalidade de imagem inicial para a avaliação diagnóstica, além de permitir a diferenciação da PC da cardiomiopatia restritiva (CMR) e outras condições que mimetizam constrição. (AU)
Constrictive pericarditis (CP) is a condition in which scarring and loss of elasticity of the pericardium result in impaired ventricular filling, diastolic dysfunction, and right heart failure. The diagnosis of this pathology is challenging, with frequent need for multimodal imaging techniques, among which echocardiography represents the initial imaging modality for the diagnostic evaluation, in addition to allowing the differentiation of CP from restrictive cardiomyopathy (RCM) and other conditions that mimic constriction. (AU)
Assuntos
Humanos , Adolescente , Idoso , Idoso de 80 Anos ou mais , Adulto Jovem , Pericardite Constritiva/fisiopatologia , Pericardite Constritiva/diagnóstico por imagem , Pericárdio/anormalidades , Insuficiência Cardíaca/etiologia , Pericárdio/anatomia & histologia , Tuberculose/complicações , Cardiomiopatia Restritiva/diagnóstico , Ecocardiografia/métodos , Espectroscopia de Ressonância Magnética/métodos , Tomografia Computadorizada por Raios X/métodosRESUMO
Background: Peritoneopericardial diaphragmatic hernia is a rare pathogenesis of congenital origin, which occurs due to a failure in the communication between the diaphragm and the pericardium during embryogenesis. Symptoms may be non-existent or non-specific, depending on the herniated organ involved and, in most cases, the diagnosis is incidental. Regarding the most indicated treatment, there are still divergences in the literature concerning the indication of conservative or surgical treatment. This study reports the case of a feline peritoneopericardial hernia, for which surgical correction was the treatment of choice. Case: A 3-month-old female kitten, no defined racial pattern, was referred to the Veterinary Hospital of the Federal Rural University of Pernambuco for treatment of a peritoneopericardial diaphragmatic hernia. This condition was diagnosed through radiographic examination after the patient having been submitted to pediatric castration and presented anesthetic complications in the transsurgery. Blood count, biochemical profile and Doppler echocardiogram were performed, which showed no significant changes. To obtain a better study and surgical planning, computed tomography was performed to observe the heart located cranially in the pericardial cavity. Caudally to the heart, hepatic parenchyma located in the pericardial cavity was observed; and hepatic vessels presenting slightly enlarged dimensions. These tomographic findings suggested peritoneopericardial diaphragmatic hernia; being the liver present in the pericardial cavity and signs of congestion in the hepatic parenchyma. Due to the likelihood of future worsening of the hernia, surgical correction was performed, with an abdominal midline incision in the preumbilical region to reposition the liver to its normal anatomy, followed by diaphragm reconstitution through a herniorrhaphy. After the surgical procedure, the patient was referred for observation in internment and, after 15 days, the skin sutures were removed. Complete correction of the hernial defect was observed on radiography performed 30 days after the surgical procedure. However, the examination showed the presence of deviation/ deformity in the topography of the sternum and costal cartilages, with slight cardiac displacement to the right hemithorax, suggesting the presence of pectus excavatum. Discussion: Peritoneopericardial diaphragmatic hernia is considered rare and, despite being one of the most common causes of congenital pericardial anomaly in felines, it has a low prevalence ranging from 0.06% to 1.45%. They are usually diagnosed from two years of age, with prevalence for older animals. However, due to having presented anesthetic changes, the patient of this case report could be diagnosed early. Among the most common organs that migrate to the thoracic cavity, the liver is the most commonly observed, which is also the hernia content of the present report. Peritoneopericardial hernia is often diagnosed through radiography and ultrasound, and these imaging tests proved to be sufficient for the diagnosis in this report. However, computed tomography was important for providing a better study of case and for the adoption of median celiotomy as a treatment. Associations with other malformations are described in the literature, with pectus excavatum being the most common and also observed in this report. Peritoneopericardial diaphragmatic hernia is a rare anomaly, rarely reported in the literature and with divergences regarding its treatment. The adoption of early surgical treatment performed in this report showed satisfactory evolution and the possibility of a favorable prognosis.
Assuntos
Animais , Feminino , Gatos , Pericárdio/anormalidades , Peritônio/cirurgia , Hérnias Diafragmáticas Congênitas/veterinária , Radiografia/veterináriaRESUMO
Abstract Pericardial agenesis is a rare congenital anomaly found predominantly in men, and its complete form is extremely rare and difficult to diagnose. This report describes the case of a pregnant patient with complete pericardial agenesis in which mode of delivery and sterilization raised debate among specialists.
Assuntos
Humanos , Feminino , Gravidez , Adulto , Pericárdio/anormalidades , Parto Obstétrico , Pericárdio/diagnóstico por imagem , Esterilização Reprodutiva , EcocardiografiaRESUMO
Pericardial defects are considered rare in animals and humans. Pericardial agenesis is characterized by partial or total pericardial sac malformation. Here is reported a case of apical partial pericardial agenesis in an adult mixed-breed male dog referred for necropsy. The pericardial sac was absent from the apical region up to the middle area of the ventricles resulting in a bilateral ventricular compression by the remaining pericardium changed the organ conformation. Microscopically, there was evidence of neutrophilic and hemorrhagic myocarditis with cardiomyocyte atrophy and interstitial fibrosis. Agenesis is often a subclinical disorder, and it is an incidental postmortem finding in most cases; however, it can cause complications in cases of cardiac structure herniation.(AU)
Assuntos
Animais , Cães , Pericárdio/anormalidades , Fibrose , Cardiopatias Congênitas/diagnóstico , Miocardite/diagnósticoRESUMO
Resumen Paciente masculino de 18 años, quien es valorado en el Servicio de Cardiología del Hospital Víctor Manuel Sanabria Martínez referido del primer nivel de atención por cuadro crónico de dolor torácico atípico acompañado de pectus excavatum y desplazamiento izquierdo del latido de punta a la exploración física. La radiografía de tórax posteroanterior evidencia una cardiomegalia moderada con levocardia máxima. El ecocardiograma transtorácico con función sistólica biventricular conservada, dilatación moderada del ventrículo derecho, sin signos de hipertensión pulmonar e imagen cardiaca de "corazón en lágrima". Angiotomografía computarizada de tórax con contraste que documenta malformación de la caja torácica con hundimiento de la región esternal, en relación con pectus excavatum. Corazón aumentado de tamaño en relación con cardiomegalia grado I y desplazamiento hacia el hemitórax izquierdo. No se observa pericardio en ninguna región. Aurícula derecha levemente dilatada con un ventrículo derecho con diámetro aumentado en su porción media y deformidad de su tracto de salida debido a la forma de la caja torácica, de tal manera que se corrobora el diagnóstico de una agenesia pericárdica completa.
Abstract An 18-year-old male patient who is evaluated in the Cardiology Department of the Víctor Manuel Sanabria Martínez Hospital, referred to the first level of attention due to chronic symptoms of atypical chest pain accompanied by pectus excavatum and left displacement of the peak beat. Chest X-ray Posteroanterior with moderate cardiomegaly with maximum levocardia. Transthoracic echocardiogram with preserved biventricular systolic function, moderate dilatation of the right ventricle, without signs of pulmonary hypertension and cardiac image of "tear heart". Thoracic Computed Angiotomography with contrast documenting malformation of the rib cage with sinking of the external region, in relation to pectus excavatum. Heart enlarged in relation to grade I cardiomegaly and displacement towards the left hemithorax. No pericardium is observed in any region. Slightly dilated right atrium with a right ventricle with an increased diameter in its middle portion and deformity of its outflow tract due to the shape of the rib cage. Corroborating the diagnosis of complete pericardial agenesis.
Assuntos
Humanos , Masculino , Adolescente , Pericárdio/anormalidades , Cardiomegalia/diagnóstico por imagem , Levocardia/diagnóstico por imagem , Pericárdio/diagnóstico por imagemRESUMO
Resumen: La agenesia pericárdica congénita es una condición infrecuente, que generalmente cursa de forma asintomática y es diagnosticada como hallazgo radiológico. Sin embargo, se describe la asociación con malformaciones cardiacas y herniaciones del miocardio con el subsiguiente riesgo de muerte súbita. La radiografía de tórax es un pilar fundamental para la sospecha diagnóstica. Presentamos un caso clínico de una paciente de 11 años que consulta por dolor torácico y disnea, que frente a los hallazgos típicos en radiografía de tórax se sospecha agenesia pericárdica, posteriormente confirmada con TC y RM.
Abstract: Congenital pericardial agenesis is an infrequent but usually asymptomatic condition, and is diagnosed as an incidental radiological finding. However, the association with cardiac malformations and myocardial herniation with the subsequent risk of sudden death has been reported. Chest plain films are a fundamental tool to raise the diagnostic suspicion. We present a clinical case of an 11-year-old patient who consulted for chest pain and dyspnea, in which, with the typical findings on chest radiography, pericardial agenesis was suspected and later confirmed by CT and MRI.
Assuntos
Humanos , Feminino , Criança , Pericárdio/anormalidades , Pericárdio/diagnóstico por imagem , Cardiopatias Congênitas , Imageamento por Ressonância Magnética , Radiografia Torácica , Tomografia Computadorizada por Raios XRESUMO
Abstract The complete or the partial absence of pericardium is a rare congenital malformation for which the patients are commonly asymptomatic and the diagnosis is incidental. The absence of the left side of the pericardium is the most common anomaly that is reported in the literature while the complete absence of pericardium or the absence of the right side of the pericardium are uncommon and their criteria are still unrecognized given their rare occurrence in clinical practice. This paper aims to report a case of 19-year-old male with the congenital partial absence of both sides of the pericardium and to highlight the symptoms and the different cardiac imaging modalities used to confirm the diagnosis of this defect.
Assuntos
Humanos , Masculino , Adulto Jovem , Pericárdio/anormalidades , Pericárdio/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico por imagem , Aorta/diagnóstico por imagem , Artéria Pulmonar/diagnóstico por imagem , Radiografia Torácica , Tomografia Computadorizada por Raios X , Achados Incidentais , Doenças AssintomáticasRESUMO
The complete or the partial absence of pericardium is a rare congenital malformation for which the patients are commonly asymptomatic and the diagnosis is incidental. The absence of the left side of the pericardium is the most common anomaly that is reported in the literature while the complete absence of pericardium or the absence of the right side of the pericardium are uncommon and their criteria are still unrecognized given their rare occurrence in clinical practice. This paper aims to report a case of 19-year-old male with the congenital partial absence of both sides of the pericardium and to highlight the symptoms and the different cardiac imaging modalities used to confirm the diagnosis of this defect.