Como eu faço avaliação ecocardiográfica de pericardite constritiva / My approach to echocardiographic assessment for constrictive pericarditis

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)

Complete Pericardial Agenesis in a Pregnant Patient: A Clinical Dilemma

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.

Agenesia Pericárdica Completa. Presentación de una caso y revisión de la literatura / Complete pericardial agenesis. Presentation of a case and review of the literature

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.

Caso clínico: agenesia pericárdica, diagnóstico imagenológico de una patología infrecuente / Clinical case: pericardial agenesis, radiological diagnosis of a rare disease

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.

Partial congenital absence of the pericardium: a case report

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.

Defecto de la tabicación atrioventricular con 2 válvulas asociado a ausencia del pericardio: Informe de un caso / Atrioventricular defect with 2 valves associated with absence of the pericardium: Report of a case

Presentamos el caso de una paciente con una malformación cardiaca que representa una forma de transición anatomoembriológica del defecto de la tabicación atrioventricular entre la forma de 2 válvulas y la que tiene una válvula común. Esta entidad además se asoció con ausencia de pericardio. A través de los diferentes estudios se ha establecido con precisión la secuencia diagnóstica, determinando cuál fue la aportación de cada método y aclarando además la nomenclatura del defecto de la tabicación atrioventricular.

We present a case of a patient with a cardiac malformation that represents a form of embryo-anatomical transition of an atrioventricular septal defect between a 2 valves form to a common valve form. This entity was associated with pericardium absence. Throughout several studies we have precisely established a diagnostic sequence by determining the adequate contribution of each method and we have been able to clear out the proper nomenclature of the atrioventricular cushion defect.

Ausência parcial congênita do pericárdio / Partial congenital absence of pericardium

A ausência congênita do pericárdio é uma anomalia congênita rara, de difícil diagnóstico. Relatamos um caso de paciente de 42 anos, com dor torácica atípica, eletrocardiograma com distúrbio de condução no ramo direito, radiografia de tórax com desvio do eixo cardíaco no sentido anti-horário e ecocardiograma sugerindo sobrecarga de volume do ventrículo direito. Cateterismo cardíaco demonstrou ausência de shunts intracardíacos, e rotação e deslocamento do coração para o hemitórax esquerdo. Ressonância nuclear magnética confirmou o diagnóstico ao demonstrar ausência parcial do pericárdio à esquerda, com interposição do parênquima pulmonar entre o tronco da artéria pulmonar e a aorta.

Agenesis of the pericardium is a rare congenital anomaly, which is difficult to diagnose. We report a case of a 42-year-old patient, with atypical chest pain, electrocardiogram with an incomplete right bundle branch block, chest X-ray with a counterclockwise heart axis deviation and echocardiogram suggesting right ventricle volume overload. Cardiac catheterization showed no intracardiac shunts, and rotation and displacement of the heart into the left hemithorax. Magnetic nuclear resonance imaging confirmed the diagnosis by revealing partial absence of the left pericardium, with interposition of lung parenchyma between the main pulmonary artery and the aorta.

Gordura epicárdica medida ao ecocardiograma correlaciona-se com ateromatose carotídea / Epicardial fat measured by echocardiography correlates to carotid atheromatosis

Fundamento: A presença de placas carotídeas é um marcador de doença cardiovascular já estabelecido. A medida da gordura epicárdica é um novo método de avaliação da gordura visceral. A relação entre o diâmetro da gordura epicárdica com o risco cardiovascular está sendo investigada, Objetivo: Avaliar a correlação entre a espessura da gordura epicárdica, medida pelo ecocardiograma, e ateromatose das artérias carótidas, Métodos: Foram avaliados 311 pacientes consecutivos, encaminhados para a realização de ecocardiograma ou ultrassonografia carotídea, com indicações clínicas comuns. A espessura da gordura epicárdica, na parede livre do ventrículo direito, foi avaliada com o ecocardiograma e o seu diâmetro, correlacionado com a presença ou não de ateromatose das artérias carótidas. Resultados: Os pacientes (45 por cento homens) tinham idade média de 56 +- 17 anos. A espessura média de gordura epicárdica foi de 5,4 +- 2,6mm. A espessura da gordura epicárdica foi, significativamente, maior nos pacientes com ateromatose das artérias carótidas (7,0 +- 2,2, n=141 vs. 4,7 +-2,5,n=166,p<0,001). A espessura da gordura epicárdica foi maior nos pacientes com hipertensão, diabetes mellitus, doença coronariana ou dislipidemia. Após regressão múltipla...

Partial Pericardial Defect Incidentally Discovered During Coronary Bypass Surgery

A 71-yr-old male patient with three vessel coronary artery disease underwent a coronary artery bypass graft. The patient was found to have a large pericardial defect at the apex of the heart that measured approximately 18 cm in circumference. The edge of the pericardial defect impinged on the epicardial coronary arteries. The left phrenic nerve descended via the dorsal boundary of the pericardial defect. Following coronary artery bypass grafting, the pericardial defect was repaired with a polytetrafluorethylene patch. The patient had an uncomplicated postoperative course.

Doppler echocardiographic signs to differentiate between chronic lung disease and right ventricular dysfunction in a child with calcified pericardium

A 7 year-old girl was admitted with history of low-grade fever, weight loss and occasional cough for the last 6 months. Tuberculosis was suspected, for which antituberculous treatment was initiated. Chest X-ray demonstrated calcification of the pericardium with chest miliary shadowing. CT chest was done showing very fine sharply defined miliary nodularity throughout both lungs without mediastinal lymphadenopathy. The patient was referred to paediatric cardiology service to rule out constrictive pericarditis. Clinical cardiac examination as well as the electrocardiogram was normal. Echocardiography and Doppler were done showing normal cardiac anatomy and function apart from the detected pericardial calcification. Doppler tracing in the hepatic vein as well as in the superior vena cava could clearly exclude any negative effect on diastolic cardiac function, which could occur in the setup of pericardial calcification. Doppler, instead helped to identify the chronic pulmonary disease by showing increased inflow velocity throughout inspiration

Ausência congênita do pericárdio: documentação ecocardiográfica, tomográfica e por ressonância magnética nuclear / Congenital absence of the pericardium: documentation with echocardiography, computed tomography and magnetic resonance imaging

O presente caso relata uma anomalia congênita (1:14.000 necropsias) e de difícil diagnóstico, visto que os portadores dessa anomalia, geralmente são assintomaticos: a agenesia do pericárdio. O diagnóstico baseado no tripé: radiografia de tórax, ecodopplercardiograma e tomografia computadorizada ou ressonância magnética de tórax deve ser sempre lembrando, uma vez que a patologia oferece risco de evolução para evento cardíaco agudo potencialmente letal, quando de sua apresentação parcial.

Fibrilación auricular en una paciente con megaorejuela auricular izquierda / Atrial fibrillation in a patient with a giant left atrial appendage

La FA constituye una de las arritmias sostenidas más frecuentes que motivan la consulta. El sustrato comprende diferentes mecanismos, entre los que se encuentra el agrandamiento auricular izquierdo. Presentamos el caso de una mujer de 33 años con antecedentes de palpitaciones, que ingresó en la guardia por FA de alta respuesta ventricular. En la radiografía de tórax se observó una deformación del borde izquierdo de la silueta cardíaca. Posteriormente, el ecocardiograma transesofágico y la resonancia magnética nuclear evidenciaron una megaorejuela aneurismática debida, probablemente, a un defecto pericárdico. La conducta fue anticoagulación oral y tratamiento con atenolol, con evolución favorable.

Among sustained arrhythmias, atrial fibrillation is one of the most common causes of patient consultation. Its substrate comprises various mechanisms, including left atrial enlargement. In this report, we present the case of a 33 yearold woman with a history of palpitations, who was admitted to the emergency room due to atrial fibrillation with a rapid ventricular response. In the chest X-ray, the left border of the cardiac silhouette was deformed. Subsequently, both the transesophageal echocardiogram and the magnetic resonance images showed a giant aneurysmal left atrial appendage, probably due to a pericardial defect. Treatment consisted of oral anticoagulation and atenolol, with a favorable patient outcome.

Pentalogía de Cantrell. A propósito de un caso / Pentalogy of Cantrell. A case report

Ectopia cordis is an extremely rare cardiac anomaly. The heart is localized partially or totally outside the thorax cavity. This anomaly occurs as an isolated defect or combined with others midline defects. Cantrell and colleagues described, in 1958, a syndrome including defects of the abdominal wall, sternum, diaphragm, pericardium and heart. There are few successful surgical cases with this pentalogy. We describe a case with this Cantrell's pentalogy. The cardiac malformation was a univentricular heart with pulmonary stenosis. The patient underwent successful surgical palliation with a systemic-to-pulmonary anastomosis and uneventful recovering.

Surgical reconstruction for absence of sternum and pericardium in a newborn.

We report the surgical management of a 3 day old baby with absent sternum and pericardium with impending rupture of the skin cover. The repair described is innovative and may be used in place of primary repair by bringing the ribs together. Review of embryology and various modalities of closure are provided.

Doença neoplásica do pericárdio / Neoplasic disease of the pericardium

O envolvimento neoplásico de coração é raro, sendo predominantemente metastático para o pericárdio. Pode acontecer na evolução de um tumor já conhecido ou ser sua primeira manifestação. Aproximadamente metade dos pacientes tem invasão pelo tumor, sendo o restante decorrente de outras causas. As neoplasias que mais freqüentemente infiltram o pericárdio são as de pulmão, as de mama e as de linhagem hematológica. A videopericardioscopia é o método atual de escolha para o diagnóstico etiológico, por possibilitar biópsia dirigida, além de poder ser usada para procedimentos terapêuticos.

Agenesia de pericardio izquierdo: A propósito de 1 caso / Impotence of left pericardium. On purpose of 1 case

Se presenta el caso de un paciente que, diagnosticado clínica y radiológicamente como un cáncer broncopulmonar, se interviene quirúrgicamente y se encuentra sólo una rara anomalía del desarrollo: agenesia de pericardio izquierdo. Se realiza revisión de la literatura médica sobre esta afección(AU)

The case of a patient with a clinical and radiological diagnosis of bronchopulmonary cancer that was operated on is presented. Only a rare abnormality of development was found: agenesia of the left pericardium. A review of the medical literature on this affection is made(AU)