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)

Histopatología y Strain auricular en pericarditis constrictiva y miocardiopatía restrictiva / Histopathology and Atrial Strain in Constrictive Pericarditis and restrictive cardiomyopathy

Resumen La pericarditis constrictiva y la miocardiopatía restrictiva son enfermedades raras caracterizadas por síntomas de insuficiencia cardíaca congestiva. El objetivo de este estudio es mostrar el diagnóstico diferencial de estas dos patologías, mediante strain auricular y establecer su correlación con la histopatología de corazones correspondientes. Se analizan 2 casos clínicos representativos de cada una de estas patologías. Se analizan sus presentaciones clínicas, los datos ecocardiograficos y en ambos casos se realizaron biopsias endomiocardicas con lo cual se presenta la correlación histológica. El strain auricular permitió evaluar la afección de las aurículas, consideramos que el strain auricular disminuido podría estar relacionado con la presencia de fibrosis.

Abstract Histopathology and Atrial Strain in Constrictive Pericarditis and restrictive cardiomyopathy Constrictive pericarditis and restrictive cardiomyopathy are rare diseases characterized by congestive heart failure symptoms. The aim of this study is to show the differential diagnosis of these two pathologies using strain and to establish their correlation with histopathology of the corresponding hearts. Two representative clinical cases of each of these pathologies are analyzed. Their clinical presentations, echocardiographic data, and endomyocardial biopsies were performed in both cases, thus presenting the histological correlation. Atrial strain allowed us to evaluate the affection of the atria, we consider that decreased atrial strain could be related to the presence of fibrosis.

Amiloidose cardíaca isolada: Relato de caso / Isolated cardiac amyloidosis: Case report

Amiloidose cardíaca é causada por um depósito amiloide entre as fibras cardíacas que pode levar a distúrbio de condução, cardiomiopatia restritiva, baixo débito e comprometimento atrial isolado. Relata-se caso de paciente feminina, 66 anos que há três anos iniciou com perda de peso significativa (40,4 kg)evoluindo com edema de membros inferiores. Na investigação observou-se aumento cardíaco global, e o ecocardiograma evidenciou cardiopatia restritiva infiltrativa e derrame pericárdico. Pela alta suspeição clínica, solicitou-se ressonância magnética cardíaca que foi altamente sugestiva de amiloidose cardíaca.O diagnóstico definitivo se confirma pela biopsia endomiocárdica, porém a ressonância cardíaca é descrita como bom método diagnóstico com raros resultados falso-positivos.

Cardiac amyloidosis is caused by amyloid deposits in cardiac fibers that can lead to conduction disturbance, restrictive cardiomyopathy, low output and isolated atrial involvement. This case report described a female patient, 66 years old, with significant weight loss (40.4kg) that began three year sago, progressing to lower limb edema. On investigation, overall cardiac enlargement was noted,with the echocardiogram showing infiltrative restrictive cardiomyopathy and pericardial effusion. High clinical suspicions prompted a request for a cardiac MRI that strongly suggested cardiacamyloidosis. Although definitive diagnoses require endomyocardial biopsies, cardiac MRI is described as a good diagnostic method with rare false-positive results.

Rotação ventricular esquerda anormal em mulher com talassemia / Abnormal left ventricular torsion in woman with thalassemia

Introdução: Cardiomiopatia induzida pelo ferro é bem documentada em pacientes com talassemia. A ecocardiografia convencional associada a novas tecnologias pode detectar, precocemente, alterações na função ventricular esquerda nesses pacientes. Relato do caso: Mulher, 50 anos, assintomática, com diagnóstico de talassemia, mostra parâmetros ecocardiográficos convencionais e Doppler tecidual normais com alteração na torção e rotação ao speckle tracking. Comentários:A detecção precoce de alterações da função cardíaca por meio de novas tecnologias, em pacientes com talassemia, tem demonstrado importância prognóstica.

Introduction: Iron induced cardiomyophathy is well documented in patients with thalassemia. Conventional echocardiogram associated with new technologies has provided parameters for early detection of changes in left ventricular function. Case report: Woman, 50 years old, asymptomatic, diagnosed with thalassemia, shows normal conventional echocardiogram and tissue Doppler parameters but altered torsion and rotation parameters using speckle tracking. Comments: Early echocardiographic findings using speckle tracking in patients with thalassemia is important and may improve prognosis in these patients.

Discordancia entre el voltaje del electrocardiograma y el grosor parietal del ventículo izquierdo. clave diagnóstica en amiloidosis cardíaca / [Inconsistency between voltage of the electrocardiogram and the left ventricular wall thickness. diagnostic key in cardiac amyloidosis].

Restrictive cardiomyopathy is the least common form of cardiomyopathy, and the disease that most often cause it, is the system amyloidosis. We present a 62-year-old with a history of heart failure, which in its assessment highlights the discrepancy between the low voltage ventricular complexes in the electrocardiogram and the severity of left ventricular wall thickness on echocardiography. This discrepancy was the source of suspicion and subsequent confirmation of systemic amyloidosis with cardiac involvement.

Amiloidose cardíaca: relato de caso / Cardiac amyloidosis: case report

Introdução: Amiloidose cardíaca é uma doença rara. Pode ser consequência de qualquer amiloidose sistêmica, principalmente, quando essa for do tipo primária. Relato do caso: Mulher, 64 anos, diabética, hipertensa, internada, há um mês, com anasarca, distensão abdominal, hepatoesplenomegalia, emagrecimento de 30 quilos em dois anos. O ecocardiograma transtorácico demonstrou: hipertrofia concêntrica importante de VE, insuficiência mitral moderada, AE com dilatação moderada, hipertensão pulmonar discreta, EP: 5%, FE: 51%. Aspecto granuloso sugerindo infiltração amilóide. Comentários: O achado ecocardiográfico da doença é de miocardiopatia restritiva com tamanho ventricular e função sistólica normais e evidência de enrijecimento diastólico patológico.

Colecistite acalculosa complicada em pacientews com endomiocardiofibrose / Acalculous cholecystitis in a patient complicated by endomyocardial fibrosis

Relata-se o caso de paciente jovem, do sexo feminino, com quadro clínico de colecistite acalculosa e concomitante endomiocardiofibrose biventricular até então não diagnosticada. A relação entre as duas doenças é discutida, questionando a possibilidade da eosinofilia e da insuficiência cardíaca como fatores etiopatogênicos ou agravantes a ambas as entidades. A paciente evoluiu com morte de causa indeterminada.

Diagnóstico diferencial entre cardiomiopatia restritiva e pericardite constrictiva: [revisão] / Differencial diagnosis between restrictive cardiomyopathy and constrictive pericarditis: [review]

O diagnóstico diferencial entre as cardiomiopatias restritivas (CR) e a pericardite constrictiva (PC) é fundamental para o manuseio adequado de pacientes com síndrome restritiva (SR). A diferenciação entre as doenças deve ser embasada nas características anatomopatológicas e fisiopatológicas peculiares a cada afecção. Entretanto, um número expressivo de pacientes com PC tem o miocárdio comprometido. A introdução de novas técnicas da ecocardiografia (Eco) traz a perspectiva de identificação da PC, nessas condições...

Miocardiopatias restritivas / Restrictive cardiomyopathies

As miocardiopatias restritivas constituem um grupo heterogêneo de doenças que acometem o músculo cardíaco que tem em comum os sinais e sintomas de insuficiência cardíaca congestiva. Disfunção diastólica com função sistólica preservada é, com frequência única anormalidade ecocardiográfia que pode ser observada, embora a disfunção sistólica e espessamento das paredes possam também ser uma parte integral de algumas patologias específicas, particularmente em casos mais avançados, tais como a infiltração amiloide do coração e hemocromatose. De longe, a maioria das cardiopatias restritivas é secundária as desordens sistêmicas tais como amioloidose, hemocromatose, sarcoidose, esclerodermia, doenças de depósios, síndrome hipereosinofilica e endomiocardiofibrose. Bem mais rara é a miocardiopatia restritiva idiopática, diagnóstico realizado na ausência de doença sistêmica, através de biópsia ou necropsia. O diagnóstico da miocardiopatia restritiva é baseado em história clínica, examo físico, eletrocardiograma, radiografia de tórax, ecocardiografia, ressonância nuclear magnética e biópsia emdomiocárdica. Devido à sua grande disponibilidade, o ecocardiograma é provavelmente o exame mais importante para investigar e detectar a disfunção ventricular esquerda, deve ser realizado precocemente e por operador familiarizado com a grande variedade de etiologias. Finalmente o diagnóstico diferencial com pericardite constritiva é necessário.

Restrictive cardiomyopathies constitute a heterogeneous group of heart muscle conditions that all have, in common, the symptoms of heart failure. Diastolic dysfunction with preserved systolic function is often the only echocardiography abnormality that may be noted, although systolic dysfunction may also be a complete part of some specific pathologies, particularly in the most advanced cases such as amyloid infiltration of the heart and hemochromatosis. By far, the majority of restrictive cardiomyopathy are secondary to a systemic disorder such as amyloidosis, sarcoidosis, scleroderma, hemochromatosis, eosinophilic heart disease or endomyocardial fibrosis. The most uncommon diagnosis of idiopathic restrictive cardiomyopathy is supported only by the absence of specific pathology on either endomyocardial biopsies or at post-mortem. Restrictive cardiomyopathy is diagnosed based on clinical data, physical examination, electrocardiogram, chest X-ray, echocardiography, and magnetic resonance imaging and endomyocardil biopsy. With its broad availability, echocardiography is probably the most Important investigation to Identify the left ventricular dysfunction and should be performed early and by groups that are familiar with the broad variety of etiologies. Finally, the deferential diagnosis from constrictive pericarditis may be necessary.

Left ventricular noncompaction: A cardiomyopathy often mistaken.

Left ventricular noncompaction (LVNC) is a rare genetic cardiomyopathy postulated to be a defect in endomyocardial morphogenesis due to the embryonic arrest of the compaction of myocardial fibers. It could be isolated, without other structural heart defects; or associated with congenital heart defects. It is characterized by prominent ventricular myocardial trabeculations and deep intertrabecular recesses. The clinical manifestations, i.e., heart failure, arrhythmias or thromboembolism, overlap with those of other cardiac disorders. It is often misdiagnosed as restrictive or dilated cardiomyopathy. The high mortality and morbidity associated with it and familial occurrence make diagnosis important. Only 3 pediatric cases have been reported from India. We present 2 cases, that of an 11-year-old girl (familial case) with embolism (documented but rare in children) and atrial flutter (not yet reported), with mother having asymptomatic LVNC; and that of a 4-month-old girl. Both presented with heart failure. The 11-year-old child had sudden death, known to occur in LVNC.

Níveis séricos de NT pro-BNP: relação com função sistólica e diastólica nas miocardiopatias e pericardiopatias / Serum NT pro-BNP: relation to systolic and diastolic function in cardiomyopathies and pericardiopathies

FUNDAMENTO: O NT pro-BNP é marcador de disfunção sistólica e diastólica. OBJETIVO: Determinar os níveis de NT pro-BNP em pacientes com cardiopatia chagásica, hipertrófica, restritiva e afecções pericárdicas, e sua relação com medidas ecocardiográficas de disfunção sistólica e diastólica. MÉTODOS: Cento e quarenta e cinco pacientes foram divididos nos respectivos grupos: 1) cardiopatia chagásica (CCh) - 14 pacientes; 2) miocardiopatia hipertrófica (CMH) - 71 pacientes; 3) endomiocardiofibrose (EMF) - 26 pacientes; 4) derrame pericárdico (DP) - 18 pacientes; 5) e pericardite constritiva (PC) - 16 pacientes. Foi constituído um grupo-controle de 40 indivíduos sem doença cardíaca. O grau de acometimento miocárdico e o derrame pericárdico foram avaliados pelo ecocardiograma bidimensional e a restrição pelo Doppler pulsátil do fluxo mitral. O diagnóstico de PC foi confirmado por meio da ressonância magnética. Os níveis de NT pro-BNP foram medidos por imunoensaio com detecção por eletroquimioluminescência. RESULTADOS: O NT pro-BNP esteve aumentado (p < 0,001) na CCh (mediana 513,8 pg/ml), CMH (mediana 848 pg/ml), EMF (mediana 633 pg/ml), PC (mediana 568 pg/ml), DP (mediana 124 pg/ml), quando comparados ao grupo-controle (mediana 28 pg/ml). Não foram observadas diferenças estatisticamente significativas entre PC e EMF (p = 0,14). No grupo hipertrófico, o NT pro-BNP correlacionou-se com tamanho de átrio esquerdo (r = 0,40; p < 0,001) e relação E/Ea (p < 0,01). No grupo restritivo, houve uma tendência de correlação com pico de velocidade de onda E (r = 0,439; p = 0,06). CONCLUSÃO: O NT pro-BNP encontra-se aumentado nas diversas miocardiopatias e afecções pericárdicas, e apresenta relação com o grau de disfunção sistólica e diastólica.

BACKGROUND: NT pro-BNP is a marker of systolic and diastolic dysfunction. OBJECTIVE: To determine NT pro-BNP levels in patients with chagasic, hypertrophic, and restrictive heart diseases, as well as with pericardial diseases, and their relation to echocardiographic measurements of systolic and diastolic dysfunction. METHODS: A total of 145 patients were divided into the following groups: 1) Chagas' heart disease (CHD) - 14 patients; 2) hypertrophic cardiomyopathy (HCM) - 71 patients; 3) endomyocardial fibrosis (EMF) - 26 patients; 4) pericardial effusion (PE) - 18 patients; and 5) constrictive pericarditis (CP) - 16 patients. The control group was comprised of 40 individuals with no heart disease. The degree of myocardial impairment and pericardial effusion were assessed by two-dimensional echocardiography and the degree of restriction by pulsed Doppler transmitral flow. The diagnosis of CP was confirmed through magnetic resonance imaging. NT pro-BNP levels were determined through electrochemiluminescence immunoassay. RESULTS: NT pro-BNP was increased (p < 0.001) in CHD (median = 513.8 pg/ml), HCM (median = 848 pg/ml), EMF (median = 633 pg/ml), CP (median = 568 pg/ml), and PE (median = 124 pg/ml), when compared with the control group (median = 28 pg/ml). No statistically significant differences were found between CP and EMF (p = 0.14). In the hypertrophic group, NT pro-BNP was correlated with left atrial size (r = 0.40; p < 0.001) and with E/Ea ratio (p < 0.01). In the restrictive group, there was a trend of correlation with E-wave peak velocity (r = 0.439; p = 0.06). CONCLUSION: NT pro-BNP is increased in the different cardiomyopathies and pericardial diseases and is correlated with the degree of systolic and diastolic dysfunction.

Desmin-related restrictive cardiomyopathy / Cardiomiopatia restritiva por depósito de desmina

As cardiomiopatias restritivas podem possuir diversas etiologias, entre elas destacam-se as doenças de depósito por diferentes materiais, como por desmina. As desminopatias são patologias incomuns, que evoluem com distúrbios de condução, miopatias periféricas e disfunção ventricular. O presente relato descreve paciente com bloqueio átrio ventricular total como evento inicial, sendo acometido posteriormente por alterações musculares esqueléticas e insuficiência cardíaca. Na investigação foi definido diagnóstico de cardiomiopatia restritiva por acúmulo de desmina.

Restrictive cardiomyopathies may have different etiologies, among which we can point out storage diseases by accumulation of different materials such as desmin. Desminopathies are uncommon diseases that progress with conduction abnormalities, peripheral myopathies, and ventricular dysfunction. The present report describes a patient with complete atrioventricular block as the initial event; he later developed skeletal muscle alterations and heart failure. The investigation led to the diagnosis of restrictive cardiomyopathy due to desmin accumulation.

Bronquitis plástica asociada a miocardiopatía restrictiva / Plastic bronchitis associated to restrictive miocardiopathy

La bronquitis plástica se caracteriza por la formación de moldes bronquiales con ramificaciones que remedan la anatomía del árbol respiratorio. Se asocia con una enfermedad pulmonar subyacente y con cardiopatías congénitas cianóticas, especialmente luego de la cirugía correctiva. Se desconoce el mecanismo de producción de los moldes. Se propuso como posible mecanismo la existencia de anormalidades de los linfáticos pulmonares asociadas a alteraciones del drenaje, lo que se conoce como "goteo linfático endobronquial". Los síntomas incluyen tos productiva, disnea, fiebre y sibilancias. El diagnóstico se basa en el interrogatorio, radiografías y tomografías computadas de función pulmonar y se confirma por la expectoración espontánea de moldes o su visualización mediante fibrobroncoscopia. Las terapias aún son limitadas y deben estar dirigidas al tratamiento de la enfermedad de base, incluidala remoción de los moldes bronquiales. Los pacientes con cardiopatía poseen índices de mortalidad más elevados que los niños con bronquitis plástica asociada a otras patologías. Presentamos a un niño de 4 años, con bronquitis plástica asociada a miocardiopatía restrictiva.

Restrictive cardiomyopathy due to primary plasma cell leukemia.

The causes of restrictive cardiomyopathy are numerous, of which neoplastic infiltration is also known. Plasma cell leukemia is considered as the, most severe form of multiple myeloma, is an extremely rare condition. Among them, primary plasma cell leukemia has got an incidence of one in one million only. We report a case summary of a patient who was admitted with clinical features suggestive of restrictive cardiomyopathy, the underlying disorder was primary plasma cell leukemia. With chemotherapy the restrictive physiology was relieved supporting the diagnosis of plasma cell infiltration in the myocardium. We report this case due to rarity of the disease itself and its rare presentation.

value of myocardial perfusion imaging in differentiating between idiopathic dilated cardiomyopathy from the ischemic form

Differentiating between ischemic cardiomyopathy [ICM] and idiopathic dilated cardiomyopathy [IDCM] is important as coronary revascularization can improve prognosis in the ischemic subgroup. Due to inherent problems of coronary angiography in patients with depressed ejection fraction [EF] introducing a noninvasive tool to diagnose those who will benefit from angiography seems to be rewarding. We examined usefulness of myocardial perfusion scan in this group of patients. Study was performed on 64 patients [62 male and 2 female] aged 57.1 +/- 6.7y [mean +/- SD] all with dilation of the left ventricular [LV] cavity and ejection fraction less than 40% by echocardiography. Myocardial perfusion scan was performed in stress and rest phases. All the patients had coronary angiography which was used as the gold standard test. On each set of images, heart was arbitrary divided into 17 segments and perfusion abnormality in each segment was scored by a 5 grade scoring system [0-4]. Summed Stress Score was used as the scan criteria to differentiate dilated ischemic from idiopathic cardiomyopathy. Scores more than 17 were considered ischemic, and less than that, idiopathic. Results were compared with angiography. From total 40 cases of ischemic cardiomyopathy [proved by angiography] 39 were correctly diagnosed by scan and only one case was miscategorized as IDCM. All 24 cases of IDCM were correctly diagnosed by scintigraphy. Sensitivity, specificity, positive predictive value, and negative predictive value of myocardial perfusion imaging for discrimination between ischemic and idiopathic dilated cardiomyopathy were 97.5%, 100%, 100%, and 96% respectively. Considering excellent accuracy of myocardial perfusion scan with scoring system in discrimination of ischemic dilated cardiomyopathy from idiopathic dilated cardiomyopathy, this noninvasive test could be considered the main diagnostic test