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Background: The Tpe interval (Tp-e) in the surface electrocardiogram represents ventricular repolarization, a key phase in the pathogenesis of severe ventricular arrhythmias. However, there are few studies evaluating changes in this electrocardiographic interval as a risk factor for serious arrhythmias in patients with dilated cardiomyopathy. Objective: To determine whether the Tp-e interval prolongation predicts the presence of life-threatening arrhythmias in patients with dilated cardiomyopathy (DCM) with implantable cardioverter-defibrillator (ICD). Material and methods: Analytical, cross-sectional study in patients with DCM with ICDs. The Tp-e interval was measured in the V2 electrocardiographic lead and correlated with the incidence of life-threatening arrhythmias identified by the ICD. Results: 53 patients were recruited, 10 (18.8%) presented life-threatening arrhythmias. Prolongation of Tp-e interval was related to an increase in the incidence of ventricular tachycardia/fibrillation tachycardia (VT/FT) with a mean of 93 ± 20.5 ms (p = 0.003), using ROC curves to determine the thereshold of 90 ms for increased risk of VT/VF with sensitivity of 70% and specificity of 84%, with an area under the curve of 0.798. Conclusion: The prolongation of the ITp-e interval greater than 90 ms in a population with dilated cardiomyopathy predicts the presence of arrhythmic episodes, such as VT and/or VT/FT.

Introducción: el intervalo Tpe (ITp-e) en el electrocardiograma de superficie representa la repolarización ventricular, fase clave en la patogénesis de arritmias ventriculares graves. Sin embargo, existen pocos estudios que evalúen la alteración de este intervalo electrocardiográfico como factor de riesgo de arritmias graves en pacientes con miocardiopatía dilatada. Objetivo: determinar si la prolongación del ITp-e predice la presencia de arritmias potencialmente mortales en pacientes con miocardiopatía dilatada (MCD) portadores de desfibrilador automático implantable (DAI). Material y métodos: estudio, transversal analítico en pacientes con MCD portadores de DAI. Se midió el ITp-e en la derivación electrocardiográfica V2 y se correlacionó con la incidencia de arritmias potencialmente letales identificadas por el DAI. Resultados: se incluyeron 53 pacientes, 10 (18.8%) presentaron arritmias potencialmente mortales. La prolongación del ITp-e se relacionó con aumento de incidencia de taquicardia ventricular/fibrilación ventricular (TV/FV) con media de 93 ± 20.5 ms (p = 0.003), por lo que se determinó mediante curvas ROC el punto de corte de 90 ms para el aumento de riesgo de TV/FV con sensibilidad de 70% y especificidad de 84%, con área bajo la curva de 0.798. Conclusión: la prolongación del intervalo ITp-e > 90 ms en población con miocardiopatía dilatada predice la presencia de episodios arrítmicos como TV o FV.

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SUMMARY OBJECTIVE: Removal of cardiac autoantibodies by immunoadsorption might confer clinical improvement in dilated cardiomyopathy. In this pilot study, we investigated the efficacy and safety of immunoadsorption therapy in refractory heart failure patients with dilated cardiomyopathy. METHODS: This study consisted of 9 heart failure patients with dilated cardiomyopathy, NYHA III-IV, left ventricular ejection fraction <30%, unresponsive to heart failure therapy, and with cardiac autoantibodies. Patients underwent immunoadsorption therapy for five consecutive days using a tryptophan column. Changes in cardiac function (left ventricular ejection fraction, left ventricular end-diastolic diameter, left ventricular end-systolic diameter), exercise capacity (6-minute walk distance), neurohormonal (N-terminal pro-brain natriuretic peptide), proinflammatory (high-sensitive C-reactive protein), and myocardial (cardiac troponin-I), biochemical, and hematological variables were obtained at baseline and after 3 and 6 months of immunoadsorption therapy. RESULTS: Mean left ventricular ejection fraction and 6-minute walk distance significantly increased at 3 months (from 23.27±5.09 to 32.1±1.7%, p=0.01 for left ventricular ejection fraction and from 353±118 to 434±159 m, p=0.04 for 6-minute walk distance) and further increased at 6 months after immunoadsorption therapy (to 34.5±7.7%, p=0.02 for ejection fraction and to 441±136 m, p=0.04 for 6-minute walk distance). NT-proBNP level reduced from 1161(392.8-3034) to 385(116.1-656.5) ng/L (p=0.04), and high-sensitive C-reactive protein decreased from 9.74±0.96 to 4.3±5.8 mg/L (p=0.04) at 6 months. Left ventricular end-diastolic diameter (66.1±5.8 vs. 64.7±8.9 mm) and left ventricular end-systolic diameter (56.1±8.6 vs. 52.3±10.8 mm) tended to decrease but did not reach statistical significance. No significant worsening was observed in creatinine, cardiac troponin-I, and hemoglobin levels after the immunoadsorption procedure. CONCLUSION: In dilated cardiomyopathy patients with refractory heart failure, immunoadsorption may be considered a potentially useful therapeutic option to improve a patient's clinical status.

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Resumo Os testes genéticos para cardiomiopatia dilatada (CMD) apresentam uma positividade de até 40%, mas há uma grande heterogeneidade genética e outros desafios decorrentes de expressividade variável e penetrância incompleta. O heredograma é fundamental para diferenciar os casos de CMD esporádica e familiar, por meio da avaliação do histórico familiar. A CMD familiar apresenta um rendimento maior nos testes genéticos, mas a CMD esporádica não exclui a possibilidade de causa genética. Alguns genes têm fenótipos específicos, sendo o gene da Lamina ( LMNA ) o mais fortemente associado a um fenótipo de arritmias malignas e quadros de insuficiência cardíaca (IC) avançada. A presença de uma variante genética causal também pode ajudar na avaliação prognóstica, identificando quadros mais graves e com menores taxas de remodelamento reverso em comparação com indivíduos com genótipo negativo. As diretrizes atuais recomendam a avaliação e aconselhamento genético em indivíduos com CMD, além do rastreamento em cascata nos familiares de primeiro grau nos casos em que há uma ou mais variantes identificadas, sendo uma oportunidade para o diagnóstico e tratamento precoces. Familiares com genótipo positivo e fenótipo negativo são candidatos à avaliação seriada, com periodicidade que varia conforme a idade. O genótipo também auxilia na indicação individualizada de cardiodesfibrilador implantável e em recomendações quanto à atividade física e planejamento familiar. Estudos em curso esclarecem progressivamente os detalhes das relações genótipo/fenótipo de um grande número de variantes e fazem com que a genética molecular esteja cada vez mais presente na prática clínica.

Abstract Genetic tests for dilated cardiomyopathy (DCM) have a diagnostic yield of up to 40%, but there is significant genetic heterogeneity and other challenges, such as variable expressivity and incomplete penetrance. Pedigree analysis is essential for distinguishing between sporadic and familial DCM cases by assessing family history. Familial DCM yields higher results in genetic testing, but sporadic DCM does not rule out the possibility of a genetic cause. Some genes have specific phenotypes, with the Lamin gene ( LMNA ) being associated with a phenotype of malignant arrhythmias and advanced heart failure (HF). The presence of a causal genetic variant can also aid in prognostic evaluation, identifying more severe cases with lower rates of reverse remodeling (RR) compared to individuals with a negative genotype. Current guidelines recommend genetic evaluation and counseling for individuals with DCM, along with cascade screening in first-degree relatives in cases where one or more variants are identified, offering an opportunity for early diagnosis and treatment. Relatives with a positive genotype and negative phenotype are candidates for serial evaluation, with frequency varying by age. Genotype also assists in individualized recommendations for implantable cardioverter-defibrillator (ICD) placement and advice regarding physical activity and family planning. Ongoing studies are progressively elucidating the details of genotype/phenotype relationships for a large number of variants, making molecular genetics increasingly integrated into clinical practice.

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RESUMEN Introducción: El T1 mapping es una técnica que permite mejorar la caracterización tisular por resonancia magnética cardíaca (RMC), y posee creciente evidencia a su favor como herramienta de diagnóstico precoz y estratificación. Presentamos los resultados de la cuantificación del T1 nativo miocárdico en individuos sanos, estudiados en un campo de 3.0 T, a fin de proveer valores de referencia para el medio local. Material y métodos: Se incluyeron 124 individuos consecutivos derivados a nuestro centro para realización de RMC, cuyos estudios resultaron normales. Se midió el T1 mapping en un eje corto medioventricular. Se analizaron los resultados según edad y sexo. Se incluyeron también 27 pacientes con diagnóstico de miocardiopatía hipertrófica, 11 con diagnóstico de miocardiopatía dilatada y 8 con amiloidosis cardíaca. Resultados: Se analizaron 124 estudios. La media global de T1 mapping fue de 1220,7 ± 21,2 mseg. Redondeando a valores enteros, se consideró 1178-1263 mseg como "rango de normalidad" (p5-p95). Se observó un tiempo T1 ligeramente superior en mujeres. No hubo diferencias con respecto a la edad. Se observó una excelente reproducibilidad, evaluada por el coeficiente de correlación intraclase (0,97) y el método de Bland-Altman. Los valores de T1 mapping fueron significativamente superiores en los grupos de individuos portadores de miocardiopatía. Conclusiones: Reportamos valores normales de T1 mapping nativo en una población adulta local. Los mismos son levemente mayores en mujeres, diferencia que no impresiona relevante desde el punto de vista clínico. Al comparar con individuos portadores de miocardiopatía hipertrófica, dilatada o con amiloidosis cardíaca, se obtuvo una muy buena discriminación. La variabilidad interobservador fue muy baja.

ABSTRACT Background: T1mapping is a technique that improves tissue characterization by cardiovascular magnetic resonance (CMR), and there is growing evidence favoring its use as a tool for early diagnosis and stratification. We present the results of native myocardial T1 quantification in a 3.0 T field in healthy individuals, in order to provide local reference values. Methods: A total of 124 consecutive adults with normal studies, referred to our center for CMR, were included in the study. T1 relaxation time was measured in a midventricular short axis slice, analyzing age and sex dependance. For comparison, 27 patients with hypertrophic cardiomyopathy, 11 with dilated cardiomyopathy and 8 with cardiac amyloidosis were also included. Results: Mean global T1mapping of the 124 studies analyzed was 1220.7 ±21.2 msec, and rounding to unity, 1178-1263 msec (p5-p95) was considered as "normal range". A slightly longer T1 time was observed in women and no differences were found with respect to age. Excellent reproducibility was obtained, evaluated by intraclass correlation coefficient (0.97) and BlandAltman plot. T1 mapping values were significantly higher in both groups of individuals with cardiomyopathy. Conclusions: We report normal values of native T1 mapping in a local healthy adult population. Times were slightly higher in women, a difference that was not considered clinically relevant. When comparing with individuals with hypertrophic or dilated cardiomyopathy, a very good discrimination was obtained between the 3 populations. The interobserver variability was very low.

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Eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss Syndrome, is a rare pathology that belongs to the group of diseases characterized by necrotizing vasculitis of small and medium-sized systemic blood vessels. Symptomatic cardiovascular involvement occurs in 27% to 47% of Churg-Strauss cases, being one of the most serious manifestations. The diagnosis is usually confirmed with tissue biopsy showing eosinophil infiltration, but in selected cases with the recent inclusion of cardiac MRI, we can dispense with it. Early diagnosis is important because early treatment is usually associated with improvement in the condition.

La granulomatosis eosinofílica con poliangeítis, anteriormente conocida como síndrome de Churg-Strauss, es una patología poco frecuente que pertenece al grupo de enfermedades caracterizadas por vasculitis necrotizante de vasos sanguíneos sistémicos de pequeño y mediano calibre. La afectación cardiovascular sintomática ocurre entre un 27% a un 47% de los casos de Churg-Strauss, siendo una de las manifestaciones más graves. El diagnóstico suele confirmarse con biopsia de tejido con infiltración de eosinófilos, pero con la reciente inclusión de la resonancia cardíaca, podemos prescindir de ella. El diagnóstico precoz es importante debido a que el tratamiento oportuno suele asociarse con mejoría del cuadro.

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BACKGROUND: Functional mitral regurgitation (FMR) is associated with dilated cardiomyopathy (DC), heart failure (HF), and worsening left atrial function (LAF). Patients with DC and FMR may present left atrial dysfunction resulting from both ventricular dysfunction and valve disease, but it is unknown whether the presence of valve disease will lead to greater LAF impairment. OBJECTIVE: This study aimed to evaluate the relationship between LAF parameters and FMR degree in patients with DC. METHODS: This cross-sectional observational study included 214 patients with DC, 46 without FMR (control group) and 168 with mild, moderate or severe FMR. An LAF analysis was performed by speckle tracking echocardiography (STE) and atrial volumetric variation. RESULTS: LAF analyzed by STE by means of reservoir strain, conduit strain and active contraction strain was reduced in the sample, with values of 14.3%, 8.49% and 5.92%, respectively. FMR degree was significantly associated with reservoir strain (0.27 ± 0.16 versus 0.15 ± 0.09; p < 0.001) and contraction strain (19.2 ± 7.3 versus 11.2 ± 2.7; p < 0.001). FMR was also associated with a reduced LAF assessed by volumetric analysis: total atrial emptying fraction of 0.51 ± 0.13 versus 0.34 ± 0.11 and active atrial emptying fraction of 0 .27 ± 0.16 versus 0.15 ± 0.09 (p < 0.001). CONCLUSION: In a population with DC, FMR was associated with reduced LAF assessed by STE and atrial volume variation.

FUNDAMENTO: A insuficiência mitral funcional (IMF) está associada à miocardiopatia dilatada (MD), à insuficiência cardíaca (IC) e à piora da função atrial esquerda (FAE). A FAE pode decair tanto pela disfunção ventricular quanto pela valvopatia, mas não se sabe se esta leva a um prejuízo maior da FAE. OBJETIVO: Avaliar a relação entre a piora de parâmetros de FAE com o grau de IMF, em pacientes com MD. MÉTODOS: Trata-se de estudo observacional transversal, que incluiu 214 pacientes com MD, sendo 46 sem IMF (controle) e 168 com IMF discreta, moderada ou grave. A análise da FAE foi realizada por ecocardiografia por speckle tracking (STE) e por variação volumétrica atrial. RESULTADOS: A FAE, analisada por STE­ por meio do strain de reservatório, conduto e contração ativa ­ encontrou-se reduzida na amostra, com valores respectivos de 14,3%, 8,49% e 5,92%. O grau de IMF associou-se significativamente com os valores do strain de reservatório (0,27±0,16 versus 0,15±0,09; p <0,001. CONCLUSÃO: Em uma população com MD, a presença de IMF associa-se à redução da FAE de reservatório e de contração, avaliada por STE e pela variação volumétrica atrial.

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Resumo Fundamento: Fibrose cardíaca difusa é fator importante na avaliação prognóstica dos pacientes com disfunção ventricular. Mapeamento T1 nativo pela ressonância magnética cardíaca (RMC) apresenta elevada sensibilidade e é considerado preditor independente de mortalidade por todas as causas e desenvolvimento de insuficiência cardíaca (IC) nos pacientes com cardiomiopatia. Objetivos: Avaliar aplicabilidade da avaliação com mapa T1 nativo em pacientes com IC em um hospital de referência de cardiologia e sua associação com parâmetros estruturais e perfil funcional. Métodos: Estudo transversal com pacientes adultos com IC classes funcionais NYHA I e II, isquêmicos e não isquêmicos, acompanhados em hospital de referência, que realizaram RMC. Os valores de T1 nativo foram analisados em relação a parâmetros estruturais, comorbidades, etiologia e categorização da IC pela fração de ejeção do ventrículo esquerdo (FEVE). Análises foram realizadas com nível de significância de 5%. Resultados: Analisados 134 pacientes. Valores de T1 nativo elevados foram encontrados em pacientes com maior dilatação (1004,9 vs 1042,7ms, p=0,001), volume (1021,3 vs 1050,3ms, p<0,01) e disfunção ventricular (1010,1 vs 1053,4ms, p<0,001), mesmo quando analisados isoladamente os não isquêmicos. Pacientes classificados com IC com fração de ejeção reduzida apresentaram maiores valores T1 em relação aos com IC e fração de ejeção preservada (ICFEP) (992,7 vs 1054,1ms, p<0,001). Dos com ICFEP, 55,2% apresentavam T1 elevado. Conclusões: Mapeamento T1 por RMC é factível para avaliação da IC clínica. Houve associação direta entre maior valor nativo de T1 e menor fração de ejeção, maiores diâmetros e volumes do VE, independentemente da etiologia da IC.

Abstract Background: Diffuse cardiac fibrosis is an important factor in the prognostic assessment of patients with ventricular dysfunction. Cardiovascular magnetic resonance imaging (CMR) native T1 mapping is highly sensitive and considered an independent predictor of all-cause mortality and heart failure (HF) development in patients with cardiomyopathy. Objectives: To evaluate the feasibility of native T1 mapping assessment in patients with HF in a cardiology referral hospital and its association with structural parameters and functional profile. Methods: Cross-sectional study with adult patients with HF NYHA functional classes I and II, ischemic and non-ischemic, followed in a referral hospital, who underwent CMR. Native T1 values were analyzed for structural parameters, comorbidities, etiology, and categorization of HF by left ventricular ejection fraction (LVEF). Analyses were performed with a significance level of 5%. Results: Enrollment of 134 patients. Elevated native T1 values were found in patients with greater dilation (1004.9 vs 1042.7ms, p = 0.001), ventricular volumes (1021.3 vs 1050.3ms, p <0.01) and ventricular dysfunction (1010.1 vs 1053.4ms, p <0.001), also present when the non-ischemic group was analyzed separately. Patients classified as HF with reduced ejection fraction had higher T1 values than those with HF and preserved ejection fraction (HFPEF) (992.7 vs 1054.1ms, p <0.001). Of those with HFPEF, 55.2% had higher T1. Conclusions: CMR T1 mapping is feasible for clinical HF evaluation. There was a direct association between higher native T1 values and lower ejection fraction, and with larger LV diameters and volumes, regardless of the etiology of HF.

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Focal atrial tachycardia is a rare type of supraventricular tachyarrhythmia, generally present in young people, and is a rare cause of tachycardiomyopathy (10%). We present a clinical case of tachycardiomyopathy in a 30-year-old man, without comorbidities, who was diagnosed with incessant focal atrial tachycardia, refractory to medical therapy, and electrical cardioversion. Successful endocardial ablation was performed, and in outpatient follow-up at 6 months, he showed a recovery of the left ventricular ejection fraction and reduction of the left chambers to normal ranges, with progressive decrease of dyspnea.

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Abstract A 72-year-old woman was admitted for acute heart failure. The echocardiography revealed moderate depression of the left ventricular ejection fraction. Coronary disease was excluded by coronarography. Cardiac magnetic resonance showed predominantly left ventricular septal hypertrophy and severe depression of the left ventricular systolic function. There was also a bright, multifocal and patchy late gadolinium enhancement with subendocardial, mesocardial and subepicardial involvement, suggestive of sarcoidosis. Biochemical study, thoracic computed tomography and positron emission tomography were inconclusive for extra-cardiac sarcoidosis. Therefore, an endomyocardial biopsy was performed. The procedure was complicated by the development of complete atrioventricular block, requiring implantation of a cardiac resynchronization pacing device. A few days after device implantation, the patient developed fever. The echocardiography revealed extensive vegetations, and thus the diagnosis of a device-associated infective endocarditis was made. Even though antibiotic therapy was promptly started, the patient ended up dying. Biopsy results revealed lymphocytic myocarditis. This case is paradigmatic because it shows how the etiologic diagnosis of dilated cardiomyopathy can be challenging. Non-invasive diagnostic exams may not provide a definite diagnosis, requiring an endomyocardial biopsy. However, the benefits versus risks of such procedure must always be carefully weighted.

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Resumo Fundamento A síndrome de takotsubo (takotsubo) é uma forma de cardiomiopatia adquirida. Dados nacionais sobre essa condição são escassos. O Registro REMUTA é o primeiro a incluir dados multicêntricos dessa condição no nosso país. Objetivo Descrever as características clínicas, prognóstico, tratamento intra-hospitalar e mortalidade hospitalar e em 1 ano de seguimento. Métodos Estudo observacional, retrospectivo, tipo registro. Incluídos pacientes internados com diagnóstico de takotsubo ou que desenvolveram esta condição durante internação por outra causa. Os desfechos avaliados incluíram fator desencadeador, análise dos exames, uso de medicações, complicações e óbito intra-hospitalar e em 1 ano de seguimento. O nível de significância adotado foi de 5%. Resultados Foram incluídos 169 pacientes, em 12 centros no Estado do Rio de Janeiro. A idade média foi de 70,9 ± 14,1 anos e 90,5% eram do sexo feminino; 63% dos casos foram de takotsubo primário e 37% secundário. Troponina I foi positiva em 92,5% dos pacientes e a mediana de BNP foi de 395 (176,5; 1725). Supradesnivelamento do segmento ST esteve presente em 28% dos pacientes. A fração de ejeção do ventrículo esquerdo teve mediana de 40 (35; 48)%. Observamos taxa de 25,7% de ventilação mecânica invasiva e 17,4% de choque. Suporte circulatório mecânico foi utilizado em 7,7%. A mortalidade intra-hospitalar foi de 10,6% e a mortalidade ao final de 1 ano foi de 16,5%. Takotsubo secundário e choque cardiogênico foram preditores independentes de mortalidade. Conclusão Os resultados do REMUTA mostram que takotsubo não se trata de patologia benigna como se pensava, especialmente no grupo de takotsubo secundário que acarreta elevada taxa de complicações e de mortalidade. (Arq Bras Cardiol. 2020; 115(2):207-216)

Abstract Background Takotsubo syndrome (TTS) is an acquired form of cardiomyopathy. National Brazilian data on this condition are scarce. The Takotsubo Multicenter Registry (REMUTA) is the first to include multicenter data on this condition in Brazil. Objective To describe the clinical characteristics, prognosis, in-hospital treatment, in-hospital mortality, and mortality during 1 year of follow-up. Methods This is an observational, retrospective registry study including patients admitted to the hospital with diagnosis of TTS and patients admitted for other reasons who developed this condition. Evaluated outcomes included triggering factor, analysis of exams, use of medications, complications, in-hospital mortality, and mortality during 1 year of follow-up. A significance level of 5% was adopted. Results The registry included 169 patients from 12 centers in the state of Rio de Janeiro, Brazil. Mean age was 70.9 ± 14.1 years, and 90.5% of patients were female; 63% of cases were primary TTS, and 37% were secondary. Troponin I was positive in 92.5% of patients, and median BNP was 395 (176.5; 1725). ST-segment elevation was present in 28% of patients. Median left ventricular ejection fraction was 40 (35; 48)%. We observed invasive mechanical ventilation in 25.7% of cases and shock in 17.4%. Mechanical circulatory support was used in 7.7%. In-hospital mortality was 10.6%, and mortality at 1 year of follow-up was 16.5%. Secondary TTS and cardiogenic shock were independent predictors of mortality. Conclusion The results of the REMUTA show that TTS is not a benign pathology, as was once thought, especially regarding the secondary TTS group, which has a high rate of complications and mortality. (Arq Bras Cardiol. 2020; 115(2):207-216)

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Resumo A infecção pelo coronavírus denominada COVID-19 promoveu crescente interesse de cardiologistas, emergencistas, intensivistas e pesquisadores, pelo estudo do acometimento miocárdico partindo de diferentes formas clínicas decorrentes de desmodulação imunoinflamatória e neuro-humoral.O acometimento miocárdico pode ser mínimo e apenas identificado a partir de alterações eletrocardiográficas, principalmente por aumento de troponinas cardíacas, ou no outro lado do espectro pelas formas de miocardite fulminante e síndrome de takotsubo.A descrição de provável miocardite aguda tem sido comumente apoiada pela observação da troponina elevada em associação com disfunção. A clássica definição de miocardite, respaldada pela biópsia endomiocárdica de infiltrado inflamatório é rara, e foi observada em um único relato de caso até o momento, não se identificando o vírus no interior dos cardiomiócitos.Assim, o fenômeno que se tem documentado é de injúria miocárdica aguda, sendo obrigatório afastar doença coronária obstrutiva a partir da elevação de marcadores de necrose miocárdica, associada ou não à disfunção ventricular, provavelmente associada à tempestade de citoquinas e outros fatores que podem sinergicamente promover lesão miocárdica, tais como hiperativação simpática, hipoxemia, hipotensão arterial e fenômenos trombóticos microvasculares.Fenômenos inflamatórios sistêmicos e miocárdicos após infecção viral estão bem documentados, podendo evoluir para remodelamento cardíaco e disfunção miocárdica. Portanto, será importante a cardiovigilância desses indivíduos para monitorar o desenvolvimento do fenótipo de miocardiopatia dilatada.A presente revisão apresenta os principais achados etiofisiopatológicos, descrição da taxonomia desses tipos de acometimento cardíaco e sua correlação com as principais formas clínicas do componente miocárdico presente nos pacientes na fase aguda de COVID-19.

Abstract Infection with the coronavirus known as COVID-19 has promoted growing interest on the part of cardiologists, emergency care specialists, intensive care specialists, and researchers, due to the study of myocardial involvement based on different clinical forms resulting from immunoinflammatory and neurohumoral demodulation.Myocardial involvement may be minimal and identifiable only by electrocardiographic changes, mainly increased cardiac troponins, or, on the other side of the spectrum, by forms of fulminant myocarditis and takotsubo syndrome.The description of probable acute myocarditis has been widely supported by the observation of increased troponin in association with dysfunction. Classical definition of myocarditis, supported by endomyocardial biopsy of inflammatory infiltrate, is rare; it has been observed in only one case report to date, and the virus has not been identified inside cardiomyocytes.Thus, the phenomenon that has been documented is acute myocardial injury, making it necessary to rule our obstructive coronary disease based on increased markers of myocardial necrosis, whether or not they are associated with ventricular dysfunction, likely associated with cytokine storms and other factors that may synergistically promote myocardial injury, such as sympathetic hyperactivation, hypoxemia, arterial hypotension, and microvascular thrombotic phenomena.Systemic inflammatory and myocardial phenomena following viral infection have been well documented, and they may progress to cardiac remodeling and myocardial dysfunction. Cardiac monitoring of these patients is, therefore, important in order to monitor the development of the phenotype of dilated myocardiopathy.This review presents the main etiological and physiopathological findings, a description of the taxonomy of these types of cardiac involvement, and their correlation with the main clinical forms of the myocardial component present in patients in the acute phase of COVID-19.

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INTRODUCTION: Cardiomyopathies are a heterogeneous heart diseases group in terms of morphology and aetiology. Hypothesising a tropical specificity and given an absence of data in French Guiana, the primary objective of our study was to describe morphologies and aetiologies of cardiomyopathies observed at Cayenne General Hospital. METHODS: A cross-sectional study was conducted in Cayenne Hospital from 1 January 2009 to 1 June 2014 in the hospital database. Only patients with the definition of the European Society of Cardiology (ESC) were included, based on the first transthoracic ultrasonography found during the study period. Medical files were consulted. RESULTS: With 182 patients included, the prevalence of cardiomyopathies among heart diseases was estimated at 4.3% (95% CI 3.7% to 4.9%). Twelve patients had a familial or genetic aetiology (6.6%) and 170 a non-familial or non-genetic aetiology (93.4%). The morphological spectrum was: dilated for 114 patients (62.6%), hypertrophic for 27 (14.8%), unclassified for 1 (0.5%) and non-classifiable for 13 (7.1%). This group was constituted of patients with a systolic and/or diastolic functional impairment without morphological abnormality. The aetiological spectrum found 184 aetiologies including: 70.9% undetermined, 8.7% infectious (with 6.5% chagasic and 0.5% related with human immunodeficiency virus) and 6.0% with toxins. CONCLUSIONS: Cardiomyopthies are a common and severe clinical problem. The frequency of infectious aetiologies and dilated impairment are arguments for cardiomyopathies with tropical particularities. However, the preponderance of undetermined aetiologies justifies the development of further research programmes.

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INTRODUCTION: Bidirectional ventricular tachycardia (BVT) is a rare arrhythmia characterized by QRS complexes with an axis in the frontal plane alternating polarity in the precordial leads and right bundle branch block (RBBB) morphology. To the best of our knowledge, there is no previous report in dilated cardiomyopathy or in the context of a probable peripartum cardiomyopathy. CASE PRESENTATION: A 26-year-old, 9-month female patient, with no significant past medical history (the patient denies medication intake, herbs like aconite, trouble during delivery, any heart issues or family history of sudden death or cardiomyopathies) who presents to the emergency room due to 11 days of dyspnea, exacerbated by daily activities, orthopnea, and paroxysmal nocturnal dyspnea. She presented with ventricular bigeminy and systolic dysfunction with left ventricular ejection fraction (LVEF) <20%. The patient later developed a stable ventricular tachycardia (VT) treated with amiodarone, which resulted in hemodynamic instability and BVT rhythm with VT paroxysms. Without the possibility of ablation, the use of high dose beta blockers and an implantable cardioverter defibrillator resulted in the same rhythm with a lower heart rate, better NYHA functional class, and less episodes of VTs. CONCLUSION: BVT is a rare type of tachycardia that can be present in dilated cardiomyopathy. In the absence of ablation capabilities, decreasing the heart rate with beta-blockers may reduce the rate of paroxysmal VTs.

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BACKGROUND: There are well-recognized relationships between thyroid hormones, heart and peripheral vascular system. Thyroid hormones have relevant actions on the heart and circulation, generate multiple effects including hemodynamic changes and exert mediated effects on cardiac cells through gene expression. CLINICAL CASE: We present a 64-year-old woman with diagnosis of dilated cardiomyopathy with reduced ejection fraction, in whom coronary disease was thought of as the most probable etiology by clinical antecedents but in the evolution, other possible etiologies were to appear. CONCLUSIONS: Numerous complementary diagnostic studies were carried out, such as cinecoronariography, cardiac nuclear magnetic resonance imaging, laboratory analysis, to name a few, and it was concluded that the etiological cause was due to primary hypothyroidism.

INTRODUCCIÓN: Se reconoce la relación existente entre las hormonas tiroideas, el corazón y el sistema vascular periférico. Las hormonas tiroideas tienen relevantes acciones sobre el corazón y la circulación, y generan múltiples cambios, incluyendo alteraciones hemodinámicas y efectos mediados sobre las células cardiacas a través de la expresión génica. CASO CLÍNICO: Presentamos el caso de una paciente de 63 años con diagnóstico al ingreso de miocardiopatía dilatada con fracción de eyección reducida, en quien se pensó en enfermedad coronaria como primera causa, debido a sus antecedentes, pero en su evolución se fueron presentando otras posibles etiologías. CONCLUSIONES: Se realizaron numerosos estudios diagnósticos complementarios, como cinecoronariografía, resonancia magnética cardiaca y análisis de laboratorio, entre otros, y se llegó a la conclusión de que la causa fue hipotiroidismo primario.

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Abstract A 41-year-old man with end-stage heart failure due to nonischemic dilated cardiomyopathy was submitted to the Batista procedure as an alternative to heart transplantation. With surgery, the patient showed progressive clinical amelioration, achieving long-term stable NYHA functional class II, despite gradual dilation of the heart chambers. Persistent atrial fibrillation appeared on the last year of life, his clinical condition deteriorated, and the patient died 14 years, four months, and 13 days after the operation. To the best of our knowledge this seems to be the longest reported survival for a patient submitted to Batista operation.

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Abstract Dilated cardiomyopathy (DCM) is a clinical syndrome characterized by left ventricular dilatation and contractile dysfunction. It is the most common cause of heart failure in young adults. The advent of next-generation sequencing has contributed to the discovery of a large amount of genomic data related to DCM. Mutations involving genes that encode cytoskeletal proteins, the sarcomere, and ion channels account for approximately 40% of cases previously classified as idiopathic DCM. In this scenario, geneticists and cardiovascular genetics specialists have begun to work together, building knowledge and establishing more accurate diagnoses. However, proper interpretation of genetic results is essential and multidisciplinary teams dedicated to the management and analysis of the obtained information should be considered. In this review, we approach genetic factors associated with DCM and their prognostic relevance and discuss how the use of genetic testing, when well recommended, can help cardiologists in the decision-making process.

Resumo A miocardiopatia dilatada (MCD) é uma síndrome caracterizada por dilatação ventricular esquerda e disfunção contrátil, sendo considerada a causa mais comum de insuficiência cardíaca em adultos jovens. O uso do sequenciamento de nova geração tem contribuído com a descoberta de uma grande quantidade de dados genômicos relacionados à MCD, identificando mutações que envolvem genes que codificam proteínas do citoesqueleto, sarcômero e canais iônicos, os quais são responsáveis por aproximadamente 40% dos casos classificados como MCD idiopática. Nesse cenário, geneticistas e especialistas em genética cardiovascular passaram a atuar em conjunto, agregando conhecimento e estabelecendo diagnósticos mais precisos. No entanto, é fundamental interpretar corretamente os resultados genéticos, sendo necessário criar e fomentar equipes multidisciplinares dedicadas à gestão e análise das informações coletadas. Nesta revisão, abordamos os fatores genéticos associados à MCD, aspectos prognósticos, além de discutirmos como o emprego dos testes genéticos, quando bem indicados, pode ser útil na tomada de decisão na prática clínica dos cardiologistas.

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Resumen: La miocardiopatía dilatada (MCD) es la forma más común de disfunción ventricular con una prevalencia en adultos de alrededor de 1/2.500 individuos. Durante muchos años la forma más descripta de MCD en los registros fue la idiopática. En los últimos diez años, los avances en las imágenes y la genética han permitido identificar formas específicas dentro de este grupo que llamábamos comúnmente idiopático. El estudio de los pacientes con MCD debe seguir los pasos habituales, comenzando con el trabajo clínico, evaluación de antecedentes personales y familiares, examen físico, y análisis profundo de electrocardiograma y ecocardiograma. La identificación de las características clínicas sugestivas de enfermedades específicas debería conducir a un trabajo de diagnóstico de segundo nivel que puede incluir análisis bioquímicos específicos, resonancia cardíaca, estudios anatomopatológicos y genéticos. A continuación repasamos estrategias para la mejor identificación de etiologías específicas.

Summary: Dilated cardiomyopathy is the most common form of ventricular dysfunction with an adult prevalence of about 1 / 2.500 individuals. For many years the most described form of dilated cardiomyopathy in the registries was the idiopathic form. In the last ten years, advances in imaging and genetics have made it possible to identify specific forms within this group that we commonly called idiopathic. The study of patients with dilated cardiomyopathy should follow the usually steps, beginning with clinical work, evaluation of personal and family history, physical examination, and deep electrocardiogram analysis and echocardiography. The identification of clinical features suggestive of specific diseases should lead to a second-level diagnostic work that may include specific biochemical analyzes, cardiac resonance, anatomopathological and genetic studies. Next, we review strategies for the best identification of specific etiologies.