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Doxorubicin-induced cardiomyopathy (DOX-IC) is a significant and common complication in patients undergoing chemotherapy, leading to cardiac remodeling and reduced heart function. We hypothesized that the intrapericardial injection of hydrogels derived from the cardiac decellularized extracellular matrix (dECM) loaded with adipose tissue-derived stromal cells (ASC) and their secretome dampens or reverses the progression of DOX-IC. DOX-IC was induced in Wistar male rats through ten weekly intra-peritoneal injections of doxorubicin (cumulative dose: 18 mg/kg). We performed intrapericardial treatment in week five with dECM hydrogel loaded with ASC and their conditioned medium (CMed). The volume of intrapericardial injection was 2 ml/kg, the ASC density was 20 million/mL, while the hydrogel contained 100-fold concentrated CMed. Interstitial myocardial fibrosis was assessed by PicroSirius Red staining and hemodynamics parameters in pressure-volume loops. Compared to saline controls, interstitial myocardial fibrosis was reduced in ASC/CMed-loaded hydrogels treated animals (p = 0.0139). Ejection fraction and cardiac work efficiency improved in the ASC/CMed-treated rats compared to saline treatment (p = 0.0151 and p = 0.0655, respectively). The intrapericardial injection of dECM hydrogels loaded with ASC and their secretome warrants a novel therapeutic modality to improve ventricular hemodynamics and reduce cardiac remodeling in DOX-IC.

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FUNDAMENTO: A cardiomiopatia dilatada (CMD) é comum, com prevalência de 1/250 a 1/500 na população geral. É a principal causa de transplante cardíaco. Quando sua etiologia não é bem definida a investigação de uma causa genética se faz relevante. Em 40% é encontrada uma variante genética que pode explicar o fenótipo cardíaco encontrado. A prevalência de variantes do'* gene da filamina-C (FLNC) em pacientes com CMD é de até 4,5%. Tais mutações foram inicialmente relacionadas a uma forma de miopatia miofibrilar esquelética associada, em alguns casos, a uma forma não especificada de "cardiomiopatia". Por isso, desde 2012, o FLNC foi incluído na triagem genética de pacientes com cardiomiopatias hereditárias e morte súbita. RELATO DE CASO: Masculino, 15 anos, previamente hígido, deu entrada em pronto-socorro por primo-descompensação de Insuficiência Cardíaca em Perfil C. Relato de apresentar há semanas: fadiga, cansaço e dispneia aos esforços. A progressiva piora dos sintomas o fez buscar a emergência. Histórico familiar de CMD (mãe), com morte súbita aos 40 anos. O fato levantou hipótese para uma possível hereditariedade genética como etiologia à sua condição cardíaca. Ao ecocardiograma mostrou aumento importante das câmaras cardíacas esquerdas, com hipocontratilidade miocárdica difusa (FEVE=18% pelo Simpson). Disfunção Sistólica do Ventrículo Direito (FAC 18%) e sinais de hipertensão pulmonar. Foi realizada uma Ressonância Magnética Cardíaca, que demonstrou presença de realce tardio com morfologia não isquêmica sugerindo um padrão de cardiomiopatia genética. Após tentativas de otimização do tratamento e falhas no desmame do inotrópico, o transplante cardíaco foi considerado como única proposta terapêutica possível. Foi realizado teste genético para cardiomiopatias hereditárias, apresentando a variante patogênica no gene FLNC. Pacientes segue estável e em lista para o Transplante Cardíaco em uso de inotrópico. CONCLUSÃO: A pesquisa genética desempenha um papel importante na busca pela etiologia da Insuficiência Cardíaca presumivelmente idiopática. Especialmente por levar a diagnósticos mais específicos e oferecer um manejo mais adequado ao paciente. Em casos em que as alterações genéticas estejam associadas a um alto risco de morte súbita o implante de CDI pode ser indicado conforme a orientação das últimas diretrizes. Por isso, em pacientes com insuficiência cardíaca idiopática, o teste genético deve ser considerado como parte da investigação diagnóstica. Com finalidade de uma estratificação de risco mais precisa e a implementação de medidas terapêuticas de relevância prognóstica.

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Heart failure (HF) studies typically focus on ischemic and idiopathic heart diseases. Chronic chagasic cardiomyopathy (CCC) is a progressive degenerative inflammatory condition highly prevalent in Latin America that leads to a disturbance of cardiac conduction system. Despite its clinical and epidemiological importance, CCC molecular pathogenesis is poorly understood. Here we characterize and discriminate the plasma metabolomic profile of 15 patients with advanced HF referred for heart transplantation - 8 patients with CCC and 7 with idiopathic dilated cardiomyopathy (IDC) - using gas chromatography/quadrupole time-of-flight mass spectrometry. Compared to the 12 heart donor individuals, also included to represent the control (CTRL) scenario, patients with advanced HF exhibited a metabolic imbalance with 21 discriminating metabolites, mostly indicative of accumulation of fatty acids, amino acids and important components of the tricarboxylic acid (TCA) cycle. CCC vs. IDC analyses revealed a metabolic disparity between conditions, with 12 CCC distinctive metabolites vs. 11 IDC representative metabolites. Disturbances were mainly related to amino acid metabolism profile. Although mitochondrial dysfunction and loss of metabolic flexibility may be a central mechanistic event in advanced HF, metabolic imbalance differs between CCC and IDC populations, possibly explaining the dissimilar clinical course of Chagas' patients.

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Background: Different pathogens can cause dilated cardiomyopathy, one of them is Trypanosoma cruzi protozoan. T.cruzi-chronic infection causes chronic Chagasic cardiomyopathy and affects the sinus node and the conduction systembelow the bundle of His; besides, it shows excellent arrhythmogenic potential because of ventricular arrhythmias. Knowingthe clinical characteristics and performing serological tests to diagnose chronic Chagasic cardiomyopathy is essential. The serological diagnosis for searching the antibodies is based on the phase, which can be a predictor for the development of dilated cardiomyopathy. Objectives: In this work, the objective was to describe the frequency of dilated cardiomyopathy in patients with T. cruzi positive serology. Method: A total of 961 patients who were medically and clinically diagnosed with dilated cardiomyopathy were studied. Of these, 128 were diagnosed with chronic Chagasic cardiomyopathy and had positive serology for T. cruzi with two serological tests. Results: The clinical findings were obtained from the results of the electrocardiograms and were taken from the patient's clinical histories. Conclusion: In conclusion, complete blockage of the right branch of the bundle of His (44.2%) is one of the primary conduction disorders in the patients studied. Regarding seroprevalence, 14% of patients diagnosed with dilated cardiomyopathy had anti-T. cruzi antibodies.

Antecedentes: La cardiomiopatía dilatada puede ser causada por diferentes patógenos y uno de ellos es el protozoario Trypanosoma cruzi. La infección crónica causa la cardiomiopatía chagásica crónica, que afecta el nódulo sinusal y el sistema de conducción a nivel del haz de His; además, muestra gran potencial arritmogénico, ya que frecuentemente se presentan arritmias ventriculares. Para diagnosticar la cardiomiopatía chagásica crónica es indispensable conocer las características clínicas y realizar los ensayos serológicos. El diagnóstico serológico para la búsqueda de anticuerpos se basa en la fase de la enfermedad en la que se encuentre el individuo, los cuales pueden ser un predictor para el desarrollo de la cardiomiopatía dilatada. Objetivo: El objetivo de nuestro trabajo fue describir la frecuencia de cardiomiopatía dilatada en pacientes con serología positiva a T. cruzi en el Instituto Nacional de Cardiología Ignacio Chávez. Método: Se estudiaron 961 pacientes que fueron diagnosticados médica y clínicamente con cardiomiopatía dilatada y, de estos, 128 fueron diagnosticados con cardiomiopatía chagásica crónica, los cuales presentaban serología positiva a T. cruzi con dos pruebas serológicas. Resultados: Los hallazgos clínicos se obtuvieron de los resultados de los electrocardiogramas y fueron tomados de las historias clínicas de los pacientes. Conclusiones: En conclusión, el bloqueo completo de la rama derecha del haz de His (44.2%) es una de las principales alteraciones de la conducción en los pacientes estudiados. Con respecto a la seroprevalencia, el 14% de los pacientes con diagnóstico de cardiomiopatía dilatada tuvieron anticuerpos anti-T. cruzi.

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BACKGROUND: Woolly Hair Syndrome (WHS) is a rare birth condition that affects the structure of hair in non-black people. The pathogenesis is not yet defined. It is postulated that the hair follicle's desmosomes (specifically desmoplaquine, placoglobin and placofilin-1, which are cell structural proteins that keep the adhesion among close cells) would be altered in this pathology, leading to fragility in the cellular union. It is subdivided into two large groups: the localized or circumscribed variant and the generalized variant. From birth or first months of life, patients with WHS are clinically characterized by the presence of a portion or entire scalp area of very short frizzy hair, usually of a smaller diameter, brittle and lighter color. The most frequent skin manifestations are pilar keratosis and palmo-plantar keratodermia. The diagnosis is based on the clinical findings, and it is facilitated by trichotoscopic examination. However, a definitive diagnosis of WHS requires genetic testing. Oral agenesis, enamel defects (such as hypomineralization), atypical caries, dental inclusion, and malformed pin-shaped teeth may occur. OBJECTIVE: To describe an aesthetic alternative of oral rehabilitation using the mock-up technique in a patient with WHS. CLINICAL CASE: A 5-year-old female patient diagnosed with WHS from the National Institute of Child Health NIHCH: Breña, Lima, who was referred from the Genetic service to the Pediatric Dentistry service in order to screen outbreaks of infection associated with odontogenic origin and dental anomalies. At the ectoscopy, a patient with short capillary length, brittle and curly hair, dry skin, and nail dystrophy was observed. At the intraoral clinical examination, anterior pieces of 52, 51, 61, 62, and enamel hypomineralization were observed in all teeth. The radiographic examination showed agenesis of parts 41, 34, and 45. Integral dental treatment was performed in the operating room under general anesthesia due to the complexity of the case. Pulpectomy in pieces 52, 51, 61, 62, post of composite resin, and rehabilitation with supra-nanow filling resin using the mock-up technique were proposed as alternative treatments. CONCLUSION: The making of supra-nano filling resin-based crowns using the mock-up technique is an alternative treatment for aesthetic oral rehabilitation in deciduous dentition of patients with WHS. The aesthetic treatment was achieved using supra nano-filling resins. After 12 months of dental treatment, a favorable response was observed, improving the chewing, phonation and aesthetics of the patient.

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Dilated cardiomyopathy (DCM) is a disease of the heart muscle diagnosed by alterations resulting from ventricular systolic dysfunction with enlargement of the heart chambers, which is still underdiagnosed in non-human primates. This report is the first case of the DCM phenotype diagnosed by echocardiography and confirmed by necropsy in Callithrix penicillata.

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Cardiovascular diseases are rarely reported in birds. Among pet birds, they are most documented in psittacine birds. Dilated cardiomyopathy is a myocardial disease frequently found in poultry but with few reports in both pet and wild birds. An elderly male green-winged saltator (Saltator similis) died suddenly after presenting intermittent clinical signs of dyspnea, weakness, and vocalization changes. A general increase in cardiac size with a bulging apex was observed at necropsy. The heart exhibited an enlarged left ventricular space and flaccid musculature on the cut section. The liver was diffusely enlarged with rounded edges. No other gross lesions were observed. Samples were collected in 10% formalin and routinely processed. Histologically, bundles of myocardial fibers were irregularly arranged, with markedly elongated myofibrils and irregular cell contours. Additionally, multifocal areas of disruption were observed between the myofibrils. Diffuse hepatic congestion of sinusoids and portal veins was observed, and diffuse edema in the interstitium and para bronchi was present in the lungs. The clinical signs observed were nonspecific and can be found in several diseases of the cardiovascular and respiratory systems of birds. The gross and histological findings are compatible with what has been described for dilated cardiomyopathy in birds; however, left ventricular dilatation is uncommon in avian patients. Heart diseases in pet birds are still underdiagnosed, and reports of cardiomyopathies are limited to psittacine birds. To the authors' knowledge, this is the first report of a dilated cardiomyopathy in a passerine bird.(AU)

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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.

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.

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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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OBJECTIVE: To evaluate the frequency of iron status assessment in pediatric heart failure and the prevalence and adverse effects of absolute iron deficiency in dilated cardiomyopathy-induced heart failure. STUDY DESIGN: We retrospectively reviewed records of children with chronic heart failure at our center between 2010 and 2020. In children with dilated cardiomyopathy, we analyzed baseline cardiac function, hemoglobin level, and subsequent risk of composite adverse events (CAE), including death, heart transplant, ventricular assist device (VAD) placement, and transplant registry listing. Absolute iron deficiency and iron sufficiency were defined as transferrin saturations <20% and ≥30%, respectively; and indeterminant iron status as 20%-29%. RESULTS: Of 799 patients with chronic heart failure, 471 (59%) had no iron-related laboratory measurements. Of 68 children with dilated cardiomyopathy, baseline transferrin saturation, and quantitative left ventricular ejection fraction (LVEF), 33 (49%) and 14 (21%) were iron deficient and sufficient, respectively, and 21 (31%) indeterminant. LVEF was reduced to 23.6 ± 12.1% from 32.9 ± 16.8% in iron deficiency and sufficiency, respectively (P = .04), without a significant difference in hemoglobin. After stratification by New York Heart Association classification, in advanced class IV, hemoglobin was reduced to 10.9 ± 1.3 g/dL vs 12.7 ± 2.0 g/dL in iron deficiency and sufficiency, respectively (P = .01), without a significant difference in LVEF. CONCLUSIONS: In this single-center study, iron deficiency was not monitored in most children with chronic heart failure. In pediatric dilated cardiomyopathy-induced heart failure, absolute iron deficiency was prevalent and associated with clinically consequential and possibly correctable decreases in cardiac function and hemoglobin concentration.

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The case of a 49-year-old man with acute onset of heart failure is presented. The initial work-up showed a dilated cardiomyopathy with severely reduced left ventricular ejection fraction. In the differential diagnostic process, hypertensive, ischaemic, and valvular aetiologies were discarded. Subsequently, a cardiac magnetic resonance revealed global hypokinesis and inferior and anterior subepicardial fibrosis. Once differential diagnoses of subepicardial fibrosis (myocarditis, sarcoidosis, and Chagas disease) were discarded, a genetic panel was performed, resulting in a heterozygous mutation of desmoplakin (DSP) gene c.6697_6698del. A left-dominant DSP arrhythmogenic cardiomyopathy mutation was diagnosed. Structural myocardial abnormalities and ventricular arrhythmias characterize arrhythmogenic cardiomyopathy. Up to 50% of cases are associated with mutations in DSP genes (JUP, DSP, and PKP2). DSP is the fundamental component of the desmosome structure and provides structural support through intercellular adhesion. Therefore, when frequent differential diagnoses are discarded, genetic studies for dilated cardiomyopathy and DSP mutation should be considered.

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Dilated cardiomyopathy (DCM) is a primary myocardial disease, leading to heart failure and excessive risk of sudden cardiac death with rather poorly understood pathophysiology. In 2015, Parvari's group identified a recessive mutation in the autophagy regulator, PLEKHM2 gene, in a family with severe recessive DCM and left ventricular non-compaction (LVNC). Fibroblasts isolated from these patients exhibited abnormal subcellular distribution of endosomes, Golgi apparatus, lysosomes and had impaired autophagy flux. To better understand the effect of mutated PLEKHM2 on cardiac tissue, we generated and characterized induced pluripotent stem cells-derived cardiomyocytes (iPSC-CMs) from two patients and a healthy control from the same family. The patient iPSC-CMs showed low expression levels of genes encoding for contractile functional proteins (α and ß-myosin heavy chains and 2v and 2a-myosin light chains), structural proteins integral to heart contraction (Troponin C, T and I) and proteins participating in Ca2+ pumping action (SERCA2 and Calsequestrin 2) compared to their levels in control iPSC-derived CMs. Furthermore, the sarcomeres of the patient iPSC-CMs were less oriented and aligned compared to control cells and generated slowly beating foci with lower intracellular calcium amplitude and abnormal calcium transient kinetics, measured by IonOptix system and MuscleMotion software. Autophagy in patient's iPSC-CMs was impaired as determined from a decrease in the accumulation of autophagosomes in response to chloroquine and rapamycin treatment, compared to control iPSC-CMs. Impairment in autophagy together with the deficiency in the expression of NKX2.5, MHC, MLC, Troponins and CASQ2 genes, which are related to contraction-relaxation coupling and intracellular Ca2+ signaling, may contribute to the defective function of the patient CMs and possibly affect cell maturation and cardiac failure with time.

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INTRODUÇÃO: A taquicardiomiopatia é definida por disfunção ventricular esquerda reversível atribuída ao aumento da frequência ventricular, independente da origem da taquicardia, na ausência de outra etiologia mais provável. Apresenta-se geralmente por disfunção biventricular moderada a severa, com dilatação das cavidades e ausência de hipertrofia do ventrículo esquerdo (VE). Fibrilação atrial e Flutter de alta resposta ventricular são as mais comuns, no entanto taquicardias ventriculares (TV) também podem ser responsáveis. Os sintomas se apresentam mais precocemente nos pacientes com frequências cardíacas mais elevadas e são principalmente dispneia NYHA III-IV, palpitações e síncope. O tratamento é baseado na supressão da arritmia com medicações antiarrítmicas e/ou ablação além da terapêutica medicamentosa padrão para insuficiência cardíaca (IC). O diagnóstico é confirmado após a recuperação ou melhora da disfunção ventricular dentro de 1 a 6 meses após a resolução da taquiarritmia. MÉTODOS: Estudo de série de casos, retrospectivo e observacional. RESULTADOS: Foram avaliados três pacientes, masculinos, cujas idades eram 19, 23 e 61 anos, todos brancos. A apresentação inicial encontrada foi insuficiência cardíaca inicialmente manejada com betabloqueador, IECA e espironolactona. Disponibilizamos mais dados na tabela 1. Após ablação da TV, os pacientes evoluíram com melhora significativa de classe funcional e incremento da fração de ejeção de VE. O tempo médio de melhora foi de 5 meses e 20 dias após o procedimento e não houve complicações ou mesmo óbitos. CONCLUSÃO: A taquicardia ventricular incessante deve ser lembrada como possibilidade etiológica da disfunção ventricular. A ablação dos focos arritmogênicos pode resultar em recuperação rápida e significativa da disfunção ventricular, mostrando-se uma terapia segura e eficaz.

RESUMO

Paciente masculino, 38 anos, tabagista ativo e usuário de crack com diagnóstico recente de cardiomiopatia dilatada com fração de ejeção reduzida, dá entrada no pronto socorro com queixa de dispneia classe funcional IV, edema de membros inferiores, extremidades frias, ortopneia e dispneia paroxística noturna de início há quatro dias da admissão. Afirma tratamento para tromboembolismo pulmonar e AVE isquêmico há 06 meses. Nega precordialgia ou outros sinais e sintomas. Iniciado então tratamento para insuficiência cardíaca descompensada, com necessidade de inotrópico e noradrenalina para estabilidade hemodinâmica. Realizado ecocardiograma transtorácico e evidenciada disfunção biventricular importante (FEVE = 16% e FAC = 16%), com imagem ecogênica sugestiva de hematoma intramiocárdico dissecante na parede inferior do ventrículo esquerdo, acinesia da parede anterior e hipocontratilidade das demais paredes do ventrículo esquerdo, associada a imagem sugestiva de trombo atapetando a parede anterior e estendendo-se até a região apical do ventrículo esquerdo. Realizada tomografia computadorizada do coração com realce tardio que evidenciou infarto do miocárdico de parede inferior em todas as porções e inferolateralmediobasal, sem viabilidade. Não se observou curva de biomarcadores de necrose miocárdica. Iniciado então tratamento para insuficiência cardíaca descompensada, com necessidade de inotrópico e noradrenalina para estabilidade hemodinâmica. Paciente aguardava compensação clínica e posterior discussão de abordagem cirúrgica. Realizado novo estudo ecocardiográfico transtorácico após 07 dias, com paciente em melhora clínica dos sintomas, já sem uso de inotrópicos, onde evidenciou-se: disfunção biventricular importante (FEVE = 23% e FAC = 20%), com presença de trombo preenchendo todo o interior do aneurisma dissecante da parede inferior. Uma vez da compensação clínica e alto risco de morbimortalidade com abordagem cirúrgica desses casos, optou-se por tratamento clínico com anticoagulante e medidas para insuficiência cardíaca. Após 21 de internação, paciente recebeu alta hospitalar, assintomático para seguimento ambulatorial. O aneurisma dissecante do ventrículo esquerdo é uma condição clínica rara e que está relacionada a alta morbimortalidade pelos relatos descritos na literatura médica.

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A prática regular de esportes pode induzir adaptações no coração, sendo essa condição comumente chamada de "coração de atleta". As alterações observadas incluem dilatação das câmaras cardíacas, aumento da espessura miocárdica, melhora do enchimento ventricular, aumento da trabeculação do ventrículo esquerdo (VE), dilatação da veia cava inferior, entre outras. Essas alterações também podem ser observadas em algumas doenças cardíacas, como cardiomiopatia (CMP) dilatada, hipertrófica e outras. Dessa forma, os exames de imagem cardíaca são fundamentais na identificação dessas alterações e na diferenciação entre o "coração de atleta" e uma possível cardiopatia.(AU)

Exercise-induced adaptation may occur in amateur and professional athletes. This condition is commonly named "athlete's heart". The alterations observed include dilation of the heart chambers, increased myocardial thickness, improved ventricular filling, increased left ventricular trabeculation, dilation of the inferior vena cava, among others. These changes can also be observed in some heart diseases, such as dilated, hypertrophic and other cardiomyopathies (CMP). Thus, cardiac imaging tests are fundamental in identifying these alterations and in differentiating between "athlete's heart" and possible heart disease. (AU)

RESUMO

BACKGROUND: Advanced heart failure with reduced ejection fraction is rarely (HFrEF) associated with very low values of NT-pro-BNP, and its evidence poses a challenge to the true cause of the dysphnea and fatigue experienced by the patients. CASE: 48 year old male, BMI 25,17, with dilated cardiomiopathy, presented with NYHA class III, ecocardiography demonstrating EF of 34%, diastolic dysfunction grade 1 and a normal right ventricule function. His EKG showed a sinus rythm with a left bundle branch block, with QRS duration of 150 ms. Despite optimized medical therapy, patient maintained symptoms to a minimum effort, and his NT-pro-BNP blood levels were never higher than 60 pg/ml. DECISION-MAKING: Patient underwent a cardiac resynchronization therapy (CRT) after one year follow up. A recent cardiopulmonary exercise testing (CPET) showed a VO2max of 12 ml/kg/min, RER = 1,1, a VE/VCO2 slope of 37,8 and no evidence of pulmonary disfunction. Right heart catheterization (RHC) without the usage of inotropes demonstrated a cardiac output of 3,79 liters/minute and a pulmonary resistence of 1,85 Wood. Due to the persistence of symptoms, without clinical improvement, the patient was placed on the waiting list for heart transplantation despite his low blood levels of NT-pro-BNP. CONCLUSION: Heart transplantation in HFrEF requires an individual approach and the patientʼs clinical presentation must not be overlooked independently of NT-pro-BNP values. Further controlled trials are still needed to provide clear guidelines on the management of HFrEF patients presenting very low values of NT-proBNP.

RESUMO

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.