Resumo Fundamento: A amiloidose por transtirretina (ATTR) é uma doença infiltrativa causada pela deposição anormal de proteína principalmente no coração e no sistema nervoso periférico. Quando acomete o coração, a doença manifesta-se como uma cardiomiopatia restritiva e, quando afeta o sistema nervoso periférico e autônomo, apresenta-se como uma polineuropatia, podendo ser chamada de Polineuropatia Amiloidótica Familiar (PAF). Existem dois subtipos de ATTR, a ATTR selvagem, em que não há variantes genéticas, e a ATTR hereditária, caracterizada por uma variante no gene que codifica a proteína transtirretina (T\TR). Em ambos os subtipos, o envolvimento cardíaco é o principal marcador prognóstico. Objetivos: Avaliar a prevalência do envolvimento cardíaco subclínico em uma amostra de pacientes com variantes genéticas no gene TTR usando a cintilografia com pirofosfato e o ecocardiograma com strain; comparar os achados cintilográficos e as medidas de strain; avaliar a associação entre PAF e o envolvimento subclínico; e analisar se existe uma associação entre uma variante genética específica e o envolvimento cardíaco. Métodos: Estudo transversal com carreadores de variantes no gene TTR sem sintomas cardiovasculares e sem alterações nos parâmetros da eletrocardiografia ou do ecocardiograma convencional. Todos os pacientes foram submetidos à cintilografia com pirofosfato e à ecocardiografia com análise de strain. O envolvimento cardíaco subclínico, definido como um escore de Perugini ≥ 2, razão Coração (C)/ Hemitórax Contralateral (CL) ≥ 1,5 em uma hora, C/CL ≥ 1,3 na terceira hora, ou um strain longitudinal global (SGL) ≤ −17%. Realizadas análises descritiva e analítica, e aplicados o teste exato de Fisher e o teste de Mann-Whitney. Um valor de p<0,05 foi considerado significativo. Resultados: Os 23 pacientes avaliados apresentavam uma idade mediana de 51 (37-57) anos, 15 (65,2%) eram do sexo feminino, 12 (52,2%) eram pardos, nove (39,1%) apresentavam hipertensão arterial sistêmica, e nove (39,1%) tinham um diagnóstico prévio de PAF. Dos nove pacientes com PAF, oito (34,8%) usavam tafamidis. As variantes genéticas identificadas foram Val142IIe, Val50Met e IIe127Val. O valor mediano do SGL foi −19% (-16% – −20%). Dos 23 pacientes, nove (39,1%; 95% CI = 29–49%) preencheram os critérios de envolvimento cardíaco, seis (26%) somente pelo critério do SGL. Não houve associação entre PAF e um carreador assintomático avaliado por ecocardiograma com análise de strain e pela cintilografia com pirofostato (p=0,19). A prevalência de hipertensão arterial sistêmica, diabetes mellitus, dislipidemia, tabagismo e SGL reduzido não foi diferente entre os grupos. A velocidade da onda e’ septal foi a única variável que apresentou diferença significativa entre os indivíduos com e sem SGL reduzido, com uma área sob a curva ROC de 0,80 (IC95% = 0,61–0,98, p = 0,027). A melhor acurácia diagnóstica foi alcançada com uma velocidade e’ septal ≤ 8,5 cm/s. Não houve associação entre o tipo de variante genética e o envolvimento cardíaco pré-clínico, nem entre o uso de tafamidis e este mesmo envolvimento (37,5% versus 40,0%, p = 0,90). Conclusão: O envolvimento cardíaco subclínico foi frequente em uma amostra de carreadores da variante genética do gene TTR. Um valor do SGL reduzido foi o achado mais comum. Não houve associação entre a presença de polineuropatia amiloidótica e o envolvimento subclínico. O tipo de variante genética não foi associado com envolvimento cardíaco precoce. Nesta amostra, o uso de tafamidis (20mg/dia) não foi associado com uma menor prevalência de envolvimento cardíaco subclínico. Fundamento (ATTR manifestase manifesta autônomo apresentase apresenta . (PAF) selvagem hereditária T\TR. T\TR T TR (T\TR) prognóstico Objetivos Métodos convencional 2 C/ C / (C) CL (CL 1 5 1, hora CCL 13 3 (SGL 17 −17% analítica MannWhitney. MannWhitney Mann Whitney. Whitney Mann-Whitney p005 0 05 p<0,0 significativo Resultados 3757 37 57 (37-57 anos 65,2% 652 65 (65,2% feminino 52,2% 522 52 (52,2% pardos 39,1% 391 39 (39,1% sistêmica 34,8% 348 34 8 (34,8% Val142IIe ValIIe Val IIe ValMet Met IIe127Val IIeVal 19 −19 16% 16 (-16 −20%. 20 −20% −20%) 95 29–49% 2949 29 49 26% 26 (26% p=0,19. p019 p=0,19 (p=0,19) mellitus dislipidemia grupos 080 80 0,8 IC95% IC95 IC (IC95 061098 61 98 0,61–0,98 0,027. 0027 0,027 027 0,027) 85 8, cms cm s cm/s préclínico, préclínico pré clínico, clínico pré-clínico 37,5% 375 (37,5 400 40 40,0% 0,90. 090 0,90 90 0,90) Conclusão comum precoce 20mg/dia 20mgdia mgdia 20mg dia mg (20mg/dia (PAF (T\TR (C −17 p00 p<0, (37-5 65,2 6 (65,2 52,2 (52,2 39,1 (39,1 34,8 (34,8 −1 (-1 −20 9 29–49 294 4 (26 p01 p=0,1 (p=0,19 08 0, IC9 (IC9 06109 0,61–0,9 002 0,02 02 37,5 (37, 40,0 09 0,9 p0 p<0 (37- 65, (65, 52, (52, 39, (39, 34, (34, − (- −2 29–4 (2 p=0, (p=0,1 (IC 0610 0,61–0, 00 0,0 37, (37 40, p< (65 (52 (39 (34 ( 29– p=0 (p=0, 061 0,61–0 (3 (6 (5 p= (p=0 06 0,61– (p= 0,61 (p 0,6

Abstract Background: Transthyretin amyloidosis (ATTR) is an infiltrative disease caused by abnormal protein deposition mainly in the heart and peripheral nervous system. When it affects the heart, the disease presents as restrictive cardiomyopathy; when it affects the peripheral and autonomic nervous system, it manifests as polyneuropathy, and is called familial amyloid polyneuropathy (FAP). There are two ATTR subtypes: wild-type ATTR, where there is no mutation, and mutant ATTR (ATTRm), which is characterized by a mutation in the gene encoding the transthyretin protein (TTR). In both subtypes, cardiac involvement is the major marker of poor prognosis. Objectives: To assess the prevalence of subclinical cardiac involvement in a sample of patients with TTR gene mutation by using pyrophosphate scintigraphy and strain echocardiography; to compare scintigraphy and strain findings; to evaluate the association between neurological manifestations (FAP) and subclinical cardiac involvement; and to analyze whether there is an association between any specific mutation and cardiac involvement. Methods: This is a cross-sectional study with carriers of the TTR gene mutation, without cardiovascular symptoms or changes in electrocardiographic or conventional echocardiographic parameters. All patients underwent pyrophosphate scintigraphy and strain echocardiography. Subclinical cardiac involvement was defined as a Perugini score ≥ 2, heart-to-contralateral lung (H/CL) ratio ≥ 1.5 at 1 h, H/CL ≥1.3 at 3 h, or global longitudinal strain (GLS) ≤ −17%. Descriptive and analytical analyses were performed and Fisher's exact test and Mann–Whitney test were applied. A value of p < 0.05 was considered significant. Results: The 23 patients evaluated had a median age of 51 years (IQR 37–57 years), 15 (65.2%) were female, 12 (52.2%) were Pardo, nine (39.1%) had systemic arterial hypertension, and nine (39.1%) had a previous diagnosis of FAP. Of the nine patients with FAP, 8 (34.8%) were on tafamidis. The associated mutations were Val142IIe, Val50Met, and IIe127Val. The median GLS in the sample was −19% (−16% to −20%). Of the 23 patients, nine (39.1%; 95% CI = 29–49%) met criteria for cardiac involvement, six (26%) by the GLS-based criteria only. There was no association between having FAP and being an asymptomatic carrier, as assessed by strain echocardiography and pyrophosphate scintigraphy (p = 0.19). The prevalence of systemic arterial hypertension, diabetes mellitus, dyslipidemia, smoking, and reduced GLS did not differ between groups. Septal e’ wave velocity was the only variable that significantly differed between individuals with and without reduced GLS, with an area under the ROC curve of 0.80 (95% CI = 0.61–0.98, p = 0.027). The best diagnostic accuracy was achieved with a septal e’ velocity ≤ 8.5 cm/s. There was no association between mutation type and preclinical cardiac involvement, nor between tafamidis use and lower degree of cardiac involvement (37.5% versus 40.0%, p = 0.90). Conclusion: Subclinical cardiac involvement was common in a sample of TTR mutation carriers without cardiac involvement. Reduced left ventricular GLS was the most frequent finding. There was no association between the presence of amyloid polyneuropathy and subclinical cardiac involvement. Type of mutation was not associated with early cardiac involvement. In this sample, the use of tafamidis 20 mg/day was not associated with a lower prevalence of subclinical cardiac involvement. Background (ATTR system cardiomyopathy . subtypes wildtype wild ATTRm, ATTRm , (ATTRm) TTR. (TTR) prognosis Objectives findings (FAP Methods crosssectional cross sectional parameters 2 hearttocontralateral contralateral HCL H CL (H/CL 5 1. h 13 ≥1. (GLS 17 −17% Fishers Fisher s MannWhitney Mann Whitney applied 005 0 05 0.0 significant Results IQR 3757 37 57 37–5 years, years) 65.2% 652 65 (65.2% female 52.2% 522 52 (52.2% Pardo 39.1% 391 39 (39.1% hypertension 34.8% 348 34 (34.8% Val142IIe ValIIe Val IIe Val50Met ValMet Met IIe127Val IIeVal 19 −19 −16% 16 (−16 −20%. −20% −20%) 95 29–49% 2949 29 49 26% 26 (26% GLSbased based carrier 0.19. 019 0.19 0.19) mellitus dyslipidemia smoking groups e 080 80 0.8 (95 061098 61 98 0.61–0.98 0.027. 0027 0.027 027 0.027) 85 8. cms cm cm/s 37.5% 375 (37.5 400 40 40.0% 0.90. 090 0.90 90 0.90) Conclusion finding mgday mg day (ATTRm (TTR ≥1 −17 00 0. 37– 65.2 6 (65.2 52.2 (52.2 39.1 (39.1 34.8 (34.8 −1 −16 (−1 −20 9 29–49 294 4 (26 01 0.1 08 (9 06109 0.61–0.9 002 0.02 02 37.5 (37. 40.0 09 0.9 65. (65. 52. (52. 39. (39. 34. (34. − (− −2 29–4 (2 ( 0610 0.61–0. 37. (37 40. (65 (52 (39 (34 29– 061 0.61–0 (3 (6 (5 06 0.61– 0.61 0.6

Abstract Cardiomyopathy is characterized by structural and functional abnormalities in the heart muscle that are not influenced by coronary disease, hypertension, valve disease, or congenital heart defects. Genetic testing, which helps diagnose the molecular bases of cardiomyopathy, can affect prognostication and clinical decisions. Recent guidelines advocate classifying cardiomyopathies based on phenotypic categories, which encompass a range of clinical presentations, underlying causes, and outcomes. Beyond deepening our grasp of the disease, genetic testing can distinguish between diseases with similar presentations, known as genocopies, which can profoundly influence treatment decisions. Genetic testing is also essential for reproductive counseling and risk evaluation among family members. Combined with advanced imaging techniques such as magnetic resonance imaging, genetic investigation offers a comprehensive understanding of the disease. Thus, as the nature of cardiomyopathies continues to be unraveled, cardiologists must stay updated on advances in genetic testing and their practical applications. disease hypertension defects cardiomyopathy decisions categories presentations causes outcomes genocopies members Thus unraveled applications

Resumo A Hipereosinofilia (HE) é definida como uma contagem de eosinófilos superior a 1500 células/microL no sangue periférico em dois exames, realizados com intervalo mínimo de um mês e/ou confirmação anatomopatológica de HE, com eosinófilos compreendendo mais de 20% de todas as células nucleadas da medula óssea. A Síndrome hipereosinofílica (SHE) indica a presença de HE com comprometimento de órgãos por ação eosinofílica, podendo ser classificada como primária (ou neoplásica), secundária (ou reativa) e idiopática. O comprometimento cardíaco ocorre em até 5% dos casos na fase aguda e em 20% na fase crônica da doença, variando de casos oligossintomáticos até miocardite aguda fulminante ou cardiomiopatia restritiva crônica (endomiocardite de Loeffler). No entanto, o grau de disfunção cardíaca não se correlaciona diretamente com o grau de eosinofilia. O envolvimento cardíaco na SHE ocorre em três fases: necrótica inicial, trombótica e necrótica final. Pode se manifestar como insuficiência cardíaca, arritmias e fenômenos tromboembólicos. O diagnóstico de cardiopatia é baseado em métodos de imagem multimodalidade, com ênfase na importância do ecocardiograma transtorácico (ETT). Em pacientes com janela acústica limitada, podem ser utilizados agentes de contraste ultrassonográfico, que permitem melhor visualização das bordas endocárdicas e da região ventricular apical. Técnicas para análise da deformação miocárdica podem evidenciar redução do strain em segmentos apicais e preservação nos demais segmentos (reverse apical sparing). A ressonância magnética cardíaca permite a caracterização do realce tardio subendocárdico de gadolínio, e a biópsia endomiocárdica é considerada o padrão ouro no diagnóstico de cardiopatia. O tratamento é baseado na etiologia da SHE. (HE 150 célulasmicroL microL exames eou 20 óssea (SHE eosinofílica neoplásica, neoplásica , neoplásica) reativa idiopática 5 doença endomiocardite Loeffler. Loeffler . Loeffler) entanto eosinofilia fases inicial final tromboembólicos multimodalidade ETT. ETT (ETT) limitada ultrassonográfico reverse sparing. sparing sparing) gadolínio 15 2 (ETT 1

Abstract Hypereosinophilia (HE) is defined as an eosinophil count exceeding 1500 cells/microL in peripheral blood in two tests, performed with an interval of at least one month and/or anatomopathological confirmation of HE, with eosinophils comprising more than 20% of all nucleated cells in the bone marrow. Hypereosinophilic syndrome (HES) indicates the presence of HE with organ involvement due to eosinophil action, which can be classified as primary (or neoplastic), secondary (or reactive), and idiopathic. Cardiac involvement occurs in up to 5% of cases in the acute phase and 20% of the chronic phase of the disease, ranging from oligosymptomatic cases to fulminant acute myocarditis or chronic restrictive cardiomyopathy (Loeffler endomyocarditis). However, the degree of cardiac dysfunction does not directly correlate with the degree of eosinophilia. The cardiac involvement of HES occurs in three phases: initial necrotic, thrombotic, and finally necrotic. It can manifest as heart failure, arrhythmias, and thromboembolic phenomena. The diagnosis of cardiopathy is based on multimodality imaging, with an emphasis on the importance of echocardiography (echo) as the primary examination. TTE with enhanced ultrasound agents can be used for better visualization, allowing greater accuracy in assessing ventricular apex, and myocardial deformation indices, such as longitudinal strain, may be reduced, especially in the ventricular apex (reverse apical sparing). Cardiac magnetic resonance imaging allows the characterization of subendocardial late gadolinium enhancement, and endomyocardial biopsy is considered the gold standard in diagnosing cardiopathy. Treatment is based on the etiology of HES. (HE 150 cellsmicroL microL tests andor 20 marrow (HES action neoplastic, neoplastic , neoplastic) reactive, reactive reactive) idiopathic 5 disease Loeffler endomyocarditis. endomyocarditis . endomyocarditis) However eosinophilia phases necrotic thrombotic failure arrhythmias phenomena echo (echo examination visualization indices strain reduced reverse sparing. sparing sparing) enhancement 15 2 1

Abstract Background: In transthyretin amyloid cardiomyopathy (ATTR-CM), the assessment of functional capacity is of great importance. A distance < 300m covered in the 6-minute walk test (6MWT) is linked to a poorer prognosis in heart failure (HF) patients. However, in ATTR-CM, this association is not well-established, particularly among its various presentations and variants. Objectives: To evaluate functional capacity and, the prognostic value of the 6MWT in patients with ATTR-CM and to identify their clinical characteristics according to the type of ATTR-CM. Methods: This was a retrospective, single-center study that included patients with a confirmed diagnosis of ATTR-CM who performed the 6MWT. Median follow-up was 19 months (8 – 29). The significance level was set at P < 0.05. Results: A total of 32 patients were analyzed; 59.4% had the hereditary form (ATTRh), of which 63.2% were Val142Ile. Mean left ventricular ejection fraction (LVEF) was lower in the ATTRh group when compared to the wild-type form (ATTRwt) [42 ±11 versus 52± 9 %; p = 0.029]. Median distance walked in the 6MWT was 328 m (269 – 397; IQR) in ATTRwt and 304 m (246 – 387; IQR) in ATTRh, with no difference between groups (p = 0.833). In patients with a distance <300 m in the 6MWT, survival was lower (28.8 vs 36.3 months; p = 0.041). Conclusions: A distance <300 m walked in the 6MWT was linked to shorter event-free survival in patients with ATTR-CM. Functional capacity of most patients was very low, and no differences were observed in the distance covered between the wild-type form and the hereditary form of ATTR. Background ATTRCM, ATTRCM ATTR CM , (ATTR-CM) importance 6minute minute 6 MWT (6MWT HF (HF However CM, wellestablished, wellestablished well established, established well-established variants Objectives ATTRCM. CM. Methods retrospective singlecenter single center followup follow up 1 8 ( 29. 29 . 29) 005 0 05 0.05 Results 3 analyzed 594 59 4 59.4 (ATTRh) 632 63 2 63.2 Val142Ile ValIle Val Ile LVEF (LVEF wildtype wild (ATTRwt 42 [4 11 ±1 52 % 0.029. 0029 0.029 029 0.029] 269 (26 397 IQR 30 246 (24 387 0.833. 0833 0.833 833 0.833) 300 <30 28.8 288 28 (28. 363 36 36. 0.041. 0041 0.041 041 0.041) Conclusions eventfree event free low (ATTR-CM 00 0.0 5 59. (ATTRh 63. [ ± 002 0.02 02 26 (2 39 24 38 083 0.83 83 <3 28. (28 004 0.04 04 0. 08 0.8

Abstract Amyloidosis is caused by the extracellular deposition of insoluble protein arrangements, formed from structural changes in different precursor proteins. The prominent clinical and epidemiological relevance of transthyretin amyloid cardiomyopathy (ATTR-CM), whose increased incidence is associated with the aging of the global population, given its high prevalence in the elderly population, has motivated the intensification of the search for specific therapeutic targets capable of altering the natural course of the disease. The past few years have been especially prolific with the emergence of significant new therapies for ATTR-CM, with important trials demonstrating positive results and changing the natural history of an inexorable and deadly disease. Up to now, the available drugs mainly promote stabilization of disease progression in patients with no advanced heart disease but grant no reversal of the structural cardiac disease in most cases. The transthyretin (TTR)-depleters based on monoclonal antibodies directed against the deposited amyloid fibers hold promise to fulfill these expectations. The DepleTTR-CM will test the safety and efficacy of a monoclonal antibody directed against TTR fibrils deposited in the myocardium of patients with ATTR-CM. This will be the first multicenter randomized controlled trial to test this new class of anti-amyloid treatment, the "amyloid fibril depleters". This is a strategy designed to revert the amyloid accumulation in the cardiac interstitium, which is expected to translate into relevant clinical benefits, with the potential to close a gap in the ATTR-CM treatment strategies, mainly in patients with more severe disease. arrangements proteins ATTRCM, ATTRCM ATTR CM , (ATTR-CM) population CM, now cases TTRdepleters depleters expectations DepleTTRCM DepleTTR ATTRCM. CM. antiamyloid anti depleters. . depleters" interstitium benefits strategies (ATTR-CM

Abstract The production of second-generation biodiesel is seen as more sustainable than first-generation biodiesel as it is produced from residues such as animal fats, a by-product of the meat industry. In Brazil, the biodiesel sector has become the main market for animal fats, absorbing around 712 thousand tons in 2018, equivalent to 38% of the fat produced. In order to reach this level, research and development were carried out supported by the Brazilian government through the Brazilian Biodiesel Technology and Innovation Network. The objective of this study is to map the scientific networks formed by Brazilian organizations that have collaborated to solve research problems regarding animal fats for biodiesel production through Social Networks Analysis and The Theory of Complex Networks. An exploratory-descriptive study was conducted in the main collection of Web of Science (WoS). It was observed that research started in 2008 and involved more than 80 institutions. The institutional network formed was of a small-world type, composed of 20 clusters. The research was indexed in 19 knowledge areas, with an emphasis on Chemistry, Energy and Fuels, and Engineering and 70% of them received some type of funding to conduct the research.

Resumo Com o aumento da expectativa de vida da população e a maior frequência de fatores de risco como obesidade, hipertensão arterial e diabetes, espera-se um aumento na prevalência de insuficiência cardíaca com fração de ejeção preservada (ICFEp). Entretanto, no momento, o diagnóstico e o tratamento de pacientes com ICFEp permanecem desafiadores. O diagnóstico sindrômico de ICFEp inclui diversas etiologias e doenças com tratamentos específicos, mas que apresentam pontos em comum em relação à apresentação clínica e à avaliação laboratorial no que diz respeito aos biomarcadores como BNP e NT-ProBNP, à avaliação ecocardiográfica do remodelamento cardíaco e às pressões de enchimento diastólico ventricular esquerdo. Extensos ensaios clínicos randomizados envolvendo a terapia nesta síndrome falharam na demonstração de benefícios para o paciente, fazendo-se necessária uma reflexão acerca do diagnóstico, dos mecanismos de morbidade, da taxa de mortalidade e da reversibilidade. Na revisão, serão abordados os conceitos atuais, as controvérsias e, especialmente, os desafios no diagnóstico da ICFEp através de uma análise crítica do escore da European Heart Failure Association.

Abstract With the increase in the population’s life expectancy and the higher frequency of risk factors such as obesity, hypertension and diabetes, an increase in the prevalence of heart failure with preserved ejection fraction (HFpEF) is expected. However, to date, the diagnosis and treatment of patients with HFpEF remain challenging. The syndromic diagnosis of HFpEF includes several etiologies and diseases with specific treatments but has points in common regarding the clinical presentation, laboratory evaluation related to biomarkers, such as BNP and NT-ProBNP, and echocardiographic evaluation of cardiac remodeling and left ventricular diastolic filling pressures. Extensive randomized clinical trials involving the treatment of this condition have failed to demonstrate benefits to the patient, making it necessary to reflect on the diagnosis, mechanisms of morbidity, mortality and reversibility in this syndrome. In this review, the current concepts, controversies and challenges, especially regarding diagnosis, will be addressed, critically analyzing the European Heart Failure Association score for the diagnosis of HFpEF.

Resumo Fundamento Amiloidose sistêmica é uma doença com manifestações clínicas diversas. O diagnóstico envolve suspeita clínica, aliada a métodos complementares. Objetivo Descrever o perfil clínico, laboratorial, eletrocardiográfico e de imagem no acometimento cardíaco da amiloidose sistêmica. Métodos Estudo de uma amostra de conveniência, analisando dados clínicos, laboratoriais, eletrocardiográficos, ecocardiográficos, medicina nuclear e ressonância magnética. Considerou-se significância estatística quando p < 0,05. Resultados Avaliaram-se 105 pacientes (com mediana de idade de 66 anos), sendo 62 homens, dos quais 83 indivíduos apresentavam amiloidose por transtirretina (ATTR) e 22 amiloidose por cadeia leve (AL). Na ATTR, 68,7% eram de caráter hereditário (ATTRh) e 31,3% do tipo selvagem (ATTRw). As mutações mais prevalentes foram Val142Ile (45,6%) e Val50Met (40,3%). O tempo de início dos sintomas ao diagnóstico foi 0,54 e 2,15 anos nas formas AL e ATTR (p < 0,001), respectivamente. O acometimento cardíaco foi observado em 77,9% dos ATTR e 90,9% dos AL. Observaram-se alterações de condução atrioventricular em 20% e intraventricular em 27,6% dos pacientes, sendo 33,7 % na ATTR e 4,5% das AL (p = 0,006). A forma ATTRw apresentou mais arritmias atriais que os ATTRh (61,5% x 22,8%; p = 0,001). Ao ecocardiograma a mediana da espessura do septo na ATTRw x ATTRh x AL foi de 15 mm x 12 mm x 11 mm (p = 0,193). Observou-se BNP elevado em 89,5% dos indivíduos (mediana 249 ng/mL, IQR 597,7) e elevação da troponina em 43,2%. Conclusão Foi possível caracterizar, em nosso meio, o acometimento cardíaco na amiloidose sistêmica, em seus diferentes subtipos, através da história clínica e dos métodos diagnósticos descritos.

Abstract Background Systemic amyloidosis is a disease with heterogeneous clinical manifestations. Diagnosis depends on clinical suspicion combined with specific complementary methods. Objective To describe the clinical, laboratory, electrocardiographic, and imaging profile in patients with systemic amyloidosis with cardiac involvement. Methods This study was conducted with a convenience sample, analyzing clinical, laboratory, electrocardiographic, echocardiographic, nuclear medicine, and magnetic resonance data. Statistical significance was set at p < 0.05. Results A total of 105 patients were evaluated (median age of 66 years), 62 of whom were male. Of all patients, 83 had transthyretin (ATTR) amyloidosis, and 22 had light chain (AL) amyloidosis. With respect to ATTR cases, 68.7% were the hereditary form (ATTRh), and 31.3% were wild type (ATTRw). The most prevalent mutations were Val142Ile (45.6%) and Val50Met (40.3%). Time from onset of symptoms to diagnosis was 0.54 and 2.15 years, in the AL and ATTR forms, respectively (p < 0.001). Cardiac involvement was observed in 77.9% of patients with ATTR and in 90.9% of those with AL. Alterations were observed in atrioventricular and intraventricular conduction in 20% and 27.6% of patients, respectively, with 33.7% in ATTR and 4.5% in AL (p = 0.006). In the ATTRw form, there were more atrial arrhythmias than in ATTRh (61.5% versus 22.8%; p = 0.001). On echocardiogram, median septum thickness in ATTRw, ATTRh, and AL was 15 mm, 12 mm, and 11 mm, respectively (p = 0.193). Elevated BNP was observed in 89.5% of patients (median 249, ICR 597.7), and elevated troponin was observed in 43.2%. Conclusion In this setting, it was possible to characterize cardiac involvement in systemic amyloidosis in its different subtypes by means of clinical history and the diagnostic methods described.

Abstract Background: Pericardial effusion is a relatively common finding and can progress to cardiac tamponade; etiological diagnosis is important for guiding treatment decisions. With advances in medicine and improvement in the social context, the most frequent etiological causes have changed. Objectives: To evaluate the clinical and laboratory characteristics, etiology, and clinical course of patients with pericardial effusion and cardiac tamponade. Materials and methods: Patients with pericardial effusion classified as small (< 10 mm), moderate (between 10-20 mm), or severe (> 20 mm) were included. Data from the clinical history, physical examination, laboratory tests, and complementary tests were evaluated in patients with pericardial effusion and cardiac tamponade. The significance level was set at 5%. Results: A total of 254 patients with a mean age of 53.09 ± 17.9 years were evaluated, 51.2% of whom were female. A total of 40.4% had significant pericardial effusion (> 20 mm). Pericardial tamponade occurred in 44.1% of patients. Among pericardial effusion patients without tamponade, the most frequent etiologies were: idiopathic (44.4%) and postsurgical (17.6%), while among those with tamponade, the most frequent etiologies were postsurgical (21.4%) and postprocedural (19.6%). The mean follow-up time was 2.2 years. Mortality was 42% and 23.2 in those with and without tamponade, respectively (p=0.001). Conclusions: There is an etiological difference between pericardial effusion patients with and without cardiac tamponade. An idiopathic etiology is more common among those without tamponade, while postinterventional/postsurgical is more common among those with tamponade. The tamponade group had a higher mortality rate.

Resumo Fundamento As características histopatológicas da doença de Chagas (DCC) são: presença de miocardite, destruição das fibras cardíacas e fibrose miocárdica. A Galectina-3 (Gal-3) é um biomarcador envolvido no mecanismo de fibrose e inflamação que pode ser útil para a estratificação de indivíduos com DCC por risco. Objetivos Nosso objetivo foi avaliar se níveis elevados de Gal-3 estão associados a formas graves de cardiomiopatia chagásica (CC) e são preditivos de mortalidade. Métodos Estudamos doadores de sangue (DS) positivos para anti-T. cruzi: não-CC-DS (187 DS sem CC com eletrocardiograma [ECG] e fração de ejeção do ventrículo esquerdo [FEVE] normais); CC-Não-Dis-DS (46 DS com CC e apresentando ECG anormal, mas FEVE normal); e 153 controles negativos correspondentes. Esta amostra foi composta por 97 pacientes com CC grave (CC-Dis). Usamos as correlações de Kruskall-Wallis e Spearman para testar a hipótese de associações, assumindo um p bicaudal <0,05 como significativo. Resultados O nível de Gal-3 foi de 12,3 ng/mL para não-CC-DS, 12,0 ng/mL para CC-Não-Dis-DS, 13,8 ng/mL para controles e 15,4 ng/mL para CC-Dis. FEVE <50 foi associada a níveis mais elevados de Gal-3 (p=0,0001). Em nosso modelo de regressão linear ajustado, encontramos associação entre os níveis de Gal-3 e os parâmetros do ecocardiograma em indivíduos positivos para T. cruzi. Nos pacientes CC-Dis, encontramos uma associação significativa de níveis mais elevados de Gal-3 (≥15,3 ng/mL) e morte ou transplante cardíaco em acompanhamento de cinco anos (Hazard ratio – HR 3,11; IC95% 1,21– 8,04; p=0,019). Conclusões Em pacientes com CC, níveis mais elevados de Gal-3 estiveram significativamente associados a formas graves da doença e maior taxa de mortalidade em longo prazo, o que significa que pode ser um meio efetivo para identificar pacientes de alto risco. (Arq Bras Cardiol. 2021; 116(2):248-256)

Abstract Background The histopathological characteristics of Chagas disease (ChD) are: presence of myocarditis, destruction of heart fibers, and myocardial fibrosis. Galectin-3 (Gal-3) is a biomarker involved in the mechanism of fibrosis and inflammation that may be useful for risk stratification of individuals with ChD. Objectives We sought to evaluate whether high Gal-3 levels are associated with severe forms of Chagas cardiomyopathy (CC) and whether they are predictive of mortality. Methods We studied anti-T. cruzi positive blood donors (BD): Non-CC-BD (187 BD without CC with normal electrocardiogram [ECG] and left ventricular ejection fraction [LVEF]); CC-Non-Dys-BD (46 BD with CC with abnormal ECG but normal LVEF); and 153 matched serum-negative controls. This cohort was composed of 97 patients with severe CC (CC-Dys). We used Kruskall-Wallis and Spearman’s correlation to test hypothesis of associations, assuming a two-tailed p<0.05 as significant. Results The Gal-3 level was 12.3 ng/mL for Non-CC-BD, 12.0 ng/mL for CC-Non-Dys-BD, 13.8 ng/mL for controls, and 15.4 ng/mL for CC-Dys. LVEF<50 was associated with higher Gal-3 levels (p=0.0001). In our linear regression adjusted model, we found association between Gal-3 levels and echocardiogram parameters in T. cruzi-seropositive subjects. In CC-Dys patients, we found a significant association of higher Gal-3 levels (≥15.3 ng/mL) and subsequent death or heart transplantation in a 5-year follow-up (Hazard ratio – HR 3.11; 95%CI 1.21–8.04; p=0.019). Conclusions In ChD patients, higher Gal-3 levels were significantly associated with severe forms of the disease and more long-term mortality, which means it may be a useful means to identify high-risk patients. (Arq Bras Cardiol. 2021; 116(2):248-256)

OBJECTIVES: To evaluate the effects of sympathectomy on the myocardium in an experimental model. METHODS: The study evaluated three groups of male Wistar rats: control (CT; n=15), left unilateral sympathectomy (UNI; n=15), and bilateral sympathectomy (BIL; n=31). Sympathectomy was performed by injection of absolute alcohol into the space of the spinous process of the C7 vertebra. After 6 weeks, we assessed the chronotropic properties at rest and stress, cardiovascular autonomic modulation, myocardial and peripheral catecholamines, and beta-adrenergic receptors in the myocardium. The treadmill test consisted of an escalated protocol with a velocity increment until the maximal velocity tolerated by the animal was reached. RESULTS: The bilateral group had higher levels of peripheral catecholamines, and consequently, a higher heart rate (HR) and blood pressure levels. This suggests that the activation of a compensatory pathway in this group may have deleterious effects. The BIL group had basal tachycardia immediately before the exercise test and increased tachycardia at peak exercise (p<0.01); the blood pressure had the same pattern (p=0.0365). The variables related to autonomic modulation were not significantly different between groups, with the exception of the high frequency (HF) variable, which showed significant differences in CT vs UNI. There was no significant difference in beta receptor expression between groups. There was a higher concentration of peripheral norepinephrine in the BIL group (p=0.0001), and no significant difference in myocardial norepinephrine (p=0.09). CONCLUSION: These findings suggest that an extra cardiac compensatory pathway increases the sympathetic tonus and maintains a higher HR and higher levels of peripheral catecholamines in the procedure groups. The increase in HF activity can be interpreted as an attempt to increase the parasympathetic tonus to balance the greater sympathetic activity.