Liver stiffness as measured by transient elastography is a predictor of outcomes in patients with chronic heart failure with reduced, mid-range, and recovered left-ventricular ejection fraction.

Background: Transient elastography is a noninvasive method for assessing liver stiffness (LS), which can reflect right-sided filling pressure associated with passive liver congestion in patients with HF. Methods: A prospective, single-center observational study in which LS was measured in consecutive ambulatory patients with heart failure with reduced, mid-range, and recovered left ventricular ejection fraction, between March 2018 and June 2019. Mean follow up was 219 ± 86 days. The primary endpoint was time to first event, which was defined as a composite of cardiovascular death or HF hospitalization. Results: Eighty-five patients were included in the final analysis. Mean age was 62 ± 10 and 68% were male. Mean ejection fraction and median NT-proBNP were, respectively, 38.7 ± 14.3% and 1140 pg/mL (interquartile range 224.3-2810.3). The median LS for the entire population was 6.3 (2.5-41.2) kPa. LS correlated with NT-proBNP (r = 0.46; p < 0.0001), total bilirubin (r = 0.47; p < 0.001), direct bilirubin (r = 0.43; p = 0.0001), gama-glutamyl-transpeptidase (r = 0.54; p < 0.0001), and alkaline phosphatase (r = 0.39; p = 0.0004). A Receiver Operating Characteristic (ROC) curve was performed and a cut point of 5.9 kPa showed sensitivity of 80% and specificity of 64.1% with area under the curve of 0.73. Using Cox proportional hazard model (independent variables: LS as a continuous variable, age, gender, NT-proBNP, LVEF, and creatinine), only LS was independently associated with the primary endpoint (hazard ratio 1.05, 95% confidence interval 1.01-1.09; for each increment of one unit of LS). Conclusion: LS correlates with biomarkers of myocardial stretch and several liver function tests and is an independent predictor of outcomes in ambulatory patients with HF.

Complete Atrioventricular Block and Cardiopulmonary Involvement in Rapidly Progressive Systemic Sclerosis

Abstract The heart and lung are target organs in systemic sclerosis (SSc) and similar symptoms (dyspnea and cough) may make the differential diagnosis between the two lesions difficult. In addition, complete atrioventricular block (CAVB) is a rare complication of this disease. This case report is about a patient with SSc and pulmonary fibrosis who was admitted to the emergency room with CAVB, heart failure (HF) and progressive worsening of the underlying disease.

Distinct mortality profile in systemic sclerosis: a death certificate study in Rio de Janeiro, Brazil (2006-2015) using a multiple causes of death analysis.

The objective of this study was to assess the mortality profile related to SSc in the state of Rio de Janeiro, Brazil. We retrospectively examined all registered deaths in the region (2006-2015 period) in which the diagnosis of SSc was mentioned on any line of the death certificates (underlying cause of death [UCD], n = 223; non-UCD, n = 151). Besides the analysis of gender, age, and the causes of death, we also compared the mortality from UCDs between individuals whose death causes included SSc (cases) and those whose death causes did not include SSc (deceased controls). For the latter comparison, we used the mortality odds ratio to approximate the cause-specific standardized mortality ratio. We identified 1495 death causes among the 374 SSc cases. The mean age at death of the SSc cases (85% women) was significantly lower than that of the controls (n = 1,294,117) (58.7 vs. 65.5 years, respectively). The main death causes were circulatory system diseases, infections, and respiratory diseases (36%, 34%, and 21% of SSc cases, respectively). Compared to the deceased controls, there were proportionally more deaths among the SSc cases from pulmonary arterial hypertension, lung fibrosis, septicemia, gastrointestinal hemorrhage, other systemic connective tissue diseases, and heart failure (for death age < 50 years). We confirmed the high burden of cardiovascular, respiratory, and infectious causes in this predominantly non-Caucasian sample of SSc patients. Of interest, the percentage of infection-related deaths in our report was about three times higher than that in SSc studies with predominantly Caucasian populations.

Pacientes cirróticos com escore Child-Pugh C apresentam intervalos QT mais longo / Cirrhotic patients with Child-Pugh C have longer QT intervals

Fundamento e objetivos: A cardiomiopatia cirrótica tem sido usada para descrever a disfunção cardíaca crônica em pacientes cirróticos sem doença cardíaca estrutural prévia. Além disso, o prolongamento do intervalo QT é uma das alterações cardíacas mais importantes relacionadas à cirrose. Estudos prévios sugerem que o prolongamento QT está associado com uma taxa de mortalidade mais alta em pacientes cirróticos. O objetivo deste estudo foi analisar intervalos QTs segundo a gravidade da cirrose, medida pela classificação Child-Plugh. Materiais e métodos: Em um estudo transversal, um total de 67 pacientes com cirrose não alcoólica submeteu-se à avaliação clínica e eletrocardiográfica. A gravidade da cirrose foi classificada de acordo com o escore Child-Pugh. O intervalo QT foi medido por um eletrocardiograma de 12 derivações. Resultados: Os intervalos QTs foram mais longos em pacientes no grupo Child-Plugh C que nos grupos Child-Pugh A e B (459 ± 33 vs 436 ± 25 e 428 ± 34 ms, respectivamente, p = 0,004). Houve uma correlação positiva entre o intervalo QT e o escore Child-Pugh em indivíduos com escore Child-Pugh ≥ 7 (r = 0,50; p < 0,05) e intervalos QT ≥ 440 ms (r = 0,46, p < 0,05). Conclusão: O presente estudo mostrou que pacientes com cirrose Child-Plugh C apresentam intervalos QTs mais longos, o que reforçou a relação entre a gravidade da cirrose e achados eletrocardiográficos da cardiomiopatia cirrótica. Além disso, esse resultado foi encontrado em pacientes sem sintomas cardíacos, o que destacou a importância de um método simples e não invasivo, como o eletrocardiograma, para identificar pacientes cirróticos com cardiomiopatia

Background and aims: Cirrhotic cardiomyopathy has been used to describe chronic cardiac dysfunction in cirrhotic patients with no previous structural heart disease. Additionally, QT prolongation is one of the most important cardiac alterations related to cirrhosis. Previous studies suggest that QT prolongation is associated with a higher mortality rate among cirrhotic patients. The aim of this study was to analyze QT intervals according to cirrhosis severity as measured by the Child-Pugh classification. Materials and methods: In a cross-sectional study, a total of 67 patients with nonalcoholic cirrhosis underwent clinical and electrocardiographic evaluation. Cirrhosis severity was classified according to the Child-Pugh score. The QT interval was measured by a 12-lead electrocardiogram. Results: The QT intervals were longer in patients in the Child-Pugh C group than those in the Child-Pugh A and B groups (459 ± 33 vs 436 ± 25 and 428 ± 34 ms, respectively, p=0.004). There was a positive correlation between the QT interval and the Child-Pugh score in individuals with Child-Pugh scores ≥ 7 (r=0.50, p<0.05) and QT intervals ≥ 440 ms (r=0.46, p<0.05). Conclusion: The present study showed longer QT intervals in patients with Child-Pugh C cirrhosis, which reinforced the relationship between the severity of cirrhosis and electrocardiographic findings of cirrhotic cardiomyopathy. Moreover, this finding emerged in patients with no cardiac symptoms, which highlighted the importance of a simple and noninvasive method (ECG) to identify cirrhotic patients with cardiomyopathy

Posterior Cord Syndrome and Trace Elements Deficiency as an Uncommon Presentation of Common Variable Immunodeficiency.

Diarrhea is one of the most common symptoms in common variable immunodeficiency, but neurologic manifestations are rare. We presented a 50-year-old woman with recurrent diarrhea and severe weight loss that developed a posterior cord syndrome. Endoscopy found a duodenal villous blunting, intraepithelial lymphocytosis, and lack of plasma cells and magnetic resonance imaging of the spine was normal. Laboratory assays confirmed common variable immunodeficiency syndrome and showed low levels of trace elements (copper and zinc). Treatment was initiated with parenteral replacement of trace elements and intravenous human immunoglobulin and the patient improved clinically. In conclusion, physicians must be aware that gastrointestinal and neurologic disorders may be related to each other and remember to request trace elements laboratory assessment.

Cirrhotic Cardiomyopathy: Another Case of a Successful Approach to Treatment of Hepatorenal Syndrome.

Hepatorenal syndrome (HRS) is defined as a failure of renal function, potentially reversible, in patients with liver cirrhosis and ascites. Recently, a component of cardiomyopathy associated with HRS was described, but the use of positive inotropic medicine as part of the treatment of the acute phase has not been extensively evaluated. We report a second case in our hospital of a patient with HRS type I without previous heart disease, with secondary hemodynamic decompensation due to liver disease, in which the abnormalities in systolic function by speckle-tracking echocardiography were observed and could be reversed by the use of inotropes. After partial response to current therapies, the patient presented a clinical and laboratorial response with improvement of renal function after infusion of dobutamine. Clinical studies are needed for the therapy approach to HRS taking into account myocardial dysfunction as a major contributing factor for renal dysfunction.

A Rare Case of Ascites due to Peritoneal Amyloidosis.

BACKGROUND: The clinical manifestations of amyloidosis depend on the type of insoluble protein as well as the location of amyloid deposits in tissues or organs. In the gastrointestinal tract, the small intestine is the most common site of amyloid deposits, whereas peritoneal involvement and ascites are rare. CASE REPORT: We report on a case of ascites due to peritoneal amyloidosis. A 65-year-old patient was admitted to our institution due to anasarca and pulmonary congestion, mimicking heart failure. We started the patient on diuretics and vasodilators. Despite improvement in pulmonary congestion and peripheral edema, his ascites was not reduced. Echocardiogram revealed restrictive cardiomyopathy and a speckle-tracking pattern suggestive of cardiac amyloidosis. Subcutaneous and peritoneal biopsies revealed amyloidosis. CONCLUSIONS: Amyloidosis is rare in the peritoneum and is usually asymptomatic. Ascites occurs in only 20% of patients with peritoneal amyloidosis. We searched PubMed using "ascites" and "amyloidosis" and identified only eight case reports of amyloidosis with ascites. Physicians should be particularly careful in heart failure and anasarca cases when ascites is disproportional or not responsive to diuretic treatment. To date, there is no specific treatment for peritoneal amyloidosis.

Cardiomiopatia cirrótica / Cirrhotic cardiomyopathy

A cardiomiopatia cirrótica (CMC) é uma disfunção cardíaca crônica que acomete pacientes cirróticos sem doença cardíaca prévia. Trata-se de uma doença inicialmente assintomática que se manifesta em situações de maior demanda metabólica, devido a menor capacidade cardíaca em aumentar seu inotropismo. O diagnóstico é pautado em alterações eletrocardiográficas e ecocardiográficas. Ainda não há tratamento específico para a CMC, sendo instituídas medicações sintomáticas semelhantes ao tratamento da insuficiência cardíaca. Esta revisão tem por objetivo descrever os aspectos fisiopatológicos, clínicos e diagnósticos da CMC, evidenciando as características clínicas, laboratoriais e eletro e ecocardiográficas no rastreio da disfunção cardíaca nos pacientes cirróticos.

Cirrhotic cardiomyopathy (CCM) is a chronic cardiac dysfunction that affects cirrhotic patients without history of heart disease. It is an initially asymptomatic disease that appears in situations of increased metabolic demand due to lower cardiac capacity to increase inotropism. Diagnosis is based on disorders revealed by electrocardiography and echocardiography. There is no specific treatmentfor CCM. Similar symptomatic medications are established to treat heart failure. This review aims to describe the pathophysiological, clinical and diagnostic aspects of CCM, showing the clinical, laboratory, electrocardiographic and echocardiographic characteristics in assessing cardiac dysfunction in cirrhotic patients.

Pulmonary Disease Secondary to Reflux Mimicking Interstitial Pneumonia in Systemic Sclerosis: Case Report and Literature Review.

Systemic sclerosis is a complex disease due to the variety of clinical presentations, often superimposed on other conditions, related or not to the connective tissue. We report a 43-year-old Brazilian woman with limited systemic sclerosis and pulmonary symptoms secondary to gastroesophageal reflux disease, with a clinical presentation similar to a diffuse interstitial lung disease. Because of the frequency of interstitial lung injury due to systemic sclerosis, this was an important differential diagnosis, which could be excluded after optimized treatment of reflux disease, with clinical and radiological improvement. Clinical management of patients with collagen diseases requires clinician skills to identify the natural history and understand its nuances. This is a common situation in clinical practice, but with a few discussions in international literature.

Lúpus Eritematoso Sistêmico: Revisão das Manifestações Cardiovasculares / Systemic Lupus Erythematosus: Review of Cardiovascular Aspects

Lúpus eritematoso sistêmico (LES) é uma condição autoimune com processo fisiopatológico complexo, no qual sua atividade inflamatória é potencializadora da doença coronariana através de inflamação sistêmica, disfunção endotelial e predisposição à trombose. O acometimento cardiovascular no LES não é critério diagnóstico, sendo considerado somente como dano já estabelecido em longo prazo de doença. O objetivodeste artigo é destacar a importância da visão clínica para a identificação precoce do acometimento cardiovascular no LES. É feita uma análise crítica da abordagem cardiológica no LES, com ênfase nos aspectos clínicos, biomarcadores cardiovasculares e genética e solicitação racional dos exames complementares. Aparticularidade dos pacientes com nefrite lúpica e síndrome do anticorpo antifosfolipídeo também é destacada.A percepção do dano cardíaco subclínico é fundamental para interromper o ciclo de agressão miocárdica e evitar progressão de doença cardíaca.

Systemic lupus erythematosus (SLE) is an autoimmune condition with a complex pathophysiological process in which its inflammatory activity is an enhancer of coronary disease by systemic inflammation, endothelial dysfunction and predisposition to thrombosis. Thecardiovascular involvement in SLE is not a diagnostic criterion and is considered only as damage established in the long-term of the disease. The objective of this study is to highlight the importance of clinical vision for the early identification of cardiovascular involvement in SLE. A critical analysis of the cardiac approach in SLE, with emphasis on clinical aspects, cardiovascular biomarkers and genetics and rational request of additional tests. The particularity of patients with lupus nephritis and antiphospholipid antibodysyndrome is also highlighted. The perception of subclinical cardiac damage is critical for interrupting the cycle of myocardial injury and to avoid progression of heart disease.