Experience of Hereditary Amyloidosis with Rare Variant in Ecuador: Case Reports.

More than approximately 120 transthyretin mutations are known. Their clinical presentation is heterogeneous, as the course of disease onset depends on genetic variation and level of penetrance. They are little known in Ecuador, and some of the reported cases suggest-given analysis of family trees-that they come from a province that is possibly considered endemic. The main objective of this study is to perform a descriptive observational analysis on the presentation of transthyretin amyloidosis in families carrying the p.Ser43Asn gene of the identified index case.

Biomarkers and prediction of anthracyclic cardiotoxicity in breast cancer.

BACKGROUND: Chemotherapy with doxorubicin may lead to left ventricular dysfunction. There is a controversial recommendation that biomarkers can predict ventricular dysfunction, which is one of the most feared manifestations of anthracycline cardiotoxicity. OBJECTIVE: The aim of this study was to evaluate the behavior of biomarkers such as Troponin I, type B natriuretic peptide, creatine phosphokinase fraction MB, and myoglobin in predicting cardiotoxicity in a cohort of women with breast cancer undergoing chemotherapy with anthracycline. METHODS: This is an observational, prospective, longitudinal, unicentric study, which included 40 women with breast cancer, whose therapeutic proposal included treatment with doxorubicin. The protocol had a clinical follow-up of 12 months. Biomarkers such as Troponin I, type B natriuretic peptide, creatine phosphokinase fraction MB, and myoglobin were measured pre-chemotherapy and after the first, third, fourth, and sixth cycles of chemotherapy. RESULTS: There was a progressive increase in type B natriuretic peptide and myoglobin values in all chemotherapy cycles. Although creatine phosphokinase fraction MB showed a sustained increase, this increase was not statistically significant. Troponin, type B natriuretic peptide, myoglobin, and creatine phosphokinase fraction MB were the cardiotoxicity markers with the earliest changes, with a significant increase after the first chemotherapy session. However, they were not able to predict cardiotoxicity. CONCLUSION: Troponin I, type B natriuretic peptide, myoglobin, and creatine phosphokinase fraction MB are elevated during chemotherapy with doxorubicin, but they were not able to predict cardiotoxicity according to established clinical and echocardiographic criteria. The incidence of subclinical cardiotoxicity resulting from the administration of doxorubicin was 12.5%.

Elastografia hepática na avaliação complementar da insuficiência cardíaca / Liver elastography for complementary assessment of heart failure

A elastografia hepática (EH) avalia as consequências sistêmicas da insuficiência cardíaca (IC). Este método pode auxiliar na avaliação prognóstica dos portadores de IC. A IC pode afetar de forma secundária a função de vários órgãos e sistemas, notadamente o hepático, mediante congestão venosa. O objetivo deste artigo é mostrar, através de uma revisão narrativa, a importância da EH na avaliação complementar da IC. As consequências hepáticas na doença cardíaca, por vezes, se mostram silenciosas, sem grandes alterações no exame físico e/ou em exames laboratoriais. Nesse contexto, a EH demonstrou ser um método não invasivo recomendável para a mensuração do dano hepático causado pela IC. (AU)

Liver elastography (LE) assesses the systemic consequences of heart failure (HF). This method may help in the prognostic assessment of patients with HF. HF can secondarily affect the function of various organs and systems, especially the liver, through venous congestion. The purpose of this article is to provide a narrative review of the importance of LE in the complementary evaluation of HF. The hepatic consequences of cardiac disease are sometimes silent, without significant changes in physical examination and/or laboratory tests. In this context, LE has emerged as a recommended non-invasive method to measure liver damage caused by HF. (AU)

Biomarkers and prediction of anthracyclic cardiotoxicity in breast cancer

SUMMARY BACKGROUND: Chemotherapy with doxorubicin may lead to left ventricular dysfunction. There is a controversial recommendation that biomarkers can predict ventricular dysfunction, which is one of the most feared manifestations of anthracycline cardiotoxicity. OBJECTIVE: The aim of this study was to evaluate the behavior of biomarkers such as Troponin I, type B natriuretic peptide, creatine phosphokinase fraction MB, and myoglobin in predicting cardiotoxicity in a cohort of women with breast cancer undergoing chemotherapy with anthracycline. METHODS: This is an observational, prospective, longitudinal, unicentric study, which included 40 women with breast cancer, whose therapeutic proposal included treatment with doxorubicin. The protocol had a clinical follow-up of 12 months. Biomarkers such as Troponin I, type B natriuretic peptide, creatine phosphokinase fraction MB, and myoglobin were measured pre-chemotherapy and after the first, third, fourth, and sixth cycles of chemotherapy. RESULTS: There was a progressive increase in type B natriuretic peptide and myoglobin values in all chemotherapy cycles. Although creatine phosphokinase fraction MB showed a sustained increase, this increase was not statistically significant. Troponin, type B natriuretic peptide, myoglobin, and creatine phosphokinase fraction MB were the cardiotoxicity markers with the earliest changes, with a significant increase after the first chemotherapy session. However, they were not able to predict cardiotoxicity. CONCLUSION: Troponin I, type B natriuretic peptide, myoglobin, and creatine phosphokinase fraction MB are elevated during chemotherapy with doxorubicin, but they were not able to predict cardiotoxicity according to established clinical and echocardiographic criteria. The incidence of subclinical cardiotoxicity resulting from the administration of doxorubicin was 12.5%.

A Personalized Approach to the Management of Congestion in Acute Heart Failure.

Heart failure (HF) is the common final pathway of several conditions and is characterized by hyperactivation of numerous neurohumoral pathways. Cardiorenal interaction plays an essential role in the progression of the disease, and the use of diuretics is a cornerstone in the treatment of hypervolemic patients, especially in acute decompensated HF (ADHF). The management of congestion is complex and, to avoid misinterpretations and errors, one must understand the interface between the heart and the kidneys in ADHF. Congestion itself may impair renal function and must be treated aggressively. Transitory elevations in serum creatinine during decongestion is not associated with worse outcomes and diuretics should be maintained in patients with clear hypervolemia. Monitoring urinary sodium after diuretic administration seems to improve the response to diuretics as it allows for adjustments in doses and a personalized approach. Adequate assessment of volemia and the introduction and titration of guideline-directed medical therapy are mandatory before discharge. An early visit after discharge is highly recommended, to assess for residual congestion and thus avoid readmissions.

The Impact of Cardiovascular Risk Factors and Renal Disease on Outcomes in Patients Hospitalized with COVID-19: An Observational Study from Two Public Hospitals in Brazil

Abstract Background Cardiovascular risk factors are prognostic factors in coronavirus disease 2019 (COVID-19) and have been scarcely studied in Brazil. Objective The aim of this study was to assess the impact of cardiovascular risk factors on the outcomes of patients admitted for COVID-19. Methods From July 2020 to February 2021, 200 patients from two public hospitals were enrolled. Patients were included if they had typical symptoms or signs of COVID-19, a positive real-time polymerase chain reaction test (RT-PCR) for COVID-19, and an age above 18 years. This is a prospective, observational, and longitudinal study. Data were collected within 24 h of admission. The primary endpoint was a combination of hospital lethality, mechanical ventilation, hemodialysis, or length of hospital stay >28 days. Continuous variables were compared with the Student's t-test for independent samples or the Mann-Whitney test. For comparisons of proportions, the χ 2 test was applied. ROC curves and survival curves were constructed. Multivariate logistic regression was performed to identify independent predictors of events. The level of significance was 0.05. Results There were 98 (49%) events during the hospital course, and 72 (36%) died in the hospital. Patients with a primary endpoint were older and more likely to have a history of hypertension, diabetes, chronic obstructive pulmonary disease (COPD), and chronic kidney disease (CKD). Vital signs at admission associated with events were diastolic blood pressure, respiratory rate, and oxygen saturation in ambient air (O 2 Sat). Serum creatinine >1.37 mg/dL at admission had a sensitivity of 51.6 and a specificity of 82% to predict the primary endpoint, with an area under the curve (AUC) of 0.68. In multivariate analysis, age, diabetes, CKD, and COPD were independent predictors of the primary endpoint. Age and CKD were independent predictors of in-hospital lethality. Conclusion Cardiovascular risk factors, such as diabetes and CKD, were related to a worse prognosis in patients hospitalized with COVID-19 in this sample from two public hospitals in the state of Rio de Janeiro.

Tortuosidade das Artérias Coronárias como um Novo Fenótipo para Isquemia sem Doença Arterial Coronariana / Coronary Tortuosity as a New Phenotype for Ischemia without Coronary Artery Disease

Resumo Fundamento As artérias coronárias tendem a ser mais tortuosas que outras artérias e acompanham os movimentos repetidos de flexão e relaxamento que ocorrem durante o ciclo cardíaco. A Tortuosidade das artérias Coronárias (TCor) causa alterações no fluxo coronariano, com uma redução na pressão de perfusão distal, o que pode levar à isquemia miocárdica. Objetivo Avaliar a associação entre TCor e isquemia miocárdica. Métodos Entre janeiro de 2015 e dezembro de 2017, 57 pacientes com angina e doença arterial coronariana não obstrutiva pela angiografia coronária invasiva (ACI) foram incluídos retrospectivamente. Variáveis angiográficas foram analisadas para avaliar a presença e grau de tortuosidade e correlacionadas com seus respectivos territórios vasculares na cintilografia de perfusão miocárdica com estresse. A TCor foi definida como artérias coronárias com três ou mais curvaturas com ângulos ≤ 90o, medidos durante diástole. Um nível de 5% foi estabelecido como estatisticamente significativo. Um nível de 5% foi definido como estatisticamente significativo. Resultados Um total de 17 homens e 40 mulheres foram incluídos (idade média de 58,3 anos). A TCor foi observada em 16 pacientes (28%) e em 24 das 171 artérias. Observou-se uma associação significativa entre TCor e isquemia na análise por artéria (p<0,0001). O fator angiográfico mais associado com isquemia foi o número de curvaturas em uma artéria epicárdica medido na sístole (p=0,021). Conclusão Este estudo mostrou uma associação da TCor com isquemia miocárdica em pacientes com artérias coronárias não obstruídas e angina. Observou-se uma relação entre número aumentado de curvaturas na artéria coronária medido por angiografia durante sístole e isquemia.

Abstract Background Coronary arteries tend to be more tortuous than other arteries and follow the repeated flexion and relaxation movements that occur during the cardiac cycle. Coronary tortuosity (CorT) leads to changes in coronary flow with a reduction in distal perfusion pressure, which could cause myocardial ischemia. Objective To assess the association between CorT and myocardial ischemia. Methods Between January 2015 and December 2017, 57 patients with angina and nonobstructive coronary artery disease detected by invasive coronary angiography (ICA) were retrospectively enrolled. Angiographic variables were analyzed to assess the presence and degree of tortuosity and correlated with their respective vascular territories on stress myocardial perfusion imaging (MPI). CorT was defined as coronary arteries with three or more bend angles ≤90°, measured during diastole. Statistical significance was determined at the 5% level. Results A total of 17 men and 40 women were enrolled (mean age 58.3 years). CorT was observed in 16 patients (28%) and in 24 of 171 arteries. There was a significant association between CorT and ischemia when analyzed per artery (p<0.0001). The angiographic factor most associated with ischemia was the number of bend angles in an epicardial artery measured at systole (p=0.021). Conclusion This study showed an association of CorT and myocardial ischemia in patients with unobstructed coronary arteries and angina. An increased number of coronary bend angles measured by angiography during systole was related to ischemia.

The MAGGIC risk score in the prediction of death or hospitalization in patients with heart failure: Comparison with natriuretic peptides.

BACKGROUND: The MAGGIC risk score has been validated to predict mortality in patients with heart failure (HF). OBJECTIVES: To assess the score ability to predict hospitalization and death and to compare with natriuretic peptides. METHODS: Ninety-three consecutive patients (mean age 62±10 years) with chronic HF and left ventricular ejection fraction (EF) <50% were studied. The MAGGIC score was applied at baseline and the patients were followed for 219±86 days. MAGGIC score was compared with NT-proBNP in the prediction of events. The primary end point was the time to the first event, which was defined as cardiovascular death or hospitalization for HF. RESULTS: There were 23 (24.7%) events (3 deaths and 20 hospitalizations). The median score in patients with and without events was, respectively, 20 [interquartile range 14.2-22] vs. 15.5 [11/21], p=0.16. A ROC curve was performed and a cutoff point of 12 points showed a sensitivity of 87% and specificity of 37% with an area under the curve of 0.59 (95% CI 0.48-0.69) which was lower than that of NT-proBNP (AUC 0.67; 95% CI 0.56-0.76). The mean event-free survival time for patients above and below this cutpoint was 248.8±13 vs. 290±13.7 days (log rank test with p=0.044). Using the COX proportional hazard model, age (p=0.004), NT-proBNP >1000 pg/mL (p=0.014) and the MAGGIC score (p=0.025) were independently associated with the primary outcome. CONCLUSION: The MAGGIC risk score was an independent predictor of events, including heart failure hospitalization. The addition of biomarkers improved the accuracy of the score.