The Portuguese Registry of Hypertrophic Cardiomyopathy: Overall results

INTRODUCTION: We report the results of the Portuguese Registry of Hypertrophic Cardiomyopathy, an initiative that reflects the current spectrum of cardiology centers throughout the territory of Portugal. METHODS: A direct invitation to participate was sent to cardiology departments. Baseline and outcome data were collected. RESULTS: A total of 29 centers participated and 1042 patients were recruited. Four centers recruited 49% of the patients, of whom 59% were male, and mean age at diagnosis was 53±16 years. Hypertrophic cardiomyopathy (HCM) was identified as familial in 33%. The major reason for diagnosis was symptoms (53%). HCM was obstructive in 35% of cases and genetic testing was performed in 51%. Invasive septal reduction therapy was offered to 8% (23% of obstructive patients). Most patients (84%) had an estimated five-year risk of sudden death of <6%. Thirteen percent received an implantable cardioverter-defibrillator. After a median follow-up of 3.3 years (interquartile range [P25-P75] 1.3-6.5 years), 31% were asymptomatic. All-cause mortality was 1.19%/year and cardiovascular mortality 0.65%/year. The incidence of heart failure-related death was 0.25%/year, of sudden cardiac death 0.22%/year and of stroke-related death 0.04%/year. Heart failure-related death plus heart transplantation occurred in 0.27%/year and sudden cardiac death plus equivalents occurred in 0.53%/year. CONCLUSIONS: Contemporary HCM in Portugal is characterized by relatively advanced age at diagnosis, and a high proportion of invasive treatment of obstructive forms. Long-term mortality is low; heart failure is the most common cause of death followed by sudden cardiac death. However, the burden of morbidity remains considerable, emphasizing the need for disease-specific treatments that impact the natural history of the disease

Registo Português de Miocardiopatia Hipertrófica : resultados globais

© 2017 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. Todos os direitos reservados.Introduction: We report the results of the Portuguese Registry of Hypertrophic Cardiomyopathy, an initiative that reflects the current spectrum of cardiology centers throughout the territory of Portugal. Methods: A direct invitation to participate was sent to cardiology departments. Baseline and outcome data were collected. Results: A total of 29 centers participated and 1042 patients were recruited. Four centers recruited 49% of the patients, of whom 59% were male, and mean age at diagnosis was 53±16 years. Hypertrophic cardiomyopathy (HCM) was identified as familial in 33%. The major reason for diagnosis was symptoms (53%). HCM was obstructive in 35% of cases and genetic testing was performed in 51%. Invasive septal reduction therapy was offered to 8% (23% of obstructive patients). Most patients (84%) had an estimated five-year risk of sudden death of <6%. Thirteen percent received an implantable cardioverter-defibrillator. After a median follow-up of 3.3 years (interquartile range [P25-P75] 1.3-6.5 years), 31% were asymptomatic. All-cause mortality was 1.19%/year and cardiovascular mortality 0.65%/year. The incidence of heart failure-related death was 0.25%/year, of sudden cardiac death 0.22%/year and of stroke-related death 0.04%/year. Heart failure-related death plus heart transplantation occurred in 0.27%/year and sudden cardiac death plus equivalents occurred in 0.53%/year. Conclusions: Contemporary HCM in Portugal is characterized by relatively advanced age at diagnosis, and a high proportion of invasive treatment of obstructive forms. Long-term mortality is low; heart failure is the most common cause of death followed by sudden cardiac death. However, the burden of morbidity remains considerable, emphasizing the need for diseasespecific treatments that impact the natural history of the disease.Objectivo: Apresentação dos resultados do Registo Português de Miocardiopatia Hipertrófica. Metodologia: Convite direto aos diferentes centros de cardiologia de Portugal, com análise de dados basais e de seguimento. Resultados: Foram 29 os centros participantes e 1042 doentes incluídos. Quatro centros incluíram 49% dos doentes, 59% do sexo masculino, idade média de diagnóstico 53 ± 16 anos. A doença foi considerada familiar em 33% e a presença de sintomas foi a principal causa de diagnóstico (53%). A miocardiopatia hipertrófica foi obstrutiva em 35%. O estudo genético foi efetuado em 51%. Oito por cento dos doentes fizeram terapêutica invasiva de redução septal (23% dos doentes com obstrução). A maioria dos doentes (84%) apresentava um risco estimado de morte súbita aos 5 anos < 6%. Em 13% foi colocado desfibrilhador cardioversor implantável. Após um seguimento de 3,3 anos, intervalo interquartil (P25-P75) 1,3-6,5 anos, 31% estavam assintomáticos. A mortalidade total foi de 1,19%/ano e a cardiovascular de 0,65%/ano. A incidência de morte por insuficiência cardiaca foi de 0,25%/ano, a de morte súbita de 0,22%/ano e a de morte por acidente vascular cerebal de 0,04%/ano. A mortalidade por insuficiência cardíaca e transplante cardíaco foi de 0,27%/ano e a de morte súbita e equivalentes de 0,53%/ano. Conclusões: A miocardiopatia hipertrófica em Portugal apresenta idade de diagnóstico elevada e é frequente o tratamento invasivo de formas obstrutivas. A mortalidade é baixa, a insuficiência cardíaca é a principal causa de morte, seguida pela morte súbita. A doença apresenta elevada morbilidade, realça a necessidade do desenvolvimento de tratamentos específicos com impacto na sua história natural.info:eu-repo/semantics/publishedVersio

Role of risk stratification by SPECT, PET, and hybrid imaging in guiding management of stable patients with ischaemic heart disease: expert panel of the EANM cardiovascular committee and EACVI

Risk stratification has become increasingly important in the management of patients with suspected or known ischaemic heart disease (IHD). Recent guidelines recommend that these patients have their care driven by risk assessment. The purpose of this position statement is to summarize current evidence on the value of cardiac single-photon emission computed tomography, positron emission tomography, and hybrid imaging in risk stratifying asymptomatic or symptomatic patients with suspected IHD, patients with stable disease, patients after coronary revascularization, heart failure patients, and specific patient population. In addition, this position statement evaluates the impact of imaging results on clinical decision-making and thereby its role in patient management. The document represents the opinion of the European Association of Nuclear Medicine (EANM) Cardiovascular Committee and of the European Association of Cardiovascular Imaging (EACVI) and intends to stimulate future research in this fiel

Ultrastructural changes in the Haemonchus contortus cuticle exposed to Acacia mearnsii extract.

Haemonchus contortus é um dos parasitos mais patogênicos para pequenos ruminantes em regiões tropicais e subtropicais em todo o mundo, inclusive no Brasil. O objetivo do trabalho foi analisar alterações ultraestruturais na cutícula de H. contortus após contato com extrato de Acacia mearnsii

EACVI survey on hypertrophic cardiomyopathy

Publisher Copyright: Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2021. For permissions, please email: [email protected]: The European Association of Cardiovascular Imaging (EACVI) Scientific Initiatives Committee performed a global survey to evaluate current practice for the assessment and management of patients with hypertrophic cardiomyopathy (HCM). METHODS AND RESULTS: A total of 213 centres from 38 different countries (87% European) responded to the survey. One hundred twenty-one (57%) centres followed HCM patients in a general cardiology outpatient clinic and 85 (40%) centres in a specialized HCM/cardiomyopathy clinic. While echocardiography was the primary imaging modality, cardiovascular magnetic resonance (CMR) has become an important complementary tool. Cardiac anatomy, left ventricular (LV) systolic, and diastolic function were assessed according to current European guidelines and recommendations. To evaluate LV obstruction, 49% of the centres performed bedside provocation manoeuvres in every patient and 55% of the centres used exercise stress echocardiography. The majority of centres used the 5-year risk assessment of sudden cardiac death (SCD) calculated with the HCM Risk-SCD score. However, 34% of the centres also used extensive non-infarct late gadolinium enhancement on CMR and 27% the presence of LV apical aneurysm to help select patients for primary prevention implantable cardioverter-defibrillator therapy. Ninety-nine percent of the responding centres performed regular imaging follow-up of HCM patients. CONCLUSION: Most centres followed European guidelines and recommendations for the diagnosis and management of patients with HCM. The importance of bedside provocation manoeuvres and exercise stress echocardiography to diagnose LV outflow obstruction requires emphasis. Additional risk markers for SCD are used in many centres and might indicate the need for an update of current European recommendations.publishersversionpublishe

World Heart Federation Cholesterol Roadmap: The Portuguese case

Atherosclerotic cardiovascular disease (ASCVD) remains the major cause of premature death and disability; effective cardiovascular (CV) risk prevention is fundamental. The World Heart Federation (WHF) Cholesterol Roadmap provides a framework for national policy development and aims to achieve ASCVD prevention. At the invitation of the WHF, a group of experts from the Portuguese Society of Cardiology (SPC), addressed the cholesterol burden at nationally and discussed possible strategies to include in a Portuguese cholesterol roadmap. The literature review showed that the cholesterol burden in Portugal is high and especially uncontrolled in those with the highest CV risk. An infographic scorecard was built to include in the WHF collection, for a clear idea about CV risk and cholesterol burden in Portugal, which would also be useful for health policy advocacy. The expert discussion and preventive strategies proposal followed the five pillars of the WHF document: awareness improvement; population-based approaches for CV risk and cholesterol; risk assessment/population screening; system-level approaches; surveillance of cholesterol and ASCVD outcomes. These strategies were debated by all the expert participants, with the goal of creating a national cholesterol roadmap to be used for advocacy and as a guide for CV prevention. Several key recommendations were outlined: include all stakeholders in a multidisciplinary national program; create a structured activities plan to increase awareness in the population; improve the quality of continuous CV health education; increase the interaction between different health professionals and non-health professionals; increment the referral of patients to cardiac rehabilitation; screen cholesterol levels in the general population, especially high-risk groups; promote patient self-care, engage with patients' associations; use specific social networks to spread information widely; create a national database of cholesterol levels with systematic registry of CV events; redefine strategies based on the evaluation of results; create and involve more patients' associations - invert the pyramid order. In conclusion, ASCVD and the cholesterol burden remain a strong global issue in Portugal, requiring the involvement of multiple stakeholders in prevention. The Portuguese cholesterol roadmap can provide some solutions to help urgently mitigate the problem. Population-based approaches to improve awareness and CV risk assessment and surveillance of cholesterol and ASCVD outcomes are key factors in this change. A call to action is clearly needed to fight hypercholesterolemia and ASCVD burden.info:eu-repo/semantics/publishedVersio

Prevalence and predictors of coronary artery disease in patients with a calcium score of zero

The absence of coronary calcification is associated with an excellent prognosis. However, a calcium score of zero does not exclude the presence of coronary artery disease (CAD) or the possibility of future cardiovascular events. Our aim was to study the prevalence and predictors of coronary artery disease in patients with a calcium score of zero. Prospective registry consisted of 3,012 consecutive patients that underwent cardiac CT (dual source CT). Stable patients referred for evaluation of possible CAD that had a calcium score of zero (n = 864) were selected for this analysis. The variables that were statistically significant were included in a multivariable logistic regression model. From 864 patients with a calcium score of zero, 107 (12.4 %) had coronary plaques on the contrast CT (10.8 %, n = 93 with nonobstructive CAD and 1.6 %, n = 14 with obstructive CAD). By logistic regression analysis, the independent predictors of CAD in this population were age >55 years [odds ratio (OR) 1.63 (1.05-2.52)], hypertension [OR 1.64 (1.05-2.56)] and dyslipidemia [OR 1.54 (1.00-2.36)]. In the presence of these 3 variables, the probability of having coronary plaques was 21 %. The absence of coronary artery calcification does not exclude the presence of coron

Coronary computed tomography angiography-adapted Leaman score as a tool to noninvasively quantify total coronary atherosclerotic burden

To describe a coronary computed tomography angiography (CCTA)-adapted Leaman score (CT-LeSc) as a tool to quantify total coronary atherosclerotic burden with information regarding localization, type of plaque and degree of stenosis and to identify clinical predictors of a high coronary atherosclerotic burden as assessed by the CT-LeSc. Single center prospective registry including a total of 772 consecutive patients undergoing CCTA (Dual-source CT) from April 2011 to March 2012. For the purpose of this study, 581 stable patients referred for suspected coronary artery disease (CAD) without previous myocardial infarction or revascularization procedures were included. Pre-test CAD probability was determined using both the Diamond-Forrester extended CAD consortium method (DF-CAD consortium model) and the Morise score. Cardiovascular risk was assessed with the HeartScore. The cut-off for the 3rd tercile (CT-LeSc ≥8.3) was used to define a population with a high coronary atherosclerotic burden. The median CT-LeSc in this population (n = 581, 8,136 coronary segments evaluated; mean age 57.6 ± 11.1; 55.8 % males; 14.6 % with diabetes) was 2.2 (IQR 0-6.8). In patients with CAD (n = 341), the median CT-LeSc was 5.8 (IQR 3.2-9.6). Among patients with nonobstructive CAD, most were classified in the lowest terciles (T1, 43.0 %; T2, 36.1 %), but 20.9 % were in the highest tercile (T3). The majority of the patients with obstructive CAD were classified in T3 (78.2 %), but 21.8 % had a CT-LeSc in lower terciles (T1 or T2). The independent predictors of a high CT-LeSc were: Male sex (OR 1.73; 95 % CI 1.04-2.90) diabetes (OR 2.91; 95 % CI 1.61-5.23), hypertension (OR 2.54; 95 % CI 1.40-4.63), Morise score ≥16 (OR 1.97; 95 % CI 1.06-3.67) and HeartScore ≥5 (OR 2.42; 95 % CI 1.41-4.14). We described a cardiac CT adapted Leaman score as a tool to quantify total (obstructive and nonobstructive) coronary atherosclerotic burden, reflecting the comprehensive information about localization, degree of stenosis and type of plaque provided by CCTA. Male sex, hypertension, diabetes, a HeartScore ≥5 % and a Morise score ≥16 were associated with a high coronary atherosclerotic burden, as assessed by the CT-LeSc. About one fifth of the patients with nonobstructive CAD had a CT-LeSc in the highest tercile, and this could potentially lead to a reclass

resultados globais

Funding Information: Gold: Jaba Recordati, Merck Serono, Sanofi-Genzyme, Shire; Silver: Medinfar, Servier IOi was supported by the Italian Ministry of Health (“LVH in aortic valve disease and HCM: genetic basis, biophysical correlates and viral therapy models” (RF-2013-02356787), and NET-2011-02347173 (“Mechanisms and treatment of coronary microvascular dysfunction in patients with genetic or secondary LVH”); and by Telethon Italy (GGP13162). Publisher Copyright: © 2017 Sociedade Portuguesa de CardiologiaIntroduction: We report the results of the Portuguese Registry of Hypertrophic Cardiomyopathy, an initiative that reflects the current spectrum of cardiology centers throughout the territory of Portugal. Methods: A direct invitation to participate was sent to cardiology departments. Baseline and outcome data were collected. Results: A total of 29 centers participated and 1042 patients were recruited. Four centers recruited 49% of the patients, of whom 59% were male, and mean age at diagnosis was 53±16 years. Hypertrophic cardiomyopathy (HCM) was identified as familial in 33%. The major reason for diagnosis was symptoms (53%). HCM was obstructive in 35% of cases and genetic testing was performed in 51%. Invasive septal reduction therapy was offered to 8% (23% of obstructive patients). Most patients (84%) had an estimated five-year risk of sudden death of <6%. Thirteen percent received an implantable cardioverter-defibrillator. After a median follow-up of 3.3 years (interquartile range [P25-P75] 1.3-6.5 years), 31% were asymptomatic. All-cause mortality was 1.19%/year and cardiovascular mortality 0.65%/year. The incidence of heart failure-related death was 0.25%/year, of sudden cardiac death 0.22%/year and of stroke-related death 0.04%/year. Heart failure-related death plus heart transplantation occurred in 0.27%/year and sudden cardiac death plus equivalents occurred in 0.53%/year. Conclusions: Contemporary HCM in Portugal is characterized by relatively advanced age at diagnosis, and a high proportion of invasive treatment of obstructive forms. Long-term mortality is low; heart failure is the most common cause of death followed by sudden cardiac death. However, the burden of morbidity remains considerable, emphasizing the need for disease-specific treatments that impact the natural history of the disease.publishersversionpublishe