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Copyright © 2020 Sociedade Portuguesa de Cardiologia. Published by Elsevier España, S.L.U. All rights reserved., © 2020 Sociedade Portuguesa de Cardiologia. Published by Elsevier Espa ̃na, S.L.U. This is an open access article under the CC-BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/)., Introduction and objectives: Cardiovascular disease is the leading cause of death in Portugal and atherosclerosis is the most common underlying pathophysiological process. The aim of this study was to quantify the economic impact of atherosclerosis in Portugal by estimating disease-related costs. Methods: Costs were estimated based on a prevalence approach and following a societal perspective. Three national epidemiological sources were used to estimate the prevalence of the main clinical manifestations of atherosclerosis. The annual costs of atherosclerosis included both direct costs (resource consumption) and indirect costs (impact on population productivity). These costs were estimated for 2016, based on data from the Hospital Morbidity Database, the health care database (SIARS) of the Regional Health Administration of Lisbon and Tagus Valley including real-world data from primary care, the 2014 National Health Interview Survey, and expert opinion. Results: The total cost of atherosclerosis in 2016 reached 1.9 billion euros (58% and 42% of which was direct and indirect costs, respectively). Most of the direct costs were associated with primary care (55%), followed by hospital outpatient care (27%) and hospitalizations (18%). Indirect costs were mainly driven by early exit from the labor force (91%). Conclusions: Atherosclerosis has a major economic impact, being responsible for health expenditure equivalent to 1% of Portuguese gross domestic product and 11% of current health expenditure in 2016.

International audience; Background and aimsThis European Atherosclerosis Society (EAS) Task Force provides practical guidance for combination therapy for elevated low-density lipoprotein cholesterol (LDL-C) and/or triglycerides (TG) in high-risk and very-high-risk patients.MethodsEvidence-based review.ResultsStatin-ezetimibe combination treatment is the first choice for managing elevated LDL-C and should be given upfront in very-high-risk patients with high LDL-C unlikely to reach goal with a statin, and in primary prevention familial hypercholesterolaemia patients. A proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitor may be added if LDL-C levels remain high. In high and very-high-risk patients with mild to moderately elevated TG levels (>2.3 and < 5.6 mmol/L [>200 and < 500 mg/dL) on a statin, treatment with either a fibrate or high-dose omega-3 fatty acids (icosapent ethyl) may be considered, weighing the benefit versus risks. Combination with fenofibrate may be considered for both macro- and microvascular benefits in patients with type 2 diabetes mellitus.ConclusionsThis guidance aims to improve real-world use of guideline-recommended combination lipid modifying treatment.

Introduction and Aims: Atherogenic dyslipidemia is an important contributor to residual cardiovascular (CV) risk, but it is underdiagnosed and undertreated. This study aimed to assess the opinion of Portuguese experts to generate a consensus concerning the diagnosis and treatment of atherogenic dyslipidemia, as well as to contribute toward standardization of clinical practice in this disorder. Methods: The study consisted in the application of a questionnaire to an expert panel, following a modified Delphi methodology. Results: The majority (88.4%) of the proposed items were found to be consensual. The expert panel recognized the importance of the atherogenic dyslipidemia phenotype, the role played by low-density lipoprotein cholesterol and non-high-density lipoprotein cholesterol as risk markers and therapeutic targets, the choice of statins as first-line lipid-lowering drugs, and the value of associating statins with fenofibrate as a means to reduce residual CV risk. However, the role played by triglycerides in CV risk and the therapeutic value of fibrates lacked consensus. Taking into consideration the state of the art and the opinions expressed in this study, the scientific committee developed a treatment algorithm aimed to improve the perception and treatment of atherogenic dyslipidemia. Conclusions: The experts involved in this study were shown to be familiar with the concept and the importance of atherogenic dyslipidemia. The few situations in which a consensus could not be found were mainly related to the interpretation and/or relevance of the available evidence. Resumo: Introdução e objetivos: A dislipidemia aterogénica contribui consideravelmente para o risco residual de doença cardiovascular (DCV); não obstante, esta patologia encontra-se subdiagnosticada e submedicada. O objetivo deste estudo foi analisar a opinião dos especialistas portugueses e gerar um consenso sobre o diagnóstico e o tratamento da dislipidemia aterogénica, bem como promover a uniformização da prática clínica neste contexto. Métodos: Este estudo consistiu na aplicação de um questionário a um painel de peritos seguindo uma metodologia Delphi modificada. Resultados: A maioria dos itens (88,4%) foram consensuais. Os peritos inquiridos mostraram reconhecer a importância da dislipidemia aterogénica, o papel preponderante do colesterol de lipoproteínas de baixa densidade (c-LDL) e do colesterol total excluindo o das lipoproteínas de alta densidade (colesterol não HDL) na indicação do risco cardiovascular e na definição de objetivos terapêuticos, a eleição das estatinas como fármaco antidislipidémico de primeira linha, e a pertinência da sua associação com o fenofibrato para a redução do risco residual. Não obstante, o painel manifestou incerteza no que se refere ao papel dos triglicerídeos e ao valor terapêutico dos fibratos. Tendo como base a evidência disponível na literatura e as opiniões recolhidas neste estudo, a comissão científica elaborou um algoritmo de tratamento com o objetivo de promover a sensibilização e adequação da terapêutica a doentes com dislipidemia aterogénica. Conclusões: Os inquiridos mostraram estar familiarizados com o conceito e com a importância da dislipidemia aterogénica; as poucas situações de ausência de consenso poderão estar relacionadas com uma diferente interpretação e/ou valorização da evidência científica disponível. Keywords: Atherogenic dyslipidemia, Low-density lipoprotein cholesterol, Non-high-density lipoprotein cholesterol, Triglycerides, Fenofibrate, Residual cardiovascular risk, Palavras-chave: Dislipidemiaaterogénica, c-LDL, Colesterol não-HDL, Triglicerídeos, Fenofibrato, Risco cardiovascularresidual

Among the cardiovascular risk factors, cholesterol-rich atherogenic lipoproteins play a central role in the pathogenesis of atherosclerosis. In middle-aged adults, the size of the total atherosclerotic plaque burden is influenced by both the concentration of circulating atherogenic lipoproteins and the total duration of exposure to these lipoproteins. This review describes the evidence supporting a causal link between lifelong elevations in atherogenic lipoproteins and future risk of atherosclerosis; evidence strengthened by recent epidemiological, genetic, and clinical data. By consequence, adolescence and early adulthood are a crucial time for determining later cardiovascular disease risk. Arguments showing that early optimal lipid control leads to improved outcomes will be presented and suggestions put forward for how those most at risk should be identified and managed.

© The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contactjournals.permissions@oup.com, Aims: This paper sought to estimate the burden of disease attributable to atherosclerosis in mainland Portugal in 2016. Methods and results: The burden of atherosclerosis was measured in disability-adjusted life years (DALY) following the latest 2010 Global Burden of Disease (GBD) methodology. DALYs were estimated as the sum of years of life lost (YLL) with years lived with disability (YLD). The following clinical manifestations of atherosclerosis were included: ischemic heart disease (IHD) (including acute myocardial infarction (AMI), stable angina, and ischemic heart failure (IHF)), ischemic cerebrovascular disease (ICVD) and peripheral arterial disease (PAD). YLL were estimated based on all-cause mortality data for the Portuguese population and mortality due to IHD, ICVD and PAD for the year 2016 sourced from national statistics. Standard life expectancy was sourced from the GBD study. YLD corresponded to the product of the number of prevalent cases by an average disability weight (DW) for all possible combinations of disease. Prevalence data for the different clinical manifestations of atherosclerosis were sourced from epidemiological studies. DW were sourced from the published literature. In 2016, 15,123 deaths were attributable to atherosclerosis, which corresponded to 14.3% of overall mortality in mainland Portugal. DALYs totaled 260,943, 75% due to premature death (196,438 YLL) and 25% due to disability (64,505 YLD). Conclusion: Atherosclerosis entails a high disease burden to society. A large part of this burden would be avoidable if evidence-based effective and cost-effective interventions targeting known risk factors, from prevention to treatment, were implemented., This study was funded by Bayer Portugal, Lda. Funding was independent of the study outcomes.

POEMS syndrome is a rare paraneoplastic condition associated with polyneuropathy, organomegaly, monoclonal gammopathy, endocrine and skin changes. We report a case of a man with Castleman disease and monoclonal gammopathy, with a history of chronic diarrhoea and asthenia. Gastrointestinal involvement in POEMS syndrome is not frequently referred to in the literature and its physiopathology is not fully understood. Diagnostic criteria were met during hospitalization but considering the patient’s overall health condition, therapeutic options were limited. Current treatment for POEMS syndrome depends on the management of the underlying plasma cell disorder. This report outlines the importance of a thorough review of systems and a physical examination to allow an attempted diagnosis and appropriate treatment. LEARNING POINTS POEMS syndrome should be suspected in the presence of peripheral polyneuropathy associated with monoclonal gammopathy; diagnostic workup is challenging and delay in treatment is very common. Gastrointestinal involvement is not frequent and initial presentation with chronic diarrhoea is unusual and rarely mentioned in the literature. There is no consensus on the therapeutic approach, which can include autologous transplantation and chemotherapy. Frequently, these patients present fatigue, weight loss and sarcopenia with reduction of performance status, limiting subsequent therapeutic options.

Background and aims Lifestyle has been found to play an important role in the prevention of cardiovascular disease (CVD). There is evidence to suggest that if lifestyle factors are modified successfully, the relative risk of CVD can be reduced. Risk factors for CVD such as high blood pressure, diabetes mellitus and dyslipidaemias can be modified by lifestyle changes. The main aim of this article is to provide best practice approaches and support for healthcare professionals on the role of lifestyle changes in the prevention of CVD. Methods A working group of experts discussed existing guidelines, clinical practice and evidence, and provided their recommendations on ten topics concerning the role of lifestyle in CVD prevention. These topics covered important lifestyle factors as well as tools/approaches to assess or encourage lifestyle changes. Results The group of clinical experts collaborated to provide their opinion on the following topics: one diet versus customised diets; the role of alcohol consumption in a healthy diet; a diet based on nutrient composition or on whole foods; a Mediterranean versus low-fat diet; the role of dietary supplements; physical activity; smoking cessation; the role of a nutritionist/dietitian in a CVD risk multidisciplinary team; tools to be implemented to assess diet, physical activity and smoking status; and the most effective approaches to encourage lifestyle changes. Conclusions The expert working group agreed that lifestyle changes were important in preventing CVD and provided recommendations which complemented current guidance and increased clarity on several topics.

Whipple’s disease is a chronic, rare, multisystemic, infectious entity, described for the first time in 1907. Its aetiological agent is the Gram-negative rod, Tropheryma whipplei, which was isolated for the first time in 2001 from a cardiac valve of a patient with endocarditis. We present the case of a 71-year-old man, who came into the emergency room complaining of anorexia, weakness, abdominal pain and diarrhoea with haematochezia and presented disseminated palpable purpuric lesions, predominantly in the lower limbs. The upper endoscopy showed a duodenal vasculitis and the biopsy of that location revealed aspects suggestive of Whipple’s disease. We started him on antibiotics according to the recent orientations with progressive clinical and analytical improvement, although he developed an immune reconstitution syndrome, which lasted for 2 weeks.

A key strategy in preventing cardiovascular (CV) disease is the reduction of low-density lipoprotein cholesterol (LDL-C). Statins are a crucial therapy for achieving LDL-C reductions, with the highest tolerated dose often prescribed, especially for patients who are at the greatest risk of CV disease. However, statin intolerance, heterogeneous responses to statins and non-adherence make alternative therapies necessary in some cases. Statins can be combined with a multitude of therapies with synergistic mechanisms of action to effectively manage lipid profiles, while improving safety and tolerability profiles. Addition of a cholesterol absorption inhibitor, bile acid sequestrant or fibrate to statin therapy leads to greater numbers of patients achieving and maintaining LDL-C goals. Furthermore, combination therapies can alter the plasma profiles of other molecules involved in hypercholesterolaemia, including triglycerides and high-density lipoprotein cholesterol. An additional strategy is proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibition therapy, for use in patients who are statin intolerant, patients with heterozygous or homozygous familial hypercholesterolaemia, and patients at very high CV risk, as a potential means for achieving large LDL-C reductions and maintaining LDL-C goals. Clinical trials have demonstrated that PCSK9 inhibition therapy is not only effective but can also be combined with statin therapy to ensure greater reductions in LDL-C. Current, ongoing studies are investigating the efficacy of novel therapies, including selective peroxisome proliferator-activated receptor (PPAR) alpha modulators, PCSK9-specific ribonucleic acid (RNA) interference and anti-inflammatory therapies.

Low-density lipoprotein (LDL) cholesterol (LDL-C) is the primary target in cardiovascular (CV) disease prevention and is commonly used in estimating CV risk; however, alternative markers may be needed when LDL-C is not an appropriate marker (e.g. in the presence of low LDL-C levels or elevated triglyceride [TG] levels). Non-high-density lipoprotein cholesterol (non-HDL-C) and apolipoprotein B (apoB) are markers of atherogenic lipoproteins with evidenced associations with CV risk and are, therefore, recommended as secondary targets, appropriate for use in the presence of elevated TG levels. The reported strength of the associations of non-HDL-C and apoB in comparison to LDL-C is conflicting between studies, potentially due to discordance of the markers which can alter their predictive pattern. Although LDL-C levels are commonly managed with statin treatment, a residual risk of CV events still remains, and an abnormal lipid profile can persist. Combination therapy to further reduce LDL-C levels can be beneficial; a statin therapy combined with other LDL-C-lowering therapy further reduced the number of CV events. In addition, targeting other markers, including non-HDL-C, apoB, total cholesterol and TGs may also be beneficial, specifically in patients with low HDL-C and elevated TG levels. More clinical evidence is required before definitive recommendations can be made; however, a statin–fenofibrate combination demonstrated favourable reductions in major CV events in these specific patients.

In the last 2 decades, statin therapy has proved to be the most potent isolated therapy for attenuation of cardiovascular risk. Its frequent use has been appointed as one of the most important elements for the reduction of the cardiovascular mortality in developed countries. However, the recurrent incidence of muscle symptoms in statin users raised the possibility of causal association, leading to disease entity known as statin associated muscle symptoms (SAMS). Mechanistic studies and clinical trials, specifically designed for the study of SAMS allowed a deeper understanding of the natural history and the accurate incidence. This set of information becomes essential to avoid an unnecessary risk of severe forms of SAMS. At the same time, this concrete understanding of SAMS prevents overdiagnosis and an inadequate suspension of one of the most powerful prevention strategies of our times. In this context, this Luso-Latin American Consortium gathered all available information on the subject and presents them in detail in this document as the basis for the identification and management of SAMS.

Yellow Nail Syndrome (YNS) is a rare, and probably misdiagnosed, condition. It must be considered in middle-aged patients with unexplained chronic respiratory manifestations, lymphedema and nail abnormalities. We present two cases of undiagnosed YNS until the current admissions, despite several years of investigation. The authors wish to draw attention to this syndrome, of which diagnosis is clinical and of exclusion. LEARNING POINTS Yellow nail syndrome is characterized by abnormal nails, lymphedema and respiratory manifestation. Diagnosis is clinical and should raise the suspicion of underlying medical conditions. The treatment is symptomatic.

A meeting of European experts in cardiovascular (CV) disease and lipids was convened in Paris, France, on 10 November 2014 to discuss lipid profile, and in particular atherogenic dyslipidaemia (AD), and associated CV risk. Key points that were raised and discussed during the meeting are summarised in this paper, which also accounts for further discussion and agreement on these points by the group of experts. Elevated levels of low-density lipoprotein cholesterol (LDL-c) are commonly associated with a greater CV risk than low LDL-c levels, and are routinely managed with statins. However, even for patients controlled on statins and achieving low LDL-c levels, abnormal lipid profiles observed in some patients (i.e. elevated triglyceride levels, with/without low levels of high-density lipoprotein cholesterol [HDL-c]) have been linked to the presence of a residual CV risk. Therefore, it is recommended that both triglyceride and HDL-c levels be measured, to allow for the overall CV residual risk to be adequately managed. Favourable safety and clinical data support the combination of statins with other lipid-lowering agents, such as fenofibrate. Patients who have elevated triglyceride levels plus low levels of HDL-c are most likely to achieve clinical benefit from fenofibrate-statin combination therapy. In these patients with AD, achieving target non-HDL-c levels should be a key focus of CV risk management, and the use of non-HDL-c was advocated to provide a better measure of CV risk than LDL-c levels.

Primary systemic amyloidosis or AL amyloidosis is a rare condition characterized by extracellular deposits of fibrils composed of fragments of immunoglobulin light chains. Widespread deposition of this amyloid in tissues interferes with their normal function and leads to multiple organ failure. Clinical manifestations are highly variable due to the wide range of organs involved. The heart is affected in 90% of cases; in 30% of these, cardiac dysfunction is the form of presentation and in 50% it is the cause of death. The commonest form of cardiovascular manifestation is congestive heart failure due to restrictive cardiomyopathy caused by extensive interstitial infiltration of amyloid into the myocardium. Occasionally, it can present as angina, due to infiltration of amyloid into the walls of small vessels. The authors describe the case of a patient in whom the disease presented simultaneously with heart failure and effort angina, as well as intermittent claudication.

To study the frequency, distribution and determinants of isolated systolic hypertension (ISH).Cross-sectional study, in two stages. First, consecutive patients of the Portuguese national health system aged 60 or over were selected (1999). The second stage covered people aged up to 60 years (2000).Health Centers--primary care (Regional Health Administration--Lisbon).3228 patients, 1100 male (439 aged up to 60 years and 661 agedor = 60 years) and 2128 female (860 aged up to 60 years and 1268 agedor = 60 years).Categories of hypertension. Risk stratification. Cardiovascular morbidity. Predictors of isolated systolic hypertension.The definition and staging of hypertension changed between 1988 and 1997/99 and as a consequence, there has been, in hypertensives, an increase in ISH frequency from 13% to 44% (2.4 times greater). This form of hypertension is far more frequent than grades 1 (mild hypertension = 22%), 2 (moderate hypertension = 15%) or 3 (severe hypertension = 7%). The proportion of hypertensives with ISH increases from 19% in people aged up to 40, to 30% in the fourth decade, 34% in the fifth decade, 44% in the sixth decade, 51% in the seventh decade, and 57% in those over 80 years of age. In hypertensives aged up to 50, ISH is more frequent in females. However, in those over age 50 it is predominant in males. ISH represents 60% and 37% respectively of untreated and treated hypertensives. Among untreated hypertensives, age was associated with ISH (p0.001; OR = 1.051 and 95% CI 1.037-1.065). In the over-60 subgroup, the following factors were associated: age (p = 0.013; OR = 1.048 and 95% CI 1.010-1.087); gender--male (p = 0.004; OR = 2.578 and 95% CI 1.361-4.881) and height (p = 0.044; OR = 0.966 and 95% CI 0.933-0.999).Isolated systolic hypertension is the most common form of hypertension and the most often untreated. The constitutional characteristics associated with isolated systolic hypertension in the elderly are age, gender (male) and body mass index (height). The extent of the problem justifies more attention to control of systolic blood pressure, both in research (efficacy) and in clinical practice (effectiveness).