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

The natural history of neonatal vesicoureteral

Resumo.Avaliar a evolução e tratamento do refluxo vesico--ureteral congénito (RVU) em recém-nascido ou lactente com diagnóstico pré-natal de anomalia nefro-urológica. Métodos. Estudo de coortes históricas de crianças com o diagnóstico perinatal de RVU, nascidas numa maternidade de apoio perinatal diferenciado da Região Centro do País, entre 1993 e 2002, e posteriormente acompanhadas no hospital pediátrico de referência da mesma Região. Dividimos as crianças em duas coortes, aquelas que efectuaram tratamento cirúrgico e aquelas em que foi decidido manter vigilância. Para cada coorte (se aplicável) avaliámos: sexo, o tipo e grau de RVU, tipo de cirurgia, complicações, frequência e tempo de evolução até à cura espontânea. Na coorte das crianças em vigilância comparámos a proporção de crianças que mantiveram RVU com aquelas em que se verificou cura espontânea, relativamente ao sexo e ao grau de RVU. Resultados. Oitenta e duas crianças cumpriam os critérios de inclusão. O RVU congénito foi mais frequente no sexo masculino (77%) e era maioritariamente unilateral (65%). O tratamento cirúrgico foi efectuado em 35% dos casos, sobretudo nas crianças com RVU bilateral, com unidades refluxivas (UR) de grau elevado – IV ou V - (80% vs. 12% na coorte de crianças em vigilância) e em crianças com lesão/malformação renal (ipsilateral ou contralateral) e/ou assimetria funcional renal (58% vs 24% na coorte de crianças em vigilância). Neste último grupo, verificámos uma frequência de resolução do RVU de 72% (76% no subgrupo que foi acompanhado pelos menos 48 meses). O período entre os 24 e os 36 meses foi aquele em que ocorreu o maior número de resolução de casos. Não encontrámos diferenças significativas entre sexo e grau de RVU no que respeita à cura espontânea do RVU. Conclusões. O refluxo vesico-ureteral tende a resolver-se nos primeiros anos de vida, parecendo a profilaxia e vigilância clínica medidas suficientes e seguras no casos de refluxo de baixo grau e, provavelmente, também nos casos mais graves.Abstract . To evaluate the natural history and management of perinatally diagnosed vesico-ureteral reflux (VUR). Methods. Historic cohort study of infants born at a level III maternity, between 1993 and 2002, who were diagnosed with neonatal vesico-ureteral reflux and who had subsequent follow- up in a level III children’s hospital in the centre region of Portugal. The infants were divided in two cohorts: those who required surgery and those treated with prophylactic antibiotics and non-surgical intervention. For each cohort, gender, grade of VUR, chosen surgery, complications, rate and timing of spontaneous resolution of VUR were evaluated. This rate was analyzed regarding the gender and the grade of VUR, in the group under non-surgical intervention. Results. We found 82 children with neonatal VUR. Most patients were boys (77%) with unilateral VUR (65%). Surgery was performed in 35% of patients, mostly in bilateral V, in high grade (IV or V) VUR (80% vs. 12% in the non-surgical cohort) and in infants with renal lesion, malformation or asymmetric renal function (58% vs. 24% in the non-surgical cohort). We found a 72% rate of VUR spontaneous resolution (76% in the subgroup followed up at least 48 months) in the infants managed with non-surgical the - rapy. The majority of spontaneous resolutions occurred between the ages of 24 and 36 months. In this group, regar - ding gender or grade, we found no significant differences in the resolution of VUR.info:eu-repo/semantics/publishedVersio

Isotropization of Bianchi type models and a new FRW solution in Brans-Dicke theory

Using scaled variables we are able to integrate an equation valid for isotropic and anisotropic Bianchi type I, V, IX models in Brans-Dicke (BD) theory. We analyze known and new solutions for these models in relation with the possibility that anisotropic models asymptotically isotropize, and/or possess inflationary properties. In particular, a new solution of curve ($k\neq0$) Friedmann-Robertson-Walker (FRW) cosmologies in Brans-Dicke theory is analyzed.Comment: 15 pages, 4 postscript figures, to appear in Gen. Rel. Grav., special issue dedicated in honour of Prof. H. Dehne

Care of patients with ST-elevation myocardial infarction: an international analysis of quality indicators in the acute coronary syndrome STEMI Registry of the EURObservational Research Programme and ACVC and EAPCI Associations of the European Society of Cardiology in 11 462 patients

Aims To use quality indicators to study the management of ST-segment elevation myocardial infarction (STEMI) in different regions. Methods and results Prospective cohort study of STEM I within 24 h of symptom onset (11 462 patients, 196 centres, 26 European Society of Cardiology members, and 3 affiliated countries). The median delay between arrival at a percutaneous cardiovascular intervention (PCI) centre and primary PCI was 40 min (interquartile range 20-74) with 65.8% receiving PCI within guideline recommendation of 60 min. A third of patients (33.2%) required transfer from their initial hospital to one that could perform emergency PCI for whom only 27.2% were treated within the quality indicator recommendation of 120 min. Radial access was used in 56.6% of all primary PCI, but with large geographic variation, from 76.4 to 9.1%. Statins were prescribed at discharge to 98.7% of patients, with little geographic variation. Of patients with a history of heart failure or a documented left ventricular ejection fraction <= 40%, 84.0% were discharged on an angiotensin-converting enzyme inhibitor/angiotensin receptor blocker and 88.7% were discharged on beta-blockers. Conclusion Care for STEMI shows wide geographic variation in the receipt of timely primary PCI, and is in contrast with the more uniform delivery of guideline-recommended pharmacotherapies at time of hospital discharge. [GRAPHICS]

Reperfusion therapy for ST elevation acute myocardial infarction 2010/2011: current status in 37 ESC countries

Aims Primary percutaneous coronary intervention (PPCI) is the preferred reperfusion therapy in ST-elevation myocardial infarction (STEMI). We conducted this study to evaluate the contemporary status on the use and type of reperfusion therapy in patients admitted with STEMI in the European Society of Cardiology (ESC) member countries. Methods and results A cross-sectional descriptive study based on aggregated country-level data on the use of reperfusion therapy in patients admitted with STEMI during 2010 or 2011. Thirty-seven ESC countries were able to provide data from existing national or regional registries. In countries where no such registries exist, data were based on best expert estimates. Data were collected on the use of STEMI reperfusion treatment and mortality, the numbers of cardiologists, and the availability of PPCI facilities in each country. Our survey provides a brief data summary of the degree of variation in reperfusion therapy across Europe. The number of PPCI procedures varied between countries, ranging from 23 to 884 per million inhabitants. Primary percutaneous coronary intervention and thrombolysis were the dominant reperfusion strategy in 33 and 4 countries, respectively. The mean population served by a single PPCI centre with a 24-h service 7 days a week ranged from 31 300 inhabitants per centre to 6 533 000 inhabitants per centre. Twenty-seven of the total 37 countries participated in a former survey from 2007, and major increases in PPCI utilization were observed in 13 of these countries. Conclusion Large variations in reperfusion treatment are still present across Europe. Countries in Eastern and Southern Europe reported that a substantial number of STEMI patients are not receiving any reperfusion therapy. Implementation of the best reperfusion therapy as recommended in the guidelines should be encourage

European Society of Cardiology: Cardiovascular Disease Statistics 2019

Aims The 2019 report from the European Society of Cardiology (ESC) Atlas provides a contemporary analysis of cardiovascular disease (CVD) statistics across 56 member countries, with particular emphasis on international inequalities in disease burden and healthcare delivery together with estimates of progress towards meeting 2025 World Health Organization (WHO) non-communicable disease targets. Methods and results In this report, contemporary CVD statistics are presented for member countries of the ESC. The statistics are drawn from the ESC Atlas which is a repository of CVD data from a variety of sources including the WHO, the Institute for Health Metrics and Evaluation, and the World Bank. The Atlas also includes novel ESC sponsored data on human and capital infrastructure and cardiovascular healthcare delivery obtained by annual survey of the national societies of ESC member countries. Across ESC member countries, the prevalence of obesity (body mass index ≥30 kg/m2) and diabetes has increased two- to three-fold during the last 30 years making the WHO 2025 target to halt rises in these risk factors unlikely to be achieved. More encouraging have been variable declines in hypertension, smoking, and alcohol consumption but on current trends only the reduction in smoking from 28% to 21% during the last 20 years appears sufficient for the WHO target to be achieved. The median age-standardized prevalence of major risk factors was higher in middle-income compared with high-income ESC member countries for hypertension {23.8% [interquartile range (IQR) 22.5–23.1%] vs. 15.7% (IQR 14.5–21.1%)}, diabetes [7.7% (IQR 7.1–10.1%) vs. 5.6% (IQR 4.8–7.0%)], and among males smoking [43.8% (IQR 37.4–48.0%) vs. 26.0% (IQR 20.9–31.7%)] although among females smoking was less common in middle-income countries [8.7% (IQR 3.0–10.8) vs. 16.7% (IQR 13.9–19.7%)]. There were associated inequalities in disease burden with disability-adjusted life years per 100 000 people due to CVD over three times as high in middle-income [7160 (IQR 5655–8115)] compared with high-income [2235 (IQR 1896–3602)] countries. Cardiovascular disease mortality was also higher in middle-income countries where it accounted for a greater proportion of potential years of life lost compared with high-income countries in both females (43% vs. 28%) and males (39% vs. 28%). Despite the inequalities in disease burden across ESC member countries, survey data from the National Cardiac Societies of the ESC showed that middle-income member countries remain severely under-resourced compared with high-income countries in terms of cardiological person-power and technological infrastructure. Under-resourcing in middle-income countries is associated with a severe procedural deficit compared with high-income countries in terms of coronary intervention, device implantation and cardiac surgical procedures. Conclusion A seemingly inexorable rise in the prevalence of obesity and diabetes currently provides the greatest challenge to achieving further reductions in CVD burden across ESC member countries. Additional challenges are provided by inequalities in disease burden that now require intensification of policy initiatives in order to reduce population risk and prioritize cardiovascular healthcare delivery, particularly in the middle-income countries of the ESC where need is greatest

Análise e desenvolvimento de uma estrutura monocoque para um veiculo de elevada eficiência energética

Dissertação de Mestrado Integrado em Engenharia Mecânica apresentada à Faculdade de Ciências e Tecnologia da Universidade de Coimbr

A Beirã veio de Comboio

Conta-se neste capítulo a origem da aldeia da Beirã e a sua relação com a instalação da Linha de caminho de ferro na ligação entre Lisboa a Madrid em 1880

Sindrome coronária aguda sem supradesnivelamento do segmento ST – abordagem pré hospitalar

O eletrocardiograma (ECG) de 12 derivações é o exame complementar de diagnóstico inicial de maior relevância num doente que se apresenta com dor torácica aguda e persistente. Se se documentar elevação persistente do segmento-ST, cumprindo critérios diagnósticos de Enfarte Agudo do Miocárdio com supradesnivelamento do segmento-ST, está indicada a terapêutica de reperfusão imediata. Já o diagnóstico de Síndrome coronária aguda sem supradesnivelamento do segmento-ST (SCA-SST) requer um elevado nível de suspeição, com base na apresentação clínica do doente, na presença de fatores de risco e de alterações eletrocardiográficas. Se suspeita clínica de SCA-SST, deverá administrar-se 300 mg de ácido acetilsalicílico, iniciar terapêutica antianginosa e transportar o doente monitorizado até ao Serviço de Urgência para ser submetido a avaliação adicional. Não está recomendada a administração de inibidor P2Y12 ou de anticoagulação em contexto pré-hospitalar, devendo ser reservados para o momento do diagnóstico definitivo de SCA-SST. Caso o doente mantenha dor torácica recorrente ou sinais de instabilidade clínica apesar da terapêutica instituída, deverá repetir-se ECG e, na presença de alterações dinâmicas do segmento-ST, contactar-se o Serviço de Cardiologia da área para internamento e eventual realização de coronariografia emergente.info:eu-repo/semantics/publishedVersio

Fibrilhação auricular - uma abordagem pré-hospitalar “descomplicada”

A Fibrilhação Auricular (FA) é a arritmia crónica mais comum a nível mundial, com uma prevalência que poderá duplicar nas próximas décadas. O seu diagnóstico baseia-se na análise do ECG de 12 derivações e caracteriza-se habitualmente pela ausência de ondas p e pela irregularidade dos intervalos R-R, embora possam existir exceções. Uma das principais e mais nefastas complicações da FA são os eventos tromboembólicos cerebrais ou periféricos, o que sustenta a pertinência de estabelecer o risco trombótico individual de cada doente e iniciar anticoagulação de acordo com o mesmo.info:eu-repo/semantics/publishedVersio