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Introduction: Systemic sclerosis (SSc) is a systemic autoimmune disease involving multiple organs. We present a rare case of SSc in which clinical manifestations of cardiac fibrosis occurred early in the disease course. Case report: We report the case of a 40-year-old Caucasian man, previously diagnosed with SSc, who presented with decompensated heart failure. Transthoracic echocardiography was remarkable for severe right ventricular systolic dysfunction, abnormal ventricular septal motion, severe functional tricuspid regurgitation and normal pulmonary artery systolic pressure. Left ventricular ejection fraction was 45%. Right heart catheterization revealed no signs of pulmonary hypertension. Cardiac magnetic resonance (CMR) showed diffuse myocardial infiltration, later confirmed as myocardial fibrosis by endomyocardial biopsy. Conclusions: Myocardial fibrosis is an important cause of early heart failure in SSc patients and is associated with poor prognosis. Echocardiography and CMR help establish the diagnosis and enable an appropriate therapeutic strategy to be developed in such cases. Resumo: Introdução: A esclerose sistémica (ES) é uma doença autoimune sistémica que pode envolver múltiplos órgãos. Apresentamos um caso raro de ES associada a manifestação clínica de fibrose miocárdica no decurso precoce da evolução da doença. Caso clínico: : Reportamos um caso de um homem de 40 anos, com antecedentes de ES e que se apresenta com um quadro de insuficiência cardíaca descompensada. O ecocardiograma transtorácico revelou disfunção sistólica severa do ventrículo direito, movimento anómalo do septo interventricular, insuficiência tricúspide severa e pressão sistólica da artéria pulmonar normal. A fração da ejeção era de 45%. O cateterismo direito não identificou sinais de hipertensão pulmonar. A ressonância magnética cardíaca (RMC) demonstrou infiltração miocárdica difusa, confirmada com biópsia endomiocárdica como fibrose miocárdica. Conclusões: A fibrose miocárdica é uma causa importante de insuficiência cardíaca na ES e está associada a mau prognóstico. O ecocardiograma e a RMC podem ajudar a estabelecer o diagnóstico e a desenvolver a estratégia terapêutica adequada. Keywords: Heart failure, Systemic sclerosis, Myocardial fibrosis, Palavras-chave: Insuficiência cardíaca, Esclerose sistémica, Fibrose miocárdica

Introduction: Intermediate-high risk pulmonary embolism (IHR-PE) has a poor prognosis, but is under-represented in trials of direct oral anticoagulants (DOACs) in venous thromboembolic disease (VTE). We aimed to assess whether the administration of DOACs was equivalent to the conventional (CONV) treatment of low-molecular weight heparin bridged with warfarin for treating IHR-PE. Methods: We conducted a retrospective cohort study including 59 consecutive patients admitted with IHR-PE and followed for up to three months after discharge. Two groups were created based on the anticoagulant strategy: CONV (n=35) and DOAC (n=24). The efficacy endpoints were death, recurrent PE, estimated pulmonary artery systolic pressure (PASP), right ventricular systolic function (RVSF) at discharge, and length of stay; the safety endpoint was major bleeding. Results: The two groups were similar regarding demographics, PE etiology and markers of clinical severity. There were four in-hospital deaths in the CONV group and none in the DOAC group. No recurrent PE or major bleeding event was recorded in either group. At discharge, neither PASP nor RVSF was different between the groups. Patients in the DOAC group were discharged 1.7 days earlier on average than patients in the CONV group (4.7±2.4 vs. 3.0±1.5 days, p=0.002). Conclusions: The adoption of a DOAC treatment strategy in this real-world cohort of IHR-PE patients was associated with similar efficacy and safety to the CONV approach. The fact that monitoring of anticoagulation effect was unnecessary probably led to the significant reduction in length of stay. Resumo: Introdução: O tromboembolismo pulmonar de risco intermédio-elevado (TEP-IE) condiciona um prognóstico mais agravado, mas se encontra sub-representado nos ensaios dos anticoagulantes orais diretos (ACOd) na doença tromboembólica venosa (DTV). Avaliamos se a administração de ACOd foi equivalente à estratégia terapêutica convencional (CONV) (heparina de baixo peso molecular (HBPM) + varfarina) no tratamento do TEP-IE. Métodos: Fez-se um estudo de coorte retrospetivo com 59 doentes consecutivos internados por TEP-IE, seguidos até três meses após a alta. Criaram-se dois grupos, baseados na estratégia terapêutica anticoagulante: CONV (n = 35) e DOAC (n = 24) (ACOd). Os desfechos de eficácia foram a morte, o TEP recorrente, a pressão sistólica na artéria pulmonar (PSAP), a função ventricular direita (FVD) e a duração do internamento; o desfecho de segurança foi a hemorragia major. Resultados: Os grupos eram comparáveis relativamente aos aspetos demográficos, à etiologia do TEP e aos marcadores de gravidade clínica. Ocorreram quatro mortes intra-hospitalares no grupo CONV e nenhuma no grupo DOAC. Nenhum evento de TEP recorrente ou hemorragia major ocorreu em qualquer dos grupos. À data de alta, quer a PSAP quer a FVD não diferiram entre os dois grupos. A alta ocorreu 1,7 dia mais cedo no grupo DOAC do que no grupo CONV (4,7±2,4 versus 3,0±1,5 dias, p = 0,002). Conclusões: A adoção de uma estratégia de tratamento com ACOd associou-se a um perfil de eficácia e segurança semelhante à abordagem convencional. A ausência da necessidade de monitoração do efeito anticoagulante provavelmente motivou a redução na duração de internamento. Keywords: Anticoagulants, Venous thromboembolism, Real world, Pulmonary embolism, Intermediate-high risk, Warfarin, Palavras-chave: Anticoagulantes, Tromboembolismo venoso, Mundo real, Embolia pulmonar, Risco intermédio a elevado, Varfarina

AbstractBackground:Right ventricular (RV) afterload is an important risk factor for post-heart transplantation (HTx) mortality, and it results from the interaction between pulmonary vascular resistance (PVR) and pulmonary compliance (CPA). Their product, the RC time, is believed to be constant. An exception is observed in pulmonary hypertension because of elevated left ventricular (LV) filling pressures.Objective:Using HTx as a model for chronic lowering of LV filling pressures, our aim was to assess the variations in RV afterload components after transplantation.Methods:We retrospectively studied 159 patients with right heart catheterization before and after HTx. The effect of Htx on hemodynamic variables was assessed.Results:Most of the patients were male (76%), and the mean age was 53 ± 12 years. HTx had a significant effect on the hemodynamics, with normalization of the LV and RV filling pressures and a significant increase in cardiac output and heart rate (HR). The PVR decreased by 56% and CPA increased by 86%. The RC time did not change significantly, instead of increasing secondary to pulmonary wedge pressure (PWP) normalization after HTx as expected. The expected increase in RC time with PWP lowering was offset by the increase in HR (because of autonomic denervation of the heart). This effect was independent from the decrease of PWP.Conclusion:The RC time remained unchanged after HTx, notwithstanding the fact that pulmonary capillary wedge pressure significantly decreased. An increased HR may have an important effect on RC time and RV afterload. Studying these interactions may be of value to the assessment of HTx candidates and explaining early RV failure after HTx.

Resumo: A diabetes mellitus tipo 2 é actualmente reconhecida como um importante factor de risco de morbilidade e mortalidade cardiovascular, particularmente em doentes admitidos com síndromes coronárias agudas (SCA). Apesar dos recentes avanços do diagnóstico, estratificação de risco e tratamento destas entidades clínicas, a sua associação no mesmo doente continua a representar um prognóstico sombrio. O objectivo deste artigo é sistematizar a caracterização e abordagem da diabetes no contexto das SCA, em termos de diagnóstico, estratificação de risco e prognóstico a curto e longo prazo, visando desta forma contribuir para a disseminação das boas práticas clínicas na abordagem da diabetes no âmbito das SCA.A diabetes é importante factor de risco cardiovascular, sendo os diabéticos uma relevante população no contexto das SCA. Estratégias como a revascularização precoce e completa, o recurso à insulinoterapia intensiva aguda, à realização sistemática de PTGO em doentes sem diagnóstico prévio de diabetes e um enfoque particular na identificação e mitigação de comorbilidades e factores de risco, complementados por uma terapêutica antidiabética, antitrombótica e anti-isquémica optimizadas e o recurso frequente à reabilitação cardíaca e à promoção multidisciplinar da compliance terapêutica e de estilos de vida saudáveis são traves mestras de uma estratégia bem sucedida para a optimização do prognóstico das SCA no contexto de diabetes. Abstract: Type 2 diabetes is currently recognized as an important risk factor for cardiovascular morbidity and mortality, particularly in patients admitted with acute coronary syndromes (ACS). The aim of this paper is to characterize, in a sistematic way, how to approach diabetes and ACS, regarding prognosis, risk stratification and short and long-term prognosis, therefore contributing to improve good clinical practices regarding diabetes and ACS.Diabetes is an important crdiovascular risk factor, being diabetics an inportant ACS subpopulation. A comprehensive diabetes and ACS management, including early and complete revascularization, sistematic OGTT peformance in patients lacking a previous diagnosis of diabetes and a particular focus on identifying and decreasing comorbidities and risk factors, as well as in optimizing antidiabetic, antithrombotic and anti-ischemic medication, using cardiac rehabilitation and the multidisciplinary promotion of therapeutic compliance and healthy lifestyles are key factors in a successful strategy to optimize prognosis of ACS in diabetics. Palavras-chave: Síndromes coronárias agudas, Diabetes mellitus, Diagnóstico, Prognóstico, Keywords: Acute coronary syndromes, Diabetes, Diagnosis, Outcome

Renal artery aneurysms are a rare cause of secondary hypertension. Endovascular treatment with a polytetrafluoroethylene (PTFE)-coated stent can exclude aneurysms and treat hypertension. We report the case of a 23-year-old man with hypertension diagnosed three years earlier and in whom renal angiography revealed three aneurysms involving the right renal artery. A covered stent was implanted, resulting in successful exclusion of the aneurysm. Ten months after the procedure the patient is asymptomatic and with normal blood pressure without antihypertensive therapy. Resumo: Os aneurismas da artéria renal representam uma causa rara de hipertensão secundária. O tratamento endovascular com stent revestido por politetrafluoretileno (PTFE) consiste numa abordagem que permite excluir os aneurismas e tratar a hipertensão. Apresentamos o caso de um jovem de 23 anos, com o diagnóstico de hipertensão há três anos e cuja angiografia renal mostrou três aneurismas na dependência da artéria renal direita. Procedeu-se à implantação de um stent revestido por PTFE com a exclusão bem sucedida dos aneurismas. Dez meses após o procedimento o doente está assintomático e com valores normais de tensão arterial sem terapêutica anti hipertensora. Keywords: Renovascular hypertension, Renal artery aneurysm, Endovascular repair, Renal stent, Palavras-chave: Hipertensão renovascular, Aneurisma da artéria renal, Tratamento endovascular, Stent renal

Abstract Background: Right ventricular (RV) afterload is an important risk factor for post-heart transplantation (HTx) mortality, and it results from the interaction between pulmonary vascular resistance (PVR) and pulmonary compliance (CPA). Their product, the RC time, is believed to be constant. An exception is observed in pulmonary hypertension because of elevated left ventricular (LV) filling pressures. Objective: Using HTx as a model for chronic lowering of LV filling pressures, our aim was to assess the variations in RV afterload components after transplantation. Methods: We retrospectively studied 159 patients with right heart catheterization before and after HTx. The effect of Htx on hemodynamic variables was assessed. Results: Most of the patients were male (76%), and the mean age was 53 ± 12 years. HTx had a significant effect on the hemodynamics, with normalization of the LV and RV filling pressures and a significant increase in cardiac output and heart rate (HR). The PVR decreased by 56% and CPA increased by 86%. The RC time did not change significantly, instead of increasing secondary to pulmonary wedge pressure (PWP) normalization after HTx as expected. The expected increase in RC time with PWP lowering was offset by the increase in HR (because of autonomic denervation of the heart). This effect was independent from the decrease of PWP. Conclusion: The RC time remained unchanged after HTx, notwithstanding the fact that pulmonary capillary wedge pressure significantly decreased. An increased HR may have an important effect on RC time and RV afterload. Studying these interactions may be of value to the assessment of HTx candidates and explaining early RV failure after HTx.