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Percutaneous coronary intervention is currently the most common form of revascularization for symptomatic coronary artery disease. In elderly, diabetic and renal patients, there is an increased prevalence of calcified coronary disease. Rotational atherectomy (RA) can be useful in the treatment of these lesions.Plaque removal was initially proposed as an alternative to balloon angioplasty, hence RA required high-velocity protocols with large-sized burrs (over 2.0 mm). With a high incidence of acute complications and disappointing restenosis rates, the use of RA dwindled. However, the advent of drug-eluting stents, which significantly decreased the rate of restenosis, led to the repositioning of RA as an adjunctive technique in the preparation of densely calcified lesions, improving stent delivery and expansion.In recent years, a better understanding of the mechanism of action of RA has changed it from a plaque debulking to a compliance modifying technique. As a result, RA has become less aggressive, using smaller size burrs and lower rotational speeds. This conservative approach has improved immediate results, with increased safety and better long-term outcomes.In this review paper, the technique of RA is explained in the light of current knowledge. Resumo: A angioplastia coronária tornou-se a forma mais habitual de revascularização miocárdica na doença coronária sintomática. Na população mais idosa, nos diabéticos e nos doentes com insuficiência renal crónica, a prevalência de lesões calcificadas tende a aumentar. A aterectomia rotacional (AR) é uma técnica de grande utilidade no tratamento destas situações.Tendo-se considerado inicialmente que a erosão da placa podia constituir uma alternativa à angioplastia de balão, a técnica de aterectomia rotacional utilizava protocolos de alta velocidade com olivas que ultrapassavam os 2,0 mm de diâmetro. A ocorrência de complicações agudas e resultados insuficientes a longo prazo limitaram a sua difusão. O aparecimento dos stents com fármaco com redução significativa das taxas de reestenose, levou ao reposicionamento da aterectomia rotacional como técnica adjuvante na preparação das lesões permitindo uma melhor entrega e expansão dos stents, no contexto de doença aterosclerótica calcificada.Nos últimos anos, tem-se assistido a uma melhor compreensão do mecanismo de acção da aterectomia rotacional que passou de debulking para modificação de compliance da placa. Consequentemente, a técnica tem-se tornado menos agressiva: utilizam-se olivas de menor calibre, com menor velocidade de rotação, alcançando-se elevadas taxas de sucesso e de segurança no procedimento.Neste artigo procuramos reunir o conhecimento atual sobre como executar a técnica de aterectomia rotacional. Keywords: Rotational atherectomy, Rotational atherectomy technique, Percutaneous coronary intervention, Coronary calcification, Palavras-chave: Aterectomia rotacional, Técnica de aterectomia rotacional, Intervenção coronária percutânea, Calcificação coronária

AIMS: Renal dysfunction is a powerful predictor of adverse outcomes in patients hospitalized for acute coronary syndrome. Three new glomerular filtration rate (GFR) estimating equations recently emerged, based on serum creatinine (CKD-EPIcreat), serum cystatin C (CKD-EPIcyst) or a combination of both (CKD-EPIcreat/cyst), and they are currently recommended to confirm the presence of renal dysfunction. Our aim was to analyse the predictive value of these new estimated GFR (eGFR) equations regarding mid-term mortality in patients with acute coronary syndrome, and compare them with the traditional Modification of Diet in Renal Disease (MDRD-4) formula. METHODS AND RESULTS: 801 patients admitted for acute coronary syndrome (age 67.3±13.3 years, 68.5% male) and followed for 23.6±9.8 months were included. For each equation, patient risk stratification was performed based on eGFR values: high-risk group (eGFR

Introduction and Objectives: Intravenous loop diuretics are an essential part of acute heart failure management; however, data to guide their use is sparse. Our aim was to compare continuous intravenous infusion of loop diuretics with intravenous bolus administration in terms of efficacy and adverse events in patients admitted with severe acute heart failure. Methods: Over a period of three years, 110 patients were admitted to our cardiac intensive care unit with acute heart failure. Clinical, laboratory and prognostic parameters were compared according to the diuretic strategy used and mortality and readmission for acute heart failure during follow-up were analyzed. Results: Previous medical history was similar in the two groups. At admission, the continuous infusion group met criteria for worse prognosis: lower systolic blood pressure (p=0.011), more severe renal injury (p=0.008), lower left ventricular ejection fraction (p=0.016) and higher incidence of restrictive pattern of diastolic dysfunction (p=0.032). They were more often treated with vasopressors (p=0.003), inotropes (p=0.010), renal support therapy (p=0.003) and non-invasive ventilation (p

Introduction and Aim: The prognostic value of late gadolinium enhancement (LGE) for risk stratification of hypertrophic cardiomyopathy (HCM) patients is the subject of disagreement. We set out to examine the association between clinical and morphological variables, risk factors for sudden cardiac death and LGE in HCM patients. Methods: From a population of 78 patients with HCM, we studied 53 who underwent cardiac magnetic resonance. They were divided into two groups according to the presence or absence of LGE. Ventricular arrhythmias and morbidity and mortality during follow-up were analyzed. Results: Patients with LGE were younger at the time of diagnosis (p=0.046) and more often had a family history of sudden death (p=0.008) and known coronary artery disease (p=0.086). On echocardiography they had greater maximum wall thickness (p=0.007) and left atrial area (p=0.037) and volume (p=0.035), and more often presented a restrictive pattern of diastolic dysfunction (p=0.011) with a higher E/E′ ratio (p=0.003) and left ventricular systolic dysfunction (p=0.038). Cardiac magnetic resonance supported the association between LGE and previous echocardiographic findings: greater left atrial area (p=0.029) and maximum wall thickness (p

Resumo: Introdução e objetivos: O valor prognóstico do realce tardio na estratificação dos doentes com miocardiopatia hipertrófica é controverso. Este trabalho pretende avaliar a associação entre a presença de realce tardio na ressonância magnética cardíaca e características clínicas, imagiológicas e prognósticas em doentes com miocardiopatia hipertrófica. Métodos: De 78 doentes com miocardiopatia hipertrófica avaliámos retrospetivamente 53, que realizaram ressonância cardíaca. Os doentes foram divididos em dois grupos, conforme a presença ou ausência de realce tardio. Foi feito seguimento clínico referente a disritmia ventricular e a morbi‐mortalidade. Resultados: Os doentes com realce tardio eram mais jovens à data do diagnóstico (p = 0,046), mais frequentemente tinham antecedentes familiares de morte súbita (p = 0,008) e de doença coronária (p = 0,086). No ecocardiograma apresentavam maior espessura parietal máxima (p = 0,007); área (p = 0,037) e volume indexado da aurícula esquerda (p = 0,035); maior frequência de padrão restritivo de disfunção diastólica (p = 0,011), com relação E/E’ mais elevada (p = 0,003); e disfunção sistólica do ventrículo esquerdo (p = 0,038). A ressonância validou as alterações ecocardiográficas associadas à presença de realce tardio: maior área da aurícula esquerda (p = 0,029); espessura parietal máxima (p

Introduction and Objective: Worsening renal function has an unquestionably negative impact on prognosis in patients with acute heart failure (HF). In Portugal there is little information about the importance of this entity in HF patients admitted to hospital. The objective of this work was to assess the prevalence of cardiorenal syndrome and to identify its key predictors and consequences in patients admitted for acute HF. Methods: This was a retrospective study of 155 patients admitted for acute HF. Cardiorenal syndrome was defined as an increase in serum creatinine of ≥26.5 μmol/l. Clinical, laboratory and echocardiographic parameters were analyzed and compared. Mortality was assessed at 30 and 90 days. Results: Cardiorenal syndrome occurred in 46 patients (29.7%), 5.4±4.4 days after admission; 66.7% (n=24) did not recover baseline creatinine levels. The factors associated with cardiorenal syndrome were older age, chronic renal failure, moderate to severe mitral regurgitation, higher admission blood urea nitrogen, creatinine and troponin I, and lower glomerular filtration rate. Patients who developed cardiorenal syndrome had longer hospital stay, were treated with higher daily doses of intravenous furosemide, and more often required inotropic support and renal replacement therapy. They had higher in‐hospital and 30‐day mortality, and multivariate analysis identified cardiorenal syndrome as an independent predictor of in‐hospital mortality. Conclusions: Renal dysfunction is common in acute HF patients, with a negative impact on prognosis, which highlights the importance of preventing kidney damage through the use of new therapeutic strategies and identification of novel biomarkers. Resumo: Introdução e objetivos: O valor prognóstico da deterioração da função renal é indiscutível nos doentes com insuficiência cardíaca aguda. No contexto nacional sabe‐se pouco acerca do peso relativo da síndrome cardiorrenal no internamento destes doentes. Este trabalho pretende avaliar a prevalência, fatores preditores e consequências desta entidade em doentes internados por insuficiência cardíaca aguda. Métodos: Análise retrospetiva de 155 doentes internados por insuficiência cardíaca aguda. Síndrome cardiorrenal definida como um aumento ≥ a 26,5 umol/L na creatinina sérica relativamente ao valor da admissão. Avaliados e comparados dados clínicos, analíticos e ecocardiográficos. Feito seguimento, referente a mortalidade, aos 30 e 90 dias. Resultados: A síndrome cardiorrenal ocorreu em 46 (29,7%) doentes, 5,4±4,4 dias após a admissão; 66,7% (n=24) não recuperaram a função renal basal. Associaram‐se ao desenvolvimento desta entidade: idades mais avançadas; antecedentes de insuficiência renal crónica; insuficiência mitral moderada/grave; níveis na admissão mais elevados de ureia, creatinina e troponina I; e mais baixos de taxa de filtração glomerular. Os doentes com síndrome cardiorrenal tiveram internamentos mais longos; necessitaram de doses diárias máximas de furosemida mais elevadas; mais frequentemente necessitaram de inotrópicos e de terapêutica de substituição renal. A sua mortalidade no internamento e aos 30 dias foi superior, sendo a síndrome cardiorrenal um fator preditor independente de mortalidade intra‐hospitalar. Conclusões: A disfunção renal é comum em doentes com insuficiência cardíaca aguda, com impacto claramente negativo no prognóstico, devendo a prevenção da lesão renal ser um objetivo primário passando por novas estratégias terapêuticas e identificação de novos biomarcadores. Keywords: Acute heart failure, Cardiorenal syndrome, Prognosis, Palavras‐chave: Insuficiência cardíaca aguda, Síndrome cardiorrenal, Prognóstico

Acute heart failure in patients with severe aortic stenosis and left ventricular systolic dysfunction is well known for its dire prognosis and limited therapeutic options.The authors describe the case of a man admitted for non-ST-elevation myocardial infarction. Diagnostic exams revealed severe aortic stenosis, with good left ventricular systolic function, and two-vessel coronary artery disease. The development of cardiogenic shock with left ventricular systolic dysfunction on day four led to changes in the therapeutic strategy. Percutaneous aortic balloon valvuloplasty coupled with complete myocardial revascularization was performed with a view to future surgical intervention. After discharge, the patient was readmitted with cardiogenic shock after acute pulmonary edema and cardiopulmonary arrest. Ventilator weaning was not possible due to acute heart failure and so it was decided to administer levosimendan, which resulted in substantial clinical and echocardiographic improvement. The patient subsequently underwent successful aortic valve replacement.This case highlights the challenge that characterizes the management of patients with concomitant coronary artery disease, left ventricular systolic dysfunction and severe aortic stenosis. Percutaneous aortic balloon valvuloplasty and levosimendan were safe and effective in the treatment of acute heart failure, acting as a bridge to surgery. Resumo: A insuficiência cardíaca aguda na estenose aórtica grave com compromisso da função sistólica global tem um prognóstico reservado, com opções terapêuticas limitadas.Os autores descrevem o caso clínico de um doente admitido por enfarte agudo do miocárdio sem supradesnivelamento de ST, no qual o estudo complementar revelou estenose aórtica grave, com boa função ventricular esquerda, e doença coronária de 2 vasos. O desenvolvimento de choque cardiogénico, com compromisso grave da função sistólica global, ao quarto dia de internamento, alterou a estratégia terapêutica, optando-se por realizar valvuloplastia aórtica com balão e revascularização miocárdica percutânea completa, com vista a posterior referenciação para cirurgia valvular. Após a alta, apresentou novo quadro de choque cardiogénico em contexto de insuficiência cardíaca aguda e paragem cardiorrespiratória. Por dificuldade na extubação, iniciou perfusão de levosimendan, com franca melhoria clínica e ecocardiográfica. Posteriormente, o doente foi submetido com sucesso a implantação de prótese valvular aórtica.Este caso demonstra o desafio que caracteriza a estabilização e o tratamento destes doentes. A valvuloplastia aórtica com balão e o levosimendan foram eficazes e seguros no tratamento da insuficiência cardíaca aguda e permitiram estabelecer a ponte para a cirurgia. Keywords: Severe aortic stenosis, Cardiogenic shock, Aortic balloon valvuloplasty, Levosimendan, Palavras-chave: Estenose aórtica grave, Choque cardiogénico, Valvuloplastia aórtica com balão, Levosimendan

A new risk stratification scheme incorporating the original CHADS2 score and renal function, entitled R(2)CHADS(2), was validated in the ROCKET-AF and ATRIA study cohorts.Adjusting and validating a modified R-CHA2DS2VASc score as a predictor of ischaemic stroke and all-cause mortality in patients discharged following admission for a Myocardial Infarction (MI).Observational retrospective single-centre cohort study including 1711 patients admitted with MI and discharged alive. We tested the prognostic performance of R-CHA2DS2VASc, based on the original CHA2DS2VASc score with few modifications (addition of renal function parameters [glomerular filtration rate and urea], performance of a revascularization procedure and history of atrial fibrillation). R-CHA2DS2VASc was evaluated for its discriminative performance and calibration in the prediction of ischaemic stroke (primary endpoint), all-cause mortality and a composite endpoint of ischemic stroke plus all-cause mortality (secondary outcomes) during follow-up.R-CHA2DS2VASc score's areas under the curve (AUC) for the occurrence of primary and secondary outcomes were: Ischaemic stroke: AUC 0.717 ± 0.031, p0.001 (vs. 0.681 ± 0.043 for CHA2DS2VASc, p=0.290); all-cause mortality during follow-up: AUC 0.811 ± 0.014, p0.001 (vs. 0.782 ± 0.019 for GRACE, p=0.245); composite endpoint: AUC 0.803 ± 0.014, p0.001. The integrated discrimination improvement index (IDI) and relative IDI for the primary endpoint were 0.015 and 28.2%, respectively, while the IDI and relative IDI for all-cause mortality were 0.13 and 72.1%, suggesting a large improvement in risk stratification. An R-CHA2DS2VASc score below 3 had a negative predictive value of 98.6% for the occurrence of ischaemic stroke.The modified R-CHA2DS2VASc score has shown good calibration and high discriminative performance in the prediction of post-discharge ischaemic stroke and all-cause mortality. The inclusion of renal function in thromboembolic risk predicting schemes seems warranted.

Resumo: Introdução: Na fibrilhação auricular não valvular, 90% dos trombos originam-se no apêndice auricular esquerdo. O seu encerramento percutâneo mostrou ser não inferior à varfarina na profilaxia do tromboembolismo. Objetivo: Avaliar a experiência inicial de um centro no encerramento percutâneo do apêndice auricular esquerdo em doentes com elevado risco tromboembólico sem possibilidade, com contraindicação ou falência da anticoagulação oral. Métodos: Doentes com fibrilhação auricular não valvular, score CHADS2 ≥ 2 sem possibilidade, com contraindicação ou falência da anticoagulação oral foram submetidos a encerramento percutâneo do apêndice auricular esquerdo de acordo com a técnica padrão. Após implantação, foi mantida dupla antiagregação durante um mês e simples indefinidamente. Realizado seguimento com avaliação clínica, ecocardiografia transtorácica e transesofágica. Resultados: O procedimento foi conseguido em 22 dos 23 doentes selecionados (95,7%): 70 ± 9 anos, score CHADS2 de 3,2 ± 0,9 e CHA2DS2-VASC de 4,7 ± 1,4. Apenas no primeiro doente o dispositivo foi substituído por sobredimensionamento. Foram observadas as seguintes complicações periprocedimento: um pseudoaneurisma femoral, 3 hematomas femorais e 2 hemorragias da orofaringe, resolvidos com medidas locais. Durante o seguimento de 12 ± 8 meses foram identificados um fluxo peridispositivo ligeiro e um trombo aderente ao dispositivo - que resolveu sob enoxaparina. A taxa de AVC/AIT foi inferior à esperada com base no score CHADS2 (0 versus 6,7 ± 2,2%). Conclusões: Na nossa experiência inicial este procedimento mostrou ser uma alternativa exequível, segura e eficaz em doentes com fibrilhação auricular para os quais a anticoagulação oral não é uma opção. Foram identificadas complicações de baixa severidade, com uma taxa de AVC/AIT inferior à esperada. Abstract: Introduction: In non-valvular atrial fibrillation 90% of thrombi originate in the left atrial appendage (LAA). Percutaneous LAA closure has been shown to be non-inferior to warfarin for prevention of thromboembolism. Objective: To evaluate the initial experience of a single center in percutaneous LAA closure in patients with high thromboembolic risk and in whom oral anticoagulation was impractical or contraindicated or had failed. Methods: Patients with non-valvular atrial fibrillation and CHADS2 score ≥2 in whom oral anticoagulation was impractical or contraindicated or had failed underwent percutaneous LAA closure according to the standard technique. After the procedure, dual antiplatelet therapy was maintained for one month, followed by single antiplatelet therapy indefinitely. Patients were followed by clinical assessment and transthoracic and transesophageal echocardiography. Results: The procedure was performed in 22 of the 23 selected patients (95.7%), mean age 70±9 years, CHADS2 score 3.2±0.9 and CHA2DS2-VASC score 4.7±1.4. Intraprocedural device replacement was necessary only in the first patient, due to oversizing. The following periprocedural complications were observed: one femoral pseudoaneurysm, three femoral hematomas and two minor oropharyngeal bleeds, resolved by local hemostatic measures. During a 12±8 month follow-up a mild peri-device flow and a thrombus adhering to the device, resolved under with enoxaparin therapy, were identified. The rate of transient ischemic attack (TIA)/stroke was lower than expected according to the CHADS2 score (0 vs. 6.7±2.2%). Conclusions: In our initial experience, this procedure proved to be a feasible, safe and effective alternative for atrial fibrillation patients in whom oral anticoagulation is not an option. Only relatively minor complications were observed, with a lower than expected TIA/stroke rate. Palavras-chave: Fibrilhação auricular, Tromboembolismo, Acidente vascular cerebral, Apêndice auricular esquerdo, Anticoagulação oral, Implante cardíaco, Keywords: Atrial fibrillation, Thromboembolism, Stroke, Left atrial appendage, Oral anticoagulation, Cardiac implant

Background Although most patients with acute pulmonary embolism (PE) remain hospitalized during initial therapy, some may be suitable for partial or complete outpatient management, which may have a significant impact on healthcare costs. Hypothesis This article reviews the state-of-the-art data regarding recognition of very-low-risk PE patients who are potentially eligible for outpatient treatment, along with the safety, management, and cost-effectiveness of this strategy. We propose an algorithm based on collected data that may be useful/practical for identifying patients truly eligible for early discharge. Methods Comprehensive review of scientific data collected from the MEDLINE and Cochrane databases. Studies selected based on potential scientific interest. Qualitative information extracted regarding feasibility, safety, and cost-effectiveness of outpatient treatment, postdischarge management, and selection of truly low-risk patients. Results Early discharge of low-risk patients seems feasible, safe, and particularly cost-effective. Several risk scores have been developed and/or tested as prediction tools for the recognition of low-risk individuals: the Pulmonary Embolism Severity Index (PESI), simplified PESI, Hestia criteria, Geneva score, the Low-Risk Pulmonary Embolism Decision rule, and the Global Registry of Acute Cardiac Events, among others. PESI is the most well-validated model, offering the safest approach at the current time, especially when combined with additional parameters such as troponin I, N-terminal prohormone of brain natriuretic peptide, and echocardiographic markers of right-ventricular dysfunction. Conclusions Recognition of truly low-risk patients entitled to early hospital discharge and outpatient treatment is possible with current risk-stratification schemes along with selected prognostic parameters, and it may have a colossal impact on healthcare costs.

Atrial fibrillation is a common arrhythmia in clinical practice. It is associated with high morbidity and mortality due to its thromboembolic potential, which makes thromboembolic prevention particularly important. Warfarin has been the first-line therapy for this purpose, but it has various limitations and is often contraindicated or underutilized. The fact that thrombi are frequently located in the left atrial appendage in atrial fibrillation led to the development of percutaneous closure for thromboembolic prevention. This article examines the current evidence on percutaneous closure of the left atrial appendage by reviewing the results of the numerous clinical trials on the technique. Resumo: A fibrilhação auricular é uma arritmia frequente na prática clínica. Associa-se à morbilidade e à mortalidade elevadas, em virtude do seu potencial tromboembólico, o que confere especial relevância à profilaxia do tromboembolismo. Para este efeito, a varfarina tem sido a terapêutica de primeira linha, no entanto, associa-se a inúmeras limitações, que a tornam contraindicada ou sub-utilizada. A localização frequente no apêndice auricular esquerdo dos trombos formados na fibrilhação auricular conduziu a que o seu encerramento percutâneo fosse desenvolvido para profilaxia do tromboembolismo. Este artigo pretende fazer um enquadramento da evidência atual para o encerramento percutâneo do apêndice auricular esquerdo, através de uma revisão e atualização dos resultados dos inúmeros estudos realizados até ao momento. Keywords: Atrial fibrillation, Thromboembolism, Stroke, Left atrial appendage, Anticoagulation, Cardiac implant, Palavras-chave: Fibrilhação auricular, Tromboembolismo, Acidente vascular cerebral, Apêndice auricular esquerdo, Anticoagulação oral, Implante cardíaco

Resumo: A fibrilhação auricular é uma arritmia frequente na prática clínica. Associa-se a morbilidade e a mortalidade elevadas, em virtude do seu potencial tromboembólico, o que confere especial relevância à profilaxia do tromboembolismo. Para este efeito, a varfarina tem sido a terapêutica de primeira linha, no entanto, associa-se a inúmeras limitações, que a tornam contraindicada ou sub-utilizada. A localização frequente no apêndice auricular esquerdo dos trombos formados na fibrilhação auricular conduziu a que o seu encerramento percutâneo fosse desenvolvido para profilaxia do tromboembolismo. Este artigo pretende fazer um enquadramento da evidência atual para o encerramento percutâneo do apêndice auricular esquerdo, através de uma revisão e atualização dos resultados dos inúmeros estudos realizados até ao momento. Abstract: Atrial fibrillation is a common arrhythmia in clinical practice. It is associated with high morbidity and mortality due to its thromboembolic potential, which makes thromboembolic prevention particularly important. Warfarin has been the first-line therapy for this purpose, but it has various limitations and is often contraindicated or underutilized. The fact that thrombi are frequently located in the left atrial appendage in atrial fibrillation led to the development of percutaneous closure for thromboembolic prevention. This article examines the current evidence on percutaneous closure of the left atrial appendage by reviewing the results of the numerous clinical trials on the technique. Palavras-chave: Fibrilhação auricular, Tromboembolismo, Acidente vascular cerebral, Apêndice auricular esquerdo, Anticoagulação oral, Implante cardíaco, Keywords: Atrial fibrillation, Thromboembolism, Stroke, Left atrial appendage, Anticoagulation, Cardiac implant

On admission, he was hypotensive (85/55 mmHg) and tachypneic (40 bpm); cardiac auscultation revealed arrhythmia, with no murmurs; pulmonary auscultation revealed decreased breath sounds in both bases, and fine crackles in both bases; peripheral edema was also present. ECG showed AF with controlled ventricular response, alterations suggestive of ST-segment elevation in DI and aVL, T-wave inversion in inferior wall, straight ST-T segment in V3 V6, with no dynamic changes in serial electrocardiograms. Chest radiography revealed increased cardiothoracic ratio and effacement of the left costophrenic angle. Blood gas showed hypoxemia with metabolic alkalosis. Laboratory tests showed renal failure (BUN 14.8 mmol/L; creatinine 155 umol/L); hypokalemia (K 2.9 mmol/L); troponin I 0.66 ng/mL; NT-proBNP 4,060 pg/mL; and C-reactive protein 7.6 mg/dL.

Resumo: Os autores apresentam um caso de amiloidose sistémica com envolvimento cardíaco e discutem a importância de um elevado índice de suspeição para o diagnóstico, os meios de diagnóstico e as opções terapêuticas à luz do conhecimento atual. Homem de 78 anos, com antecedentes de doença renal crónica e fibrilhação auricular, admitido por insuficiência cardíaca aguda de etiologia desconhecida. O ecocardiograma transtorácico mostrou ventrículo esquerdo não dilatado, com compromisso severo da função sistólica global com overlap fenotípico de miocardiopatia hipertrófica e restritiva. Após estudo complementar alargado, o diagnóstico definitivo de amiloidose foi obtido por biópsia da gordura abdominal. Abstract: The authors present a case of systemic amyloidosis with cardiac involvement. We discuss the need for a high level of suspicion to establish a diagnosis, diagnostic techniques and treatment options. Our patient was a 78‐year‐old man with chronic renal disease and atrial fibrillation admitted with acute decompensated heart failure of unknown cause. The transthoracic echocardiogram revealed severely impaired left ventricular function with phenotypic overlap between hypertrophic and restrictive cardiomyopathy. After an extensive diagnostic workup, which included an abdominal fat pad biopsy, the final diagnosis was amyloidosis. Palavras‐chave: Amiloidose, Miocardiopatia hipertrófica, Miocardiopatia restritiva, Insuficiência cardíaca, Keywords: Amyloidosis, Hypertrophic cardiomyopathy, Restrictive cardiomyopathy, Heart failure

A 72-year-old man with severe lactose intolerance was admitted for non-ST-segment elevation myocardial infarction. The coronary angiogram revealed occlusion of the distal third of the first diagonal artery and several non-significant lesions. The pre-discharge echocardiogram revealed moderate left ventricular systolic dysfunction. Discharged on dual antiplatelet therapy, rosuvastatin, perindopril and carvedilol, he was repeatedly readmitted in the following days for abdominal pain/bloating, diarrhea and nausea despite avoiding food products containing lactose. To date, there has been no comprehensive study on the relationship between lactose intolerance and coronary disease, nor has its impact on therapeutics been appropriately addressed. Intolerance to lactose-containing prescription medicines is an extremely rare phenomenon and few strategies are available to overcome this condition, as it has received little attention from the scientific community. Commercial forms of the lactase enzyme and probiotics can limit symptom severity, but different routes of administration, different brands of the same medicine or completely different medicines may be necessary. Some measures were proposed to our patient and, soon afterwards, he was completely asymptomatic in both gastrointestinal and cardiovascular terms. Resumo: Um indivíduo de 72 anos, sexo masculino, com história de intolerância severa à lactose, foi admitido por enfarte agudo do miocárdio sem supradesnivelamento do segmento ST. A coronariografia revelou oclusão do terço distal da primeira artéria diagonal e múltiplas lesões não significativas, enquanto o ecocardiograma pré-alta revelou disfunção ventricular esquerda de grau moderado. O doente teve alta, medicado com dupla-antiagregação (ácido acetilsalicílico e clopidogrel), rosuvastatina, perindopril e carvedilol, porém foi repetidamente re-admitido nos dias seguintes por quadro de dor abdominal, flatulência, diarreia e náusea, apesar da evicção de produtos alimentares contendo lactose. Até este momento, a relação potencial entre a intolerância à lactose e a doença coronária não foi adequadamente avaliada, e nenhum estudo atentou no impacto daquela condição na qualidade e diversidade da terapêutica cardiovascular necessária em doentes com patologia cardíaca. A intolerância a medicamentos contendo lactose é um fenómeno extremamente raro, sendo também raras as estratégias para ultrapassar esta situação, tendo em conta a escassa atenção que este tema mereceu da comunidade internacional. Formas comerciais da enzima lactase e probióticos podem limitar a severidade dos sintomas, porém habitualmente formas diferentes de administração, diferentes formulações da mesma substância ou mesmo substâncias alternativas são necessárias. Algumas medidas foram propostas ao doente em causa e, uma semana depois, o mesmo apresentava-se completamente assintomático dos pontos de vista gastrointestinal e cardiovascular. Keywords: Lactose intolerance, Cardiovascular drugs, Coronary disease, Myocardial infarction, Ischemic cardiomyopathy, Palavras-chave: Intolerância à lactose, Fármacos cardiovasculares, Doença coronária, Enfarte do miocárdio, Cardiomiopatia isquémica

THROMBOLYSIS IN MYOCARDIAL INFARCTION (TIMI), PLATELET GLYCOPROTEIN IIB/IIIA IN UNSTABLE ANGINA: Receptor Suppression Using Integrilin (PURSUIT) and Global Registry of Acute Coronary Events (GRACE) scores have been developed for risk stratification in myocardial infarction (MI). The latter is the most validated score, yet active research is ongoing for improving prognostication in MI.Derivation and validation of a new model for intrahospital, post-discharge and combined/total all-cause mortality prediction - ACHTUNG-Rule - and comparison with the GRACE algorithm.1091 patients admitted for MI (age 68.4 ± 13.5, 63.2% males, 41.8% acute MI with ST-segment elevation (STEMI)) and followed for 19.7 ± 6.4 months were assigned to a derivation sample. 400 patients admitted at a later date at our institution (age 68.3 ± 13.4, 62.7% males, 38.8% STEMI) and followed for a period of 7.2 ± 4.0 months were assigned to a validation sample. Three versions of the ACHTUNG-Rule were developed for the prediction of intrahospital, post-discharge and combined (intrahospital plus post-discharge) all-cause mortality prediction. All models were evaluated for their predictive performance using the area under the receiver operating characteristic (ROC) curve, calibration through the Hosmer-Lemeshow test and predictive utility within each individual patient through the Brier score. Comparison through ROC curve analysis and measures of risk reclassification - net reclassification improvement index (NRI) or Integrated Discrimination Improvement (IDI) - was performed between the ACHTUNG versions for intrahospital, post-discharge and combined mortality prediction and the equivalent GRACE score versions for intrahospital (GRACE-IH), post-discharge (GRACE-6PD) and post-admission 6-month mortality (GRACE-6).Assessment of calibration and overall performance of the ACHTUNG-Rule demonstrated a good fit (p value for the Hosmer-Lemeshow goodness-of-fit test of 0.258, 0.101 and 0.550 for ACHTUNG-IH, ACHTUNG-T and ACHTUNG-R, respectively) and high discriminatory power in the validation cohort for all the primary endpoints (intrahospital mortality: AUC ACHTUNG-IH 0.886 ± 0.035 vs. AUC GRACE-IH 0.906 ± 0.026; post-discharge mortality: AUC ACHTUNG-R 0.827 ± 0.036 vs. AUC GRACE-6PD 0.811 ± 0.034; combined/total mortality: AUC ACHTUNG-T 0.831 ± 0.028 vs. AUC GRACE-6 0.815 ± 0.033). Furthermore, all versions of the ACHTUNG-Rule accurately reclassified a significant number of patients in different, more appropriate, risk categories (NRI ACHTUNG-IH 17.1%, p (2-sided) = 0.0021; NRI ACHTUNG-R 22.0%, p = 0.0002; NRI ACHTUNG-T 18.6%, p = 0.0012). The prognostic performance of the ACHTUNG-Rule was similar in both derivation and validation samples.All versions of the ACHTUNG-Rule have shown excellent discriminative power and good calibration for predicting intrahospital, post-discharge and combined in-hospital plus post-discharge mortality. The ACHTUNG version for intrahospital mortality prediction was not inferior to its equivalent GRACE model, and ACHTUNG versions for post-discharge and combined/total mortality demonstrated apparent superiority. External validation in wider, independent, preferably multicentre, registries is warranted before its potential clinical implementation.

The objective of this study was to derive and test a score that can accurately predict the presence of left atrial or left atrial appendage thrombus (LAAT) in order to identify patients with nonvalvular atrial fibrillation who can be spared transesophageal echocardiogram (TEE) and safely cardioverted. This cross-sectional observational study including 180 individuals (37.2% women) undergoing clinical, echocardiographic and laboratory evaluation (including cardiac troponin I and C reactive protein; CRP) during an atrial fibrillation episode. LAAT was sought on TEE and predictors of this transesophageal echocardiographic finding were assessed. Based on predictors of LAAT (CRP, atrial volume, troponin, episode duration and stroke or embolism) we derived the CATES score and tested its accuracy through receiver operating curve analysis. LAAT was found in 9.4%. CHADS2 and CHA2DS2-VASc had a modest performance in predicting these changes displaying a 0.620 (c-statistic) in average. Using CATES score displayed a higher area under the curve value 0.816 for LAAT. No patients with LAAT were observed in patients with CATES scores ranging from '0' to '2', which corresponded to 49.4% (n = 89) of the sample. We developed a score that presented a very good accuracy for the detection of LAAT in our sample. Further studies in other populations, such as with bigger dimensions, are needed to validate this score and confirm its capability of selecting a very low risk group of patients that can be spared transesophageal echocardiography.

Complete atrioventricular block is a relatively uncommon arrhythmia that is nonetheless increasingly seen in elderly people of developed countries, due to the increase in life expectancy. Congenital and degenerative etiologies are the most commonly seen among young and old patients, respectively. However, scientific literature is surprisingly scarce regarding the etiology of complete atrioventricular block in the asymptomatic otherwise healthy young and middle-aged adult population. Coronary artery disease, autoimmune disorders such as systemic lupus erythematosus or rheumatoid arthritis, history of acute or chronic infectious or hypersensitivity myocarditis, infiltrative processes, hypothyroidism, congenital cardiopathies such as left ventricular noncompaction or Ebstein anomaly, lamin A/C mutations, and pathologic hypervagotony and idiopathic degenerative scleroatrophy of the atrioventricular junctional specialized tissue (Lenegre-Lev disease) are among the most frequent etiologies of complete atrioventricular block in young or middle-aged adults. To our knowledge, no comprehensive review on the specificities of the investigation warranted in this age group has ever been developed, nor have the implications of particular diagnoses on treatment modalities been appropriately addressed. We aim at reviewing the most frequent differential diagnoses of advanced atrioventricular block in otherwise healthy asymptomatic or mildly symptomatic young or middle-aged adults and their impact on therapeutic options. Additionally, we suggest a diagnostic algorithm that may be helpful in this group of patients.

Introduction: There is disagreement regarding the best method for assessing renal dysfunction in patients with myocardial infarction (MI). This study aims to compare two commonly used formulas for measuring glomerular filtration rate (GFR) (Cockcroft–Gault [CG] and Modification of Diet in Renal Disease [MDRD]) in terms of predicting extent of coronary artery disease (CAD) and short- and long-term cardiovascular risk. Methods: We studied 452 patients admitted to a cardiac intensive care unit (ICU) with MI (age 69.01 ± 13.64 years; 61.7% male, 38.5% diabetic) and followed for two years. CG and MDRD GFR estimates were compared in terms of prediction of CAD extent, in-hospital mortality risk and cardiovascular risk during follow-up. Results: GFR

Hypertension complicates 6–8% of pregnancies and includes the following four conditions: hypertension preceding pregnancy or documented before the 20th week of gestation; pre-eclampsia (PE)/eclampsia; chronic hypertension with superimposed pre-eclampsia; and gestational hypertension. The latter is defined as a significant rise in blood pressure after the 20th week of pregnancy in previously normotensive women, to over 140/90 mmHg. When blood pressure remains above 160/110 mmHg, it is considered severe. PE is defined as the presence of proteinuria (≥300 mg/24 hours) in pregnant women with hypertension. The hypertensive syndromes of pregnancy are among the leading causes of maternal and fetal morbidity and mortality and anti-hypertensive treatment is part of the therapeutic arsenal used to prevent serious complications. Although the role of utero-placental insufficiency due to deficient migration of trophoblasts to the spiral arteries is universally accepted, the pathophysiology of PE remains largely unknown and is the subject of debate. No effective ways of predicting or preventing PE have been found, which highlights the need for further research in this field. This review aims primarily to evaluate recent advances in our understanding of the pathophysiology of gestational hypertension and especially PE, and new ways of predicting PE. Additionally, we present a brief review on the diagnosis, prevention and treatment of PE. Resumo: A Hipertensão Arterial (HTA) na gravidez complica 6 a 8% das gestações e inclui 4 principais formas de apresentação: HTA crónica, que antecede a gravidez ou é documentada antes das 20 semanas de gestação, pré-eclampsia (PE)/eclampsia, HTA crónica com PE sobreposta e HTA gestacional. A HTA gestacional define-se como uma elevação significativa da pressão arterial após as 20 semanas de gestação em gestantes previamente normotensas, atingindo valores superiores a 140/90 mmHg. Quando os valores de pressão arterial se mantêm acima de 160/110 mmHg de forma sustentada, é considerada grave. A pré-eclampsia (PE) define-se pela presença de proteinúria (≥300 mg/24 horas) em gestante com HTA. As síndromes hipertensivas da gravidez encontram-se entre as principais causas de morbimortalidade materno-fetal, sendo que a terapêutica anti-hipertensora faz parte do arsenal terapêutico utilizado na prevenção das suas graves complicações. Apesar do papel da insuficiência útero-placentária por deficiente migração dos trofoblastos para as artérias espiraladas ser universalmente aceite, a fisiopatologia da PE permanece ainda parcialmente uma incógnita e reúne alguma controvérsia. Historicamente, não são conhecidas formas suficientemente eficazes de predição e prevenção da PE, pelo que a investigação nesta área assume particular importância. Esta revisão visa primariamente avaliar os avanços científicos mais recentes nas áreas da fisiopatologia da HTA gestacional e, em particular, da PE e das novas formas de predição desta patologia. Concomitantemente, apresentamos informação sumária recente acerca do diagnóstico, prevenção e tratamento anti-hipertensor na PE. Keywords: Pregnancy-induced hypertension, Pre-eclampsia, Prediction, Pathophysiology, Antihypertensive therapy, Palavras-chave: Hipertensão gestacional, Pré-eclampsia, Predição, Fisiopatologia, Terapêutica anti-hipertensora

Introduction: Device closure of interatrial communications has become a well-established technique to treat left-to-right shunt associated with atrial septal defect (ASD) and to prevent paradoxical embolism in patients with patent foramen ovale (PFO). Guidance by transesophageal echocardiography (TEE) is the standard practice but intracardiac echocardiography (ICE) is a feasible and safe alternative for monitoring these procedures. Objectives: To report our experience in the percutaneous closure of ASD and PFO guided by ICE. Methods: We retrospectively reviewed all patients with ASD or PFO who underwent percutaneous closure guided exclusively by ICE between January 2008 and December 2010. All patients were followed clinically with regular echocardiographic evaluation (at discharge, one month, three, six and twelve months) to exclude residual shunt and device malposition. Results: A total of 127 patients (mean age 46.6±12.2 years; 71% female) underwent transcatheter device closure of ASD or PFO during the study period. Device deployment with ICE monitoring was 100% successful, with a low rate of complications and eliminating the need for additional imaging techniques. Conclusions: ICE provides anatomical detail of ASD/PFO and cardiac structures, facilitating congenital cardiac interventional procedures. It eliminates the major drawbacks associated with TEE and enables the interventional cardiologist to control all aspects of the procedure without relying on additional echocardiographic support. Resumo: Introdução: O encerramento percutâneo (EP) de defeitos do septo interauricular (SIA) é uma técnica comprovadamente eficaz para o tratamento da comunicação interauricular (CIA) e prevenção de embolia paradoxal em doentes com foramen ovale patente (FOP). A monitorização com ecocardiograma transesofágico (ETE) é o método habitualmente utilizado mas o ecocardiograma intracardíaco (EIC) constitui uma alternativa prática e segura para o controlo imagiológico destes procedimentos. Objetivo: Apresentar a experiência de um centro no EP de CIA e FOP guiado exclusivamente por EIC. Métodos: Analisámos retrospetivamente uma série de doentes consecutivos com CIA ou FOP submetidos a EP sob controlo por EIC no período de janeiro de 2008 a dezembro de 2010. Foram todos avaliados clínica, eletro e ecocardiograficamente à data da alta e após o 1.°, 3.°, 6.° e 12.° mês para excluir shunt residual (SR) e malaposição do dispositivo. Resultados: Um total de 127 doentes (46,6±12,2 anos; 71% de mulheres) foi submetido a EP de CIA ou FOP durante o referido período. A utilização do EIC durante o EP permitiu a colocação eficaz dos dispositivos em 100% dos casos, com uma reduzida taxa de complicações, dispensando a utilização adicional de outras técnicas de imagem durante os procedimentos. Conclusão: O EIC fornece informação anatómica detalhada da CIA/FOP e estruturas cardíacas adjacentes, facilitando assim os procedimentos de intervenção. Elimina os inconvenientes do ETE e possibilita ao cardiologista de intervenção controlar os vários aspetos do procedimento sem necessitar de apoio ecocardiográfico adicional. Keywords: Intracardiac echocardiography, Atrial septal defects, Transcatheter device closure, Palavras-chave: Ecocardiograma intracardíaco, Defeitos do septo interauricular, Encerramento percutâneo

Resumo: A hipertensão arterial (HTA) na gravidez complica 6 a 8% das gestações e inclui 4 principais formas de apresentação: HTA crónica, que antecede a gravidez ou é documentada antes das 20 semanas de gestação, pré-eclâmpsia (PE) / eclâmpsia, HTA crónica com PE sobreposta e HTA gestacional. A HTA gestacional define-se como uma elevação significativa da pressão arterial após as 20 semanas de gestação em gestantes previamente normotensas, atingindo valores superiores a 140/90 mmHg. Quando os valores de pressão arterial se mantêm acima de 160/110 mmHg de forma sustentada, é considerada grave. A pré-eclâmpsia (PE) define-se pela presença de proteinúria (≥ 300 mg/24 horas) em gestante com HTA. As síndromes hipertensivas da gravidez encontram-se entre as principais causas de morbimortalidade materno-fetal, sendo que a terapêutica anti-hipertensora faz parte do arsenal terapêutico utilizado na prevenção das suas graves complicações. Apesar do papel da insuficiência útero-placentária por deficiente migração dos trofoblastos para as artérias espiraladas ser universalmente aceite, a fisiopatologia da PE permanece ainda parcialmente uma incógnita e reúne alguma controvérsia. Historicamente, não são conhecidas formas suficientemente eficazes de predição e prevenção da PE, pelo que a investigação nesta área assume particular importância. Esta revisão visa primariamente avaliar os avanços científicos mais recentes nas áreas da fisiopatologia da HTA gestacional e, em particular, da PE e das novas formas de predição desta patologia. Concomitantemente, apresentamos informação sumária recente acerca do diagnóstico, prevenção e tratamento anti-hipertensor na PE. Abstract: Hypertension complicates 6-8% of pregnancies and includes the following four conditions: hypertension preceding pregnancy or documented before the 20th week of gestation; pre-eclampsia (PE) / eclampsia; chronic hypertension with superimposed pre-eclampsia; and gestational hypertension. The latter is defined as a significant rise in blood pressure after the 20th week of pregnancy in previously normotensive women, to over 140/90 mmHg. When blood pressure remains above 160/110 mmHg, it is considered severe. PE is defined as the presence of proteinuria (≥300 mg/24 h) in pregnant women with hypertension. The hypertensive syndromes of pregnancy are among the leading causes of maternal and fetal morbidity and mortality and anti-hypertensive treatment is part of the therapeutic arsenal used to prevent serious complications. Although the role of utero-placental insufficiency due to deficient migration of trophoblasts to the spiral arteries is universally accepted, the pathophysiology of PE remains largely unknown and is the subject of debate. No effective ways of predicting or preventing PE have been found, which highlights the need for further research in this field. This review aims primarily to evaluate recent advances in our understanding of the pathophysiology of gestational hypertension and especially PE, and new ways of predicting PE. Additionally, we present a brief review on the diagnosis, prevention and treatment of PE. Palavras-chave: Hipertensão gestacional, Pré-eclâmpsia, Predição, Fisiopatologia, Terapêutica anti-hipertensora, Keywords: Pregnancy-induced hypertension, Pre-eclampsia, Prediction, Pathophysiology, Antihypertensive therapy

Resumo: Introdução: O encerramento percutâneo (EP) de defeitos do septo interauricular (SIA) é uma técnica comprovadamente eficaz para o tratamento da comunicação interauricular (CIA) e prevenção de embolia paradoxal em doentes com foramen ovale patente (FOP). A monitorização com ecocardiograma transesofágico (ETE) é o método habitualmente utilizado mas o ecocardiograma intracardíaco (EIC) constitui uma alternativa prática e segura para o controlo imagiológico destes procedimentos. Objetivo: Apresentar a experiência de um centro no EP de CIA e FOP guiado exclusivamente por EIC. Métodos: Analisámos retrospetivamente uma série de doentes consecutivos com CIA ou FOP submetidos a EP sob controlo por EIC no período de janeiro de 2008 a dezembro de 2010. Foram todos avaliados clínica, eletro e ecocardiograficamente à data da alta e após o 1.°, 3.°, 6.° e 12.° mês para excluir shunt residual (SR) e malaposição do dispositivo. Resultados: Um total de 127 doentes (46,6 ± 12,2 anos; 71% de mulheres) foi submetido a EP de CIA ou FOP durante o referido período. A utilização do EIC durante o EP permitiu a colocação eficaz dos dispositivos em 100% dos casos, com uma reduzida taxa de complicações, dispensando a utilização adicional de outras técnicas de imagem durante os procedimentos. Conclusão: O EIC fornece informação anatómica detalhada da CIA/FOP e estruturas cardíacas adjacentes, facilitando assim os procedimentos de intervenção. Elimina os inconvenientes do ETE e possibilita ao cardiologista de intervenção controlar os vários aspetos do procedimento sem necessitar de apoio ecocardiográfico adicional. Abstract: Introduction: Device closure of interatrial communications has become a well-established technique to treat left-to-right shunt associated with atrial septal defect (ASD) and to prevent paradoxical embolism in patients with patent foramen ovale (PFO). Guidance by transesophageal echocardiography (TEE) is the standard practice but intracardiac echocardiography (ICE) is a feasible and safe alternative for monitoring these procedures. Objectives: To report our experience in the percutaneous closure of ASD and PFO guided by ICE. Methods: We retrospectively reviewed all patients with ASD or PFO who underwent percutaneous closure guided exclusively by ICE between January 2008 and December 2010. All patients were followed clinically with regular echocardiographic evaluation (at discharge, one month, three, six and twelve months) to exclude residual shunt and device malposition. Results: A total of 127 patients (mean age 46.6±12.2 years; 71% female) underwent transcatheter device closure of ASD or PFO during the study period. Device deployment with ICE monitoring was 100% successful, with a low rate of complications and eliminating the need for additional imaging techniques. Conclusions: ICE provides anatomical detail of ASD/PFO and cardiac structures, facilitating congenital cardiac interventional procedures. It eliminates the major drawbacks associated with TEE and enables the interventional cardiologist to control all aspects of the procedure without relying on additional echocardiographic support. Palavras-chave: Ecocardiograma intracardíaco, Defeitos do septo interauricular, Encerramento percutâneo, Keywords: Intracardiac echocardiography, Atrial septal defects, Transcatheter device closure

Introduction: Percutaneous coronary intervention (PCI) of heavily calcified lesions is a challenge for the interventional cardiologist and is associated with a high rate of restenosis and target lesion revascularization (TLR). Adequate lesion preparation by rotational atherectomy followed by drug-eluting stent implantation has shown favorable results. Objective: To report the recent experience of our center with rotational atherectomy (RA) of complex and heavily calcified coronary lesions. Methods: We retrospectively analyzed consecutive patients who underwent PCI with RA in our center between January 2009 and December 2010. A total of 42 patients were included, 65% of whom had been previously refused for coronary artery bypass grafting due to unfavorable coronary anatomy or high surgical risk. RA was performed using the standard Boston Scientific Rotablator® system. The procedure was performed ad-hoc in 50% of patients and transradial access was used in 35%. Data were collected on immediate post-procedural events and major cardiac events during follow-up – cardiovascular death, myocardial infarction, TLR and recurrent angina. Results: Of 1650 PCIs performed in a 23-month period from January 2009, 42 (2.5%) involved RA; from a total of 42 patients (mean age 70.3 ± 10.1 years, 67% male, 55% diabetic), three had left main disease, six had three-vessel disease, 18 had two-vessel disease and the other 15 had single-vessel disease. Of the lesions treated, 71% were >20 mm long and classified in 69% of cases as type C according to the ACC/AHA lesion classification, 4% being chronic total occlusions. The left anterior descending artery was treated in 56% of the procedures. The mean number of burrs used per lesion was 1.3 and a total of 69 stents were implanted, 81% of which were drug-eluting. During follow-up three patients had recurrent angina, one required TLR and two died due to a cardiovascular event. There was significant clinical improvement in 83% of patients. Conclusions: This study demonstrates that rotational atherectomy followed by stenting in heavily calcified lesions can nowadays be performed with high success rates and few complications, extending the possibility of coronary revascularization to a greater number of patients. Resumo: Introdução: A intervenção coronária percutânea (ICP) de lesões gravemente calcificadas constitui um desafio para o cardiologista de intervenção, estando associada a maior taxa de reestenose e necessidade de reintervenção na lesão alvo. A preparação adequada das lesões pela técnica de aterectomia rotacional (AR), seguida da colocação de stent farmacoactivo, tem demonstrado resultados favoráveis. Objectivo: Reportar a experiência recente do nosso centro na utilização de AR para o tratamento de lesões coronárias complexas e calcificadas. Material e Métodos: Analisámos retrospectivamente uma série de doentes consecutivos submetidos a ICP com recurso a AR no período de Janeiro de 2009 a Dezembro de 2010. Foram incluídos 42 doentes (dts), dos quais 65% tinham sido recusados para cirurgia por apresentarem múltiplas comorbilidades ou anatomia coronária desfavorável. A AR foi realizada utilizando o sistema Rotablator® da Boston Scientific. O procedimento foi realizado ad-hoc em 50% dos casos e utilizou-se a via radial em 35% dos dts. Foi avaliada a ocorrência de eventos no pós procedimento imediato e realizado follow-up (FUP) com avaliação de eventos cardiovasculares major após a alta hospitalar – morte cardiovascular (MCV), enfarte agudo do miocárdio, reintervenção na lesão alvo (RLA) e recorrência de angina (RA). Resultados: De um total de 1650 ICP realizadas durante um período de 23 meses desde Janeiro de 2009, 42 (2,5%) envolveram a técnica de AR. Num total de 42 doentes submetidos a AR (70,3 ± 10,1 anos, 67% homens, 55% de diabéticos), três apresentavam doença (DC) de tronco comum (TC), seis dts DC de 3 vasos, 18 dts DC de 2 vasos e 15 dts DC de 1 vaso. As lesões tratadas (71% > 20 mm) classificaram-se como tipo C em 69% e oclusão total crónica em 4%. Intervencionou-se a descendente anterior em 56% dos casos. Utilizou-se uma média de 1,3 olivas por lesão e implantaram-se no total 69 stents, 81% dos quais eram farmacoactivos. Verificou-se RA em três dts, RLA em apenas um dt e MCV em dois dts. Em 83% dos doentes verificou-se melhoria da classe funcional. Conclusão: A utilização da AR na ICP de lesões gravemente calcificadas, seguida de colocação de stent, é um procedimento seguro e com uma elevada taxa de sucesso, permitindo a revascularização coronária a um maior número de doentes. Keywords: Coronary calcification, Percutaneous coronary intervention, Rotational atherectomy, Drug-eluting stents, Palavras-chave: Calcificação coronária, Intervenção coronária percutânea, Aterectomia rotacional, Stents farmacoactivos

Resumo: Introdução: A intervenção coronária percutânea (ICP) de lesões gravemente calcificadas constitui um desafio para o cardiologista de intervenção, estando associada a maior taxa de reestenose e necessidade de reintervenção na lesão alvo. A preparação adequada das lesões pela técnica de aterectomia rotacional (AR), seguida da colocação de stent farmacoativo, tem demonstrado resultados favoráveis. Objectivo: Reportar a experiência recente do nosso centro na utilização de AR para o tratamento de lesões coronárias complexas e calcificadas. Material e métodos: Analisámos retrospetivamente uma série de doentes consecutivos submetidos a ICP com recurso a AR no período de Janeiro de 2009 a Dezembro de 2010. Foram incluídos 42 doentes (dts), dos quais 65% tinham sido recusados para cirurgia por apresentarem múltiplas comorbilidades ou anatomia coronária desfavorável. A AR foi realizada utilizando o sistema Rotablator® da Boston Scientific. O procedimento foi realizado ad-hoc em 50% dos casos e utilizou-se a via radial em 35% dos doentes. Foi avaliada a ocorrência de eventos no pós-procedimento imediato e realizado follow-up (FUP) com avaliação de eventos cardiovasculares major após a alta hospitalar – morte cardiovascular (MCV), enfarte agudo do miocárdio, reintervenção na lesão alvo (RLA) e recorrência de angina (RA). Resultados: De um total de 1650 ICP realizadas durante um período de 23 meses desde Janeiro de 2009, 42 (2,5%) envolveram a técnica de AR. Num total de 42 doentes submetidos a AR (70,3 ± ,1 anos, 67% homens, 55% de diabéticos), 3 apresentavam doença (DC) de tronco comum (TC), 6 dts DC de 3 vasos, 18 dts DC de 2 vasos e 15 dts DC de um vaso. As lesões tratadas (71% > 20 mm) classificaram-se como tipo C em 69% e oclusão total crónica em 4%. Intervencionou-se a descendente anterior em 56% dos casos. Utilizou-se uma média de 1,3 olivas por lesão e implantaram-se no total 69 stents, 81% dos quais eram farmacoativos. Verificou-se RA em 3 dts, RLA em apenas um dt e MCV em 2 dts. Em 83% dos doentes verificou-se melhoria da classe funcional. Conclusão: A utilização da AR na ICP de lesões gravemente calcificadas, seguida de colocação de stent, é um procedimento seguro e com uma elevada taxa de sucesso, permitindo a revascularização coronária a um maior número de doentes. Abstract: Introduction: Percutaneous coronary intervention (PCI) of heavily calcified lesions is a challenge for the interventional cardiologist and is associated with a high rate of restenosis and target lesion revascularization (TLR). Adequate lesion preparation by rotational atherectomy followed by drug-eluting stent implantation has shown favorable results. Objective: To report the recent experience of our center with rotational atherectomy (RA) of complex and heavily calcified coronary lesions. Methods: We retrospectively analyzed consecutive patients who underwent PCI with RA in our center between January 2009 and December 2010. A total of 42 patients were included, 65% of whom had been previously refused for coronary artery bypass grafting due to unfavorable coronary anatomy or high surgical risk. RA was performed using the standard Boston Scientific Rotablator® system. The procedure was performed ad-hoc in 50% of patients and transradial access was used in 35%. Data were collected on immediate post-procedural events and major cardiac events during follow-up – cardiovascular death, myocardial infarction, TLR and recurrent angina. Results: Of 1650 PCIs performed in a 23-month period from January 2009, 42 (2.5%) involved RA, a total of 42 patients (mean age 70.3±10.1 years, 67% male, 55% diabetic), three of whom had left main disease, six had three-vessel disease, 18 had two-vessel disease and the other 15 had single-vessel disease. Of the lesions treated, 71% were >20 mm long and classified in 69% of cases as type C according to the ACC/AHA lesion classification, 4% being chronic total occlusions. The left anterior descending artery was treated in 56% of the procedures. The mean number of burrs used per lesion was 1.3 and a total of 69 stents were implanted, 81% of which were drug-eluting. During follow-up three patients had recurrent angina, one required TLR and two died due to a cardiovascular event. There was significant clinical improvement in 83% of patients. Conclusions: This study demonstrates that rotational atherectomy followed by stenting in heavily calcified lesions can nowadays be performed with high success rates and few complications, extending the possibility of coronary revascularization to a greater number of patients. Palavras-chave: Calcificação coronária, Intervenção coronária percutânea, Aterectomia rotacional, Stents farmacoativos, Keywords: Coronary calcification, Percutaneous coronary intervention, Rotational atherectomy, Drug-eluting stents

Aim Some transoesophageal echocardiogram (TEE) findings are associated with an increased risk of stroke in patients with atrial fibrillation (AF). This study was designed to evaluate and compare the accuracy of CHADS2 and CHA2DS2-VASc in the prediction of these findings and test the additive value of transthoracic echocardiogram (TTE)-derived parameters as a possible refinement for these classifications. Methods and results Cross-sectional study of 405 consecutive patients who underwent TTE and TEE evaluation during AF. Stroke risk assessment was performed using the CHADS2 and CHA2DS2-VASc scores, alone and alongside with the addition of two TTE-derived parameters (left atrium area and left ventricle global systolic function). Comparisons regarding the presence of left atrial appendage thrombi (LAA T), dense spontaneous echo contrast (SEC), and left atrial appendage (LAA) low flow velocities (LFV) were performed using receiver operating characteristic curves. In low-risk patients, as assessed through the CHA2DS2-VASc score and CHADS2 and CHA2DS2-VASc scores plus echo parameters, no high-risk features were found on TEE. In subjects classified as low risk using CHADS2, this figure rose to 10%. No significant differences were found between CHADS2 and CHA2DS2-VASc in the prediction of LAA T, dense SEC, and LAA LFV. The addition of TTE-derived parameters to the previous clinical-risk scores resulted in improved prediction of the TEE endpoints. Conclusion These findings suggest that the use of TTE-derived parameters may be a valuable way of refining the available clinical risk schemes for the detection of surrogate markers of stroke. Follow-up studies using clinical endpoints will be necessary to confirm this hypothesis.

Introduction: Patients with coronary artery disease (CAD) are at increased risk of stroke. The aim of this study was to analyze the prognostic accuracy of selected clinical and laboratory variables in stroke risk prediction following discharge after myocardial infarction (MI). Methods: we analyzed 404 consecutive patients (aged 68.1 ± 13.7 years; 63.4% male; 37.4% with diabetes) without previous stroke who were discharged in sinus rhythm after being admitted for MI. The following data were collected: cardiovascular risk factors, admission blood glucose (BG), HbA1c, creatinine, peak troponin levels; glomerular filtration rate (GFR) by the MDRD formula; maximum Killip class; GRACE score for in-hospital and 6-month mortality; and extent of CAD. Patients were followed for two years and each variable was tested as a possible predictor of cerebrovascular events (stroke or transient ischemic attack [TIA]). Results: During follow-up, 27 patients were admitted for stroke or TIA. The presence of diabetes, hypertension, dyslipidemia and previously known CAD, type of MI (STEMI vs NSTEMI) and extent of CAD did not predict cerebrovascular risk. The following variables were associated with higher stroke risk: GFR 1 (p = 0.025, OR 2.71, 95% CI 1.10-6.69); GRACE in-hospital mortality > 180 (p = 0.001, OR 4.09, 95% CI 1.64-10.22); admission BG > 140 mg/dl (p = 0.001, OR 5.74, 95% CI 1.87-17.58); GRACE 6–month mortality > 150 (p = 0.001, OR 4.50, 95% CI 1.80-6.27); and peak troponin > 42 ng/ml (p = 0.032, OR 2.64, 95% CI 1.06-6.59). Logistic regression analysis produced a model with the predictors GRACE 6-month mortality > 150 (OR 3.26; p = 0.014) and admission BG > 7.7 mmol/l (OR 4.09; p = 0.017) that fitted the data well (Hosmer-Lemeshow: p=0.916). Discussion/Conclusions: In patients with MI, variables known to be predictors of in-hospital mortality, including admission BG, renal function, acute heart failure and GRACE score, were found to be useful predictors of stroke during 2-year follow-up. While both GRACE score for 6-month mortality > 150 and admission BG > 7.7 mmol/l were independent predictors of stroke, CV risk factors, previously known CAD, and extent of CAD assessed by coronary angiography did not improve stroke risk prediction. This study highlights the need for even more aggressive secondary prevention in patients most at risk. Resumo: Introdução: Doentes com doença coronária (DC) têm risco aumentado de acidente vascular cerebral (AVC). O objectivo deste estudo foi analisar o poder prognóstico de determinadas variáveis clínicas e analíticas na predição do risco de evento cerebrovascular (CBV) após enfarte agudo do miocárdio (EAM). População e métodos: 404 doentes consecutivos (idade média 68,1 ± 13,7, 63,5% do sexo masculino, 37,4% com diabetes mellitus [DM]) sem antecedentes de AVC admitidos com o diagnóstico de EAM e que sobreviveram ao internamento. Dados recolhidos: factores de risco cardiovascular (CV), glicemia na admissão (GlicAd), HbA1c, creatinina, níveis máximos de troponina I (MaxTrop), taxa de filtração glomerular (TFG) calculada pelas fórmulas MDRD e Cockcroft-Gault, GRACE score para mortalidade intra-hospitalar e aos 6 meses (MIH e M6M, respectivamente), Killip Class máximo (KCm) e extensão da coronariopatia. Os doentes tiveram alta em ritmo sinusal, foram seguidos por dois anos e cada variável avaliada como possível preditor de AVC ou acidente isquémico transitório (AIT). Resultados: Durante o follow-up, 27 doentes foram admitidos por evento cerebrovascular. A presença de DM, hipertensão, dislipidemia e DC prévia, o tipo de EAM (STEMI versus NSTEMI) e a extensão da coronariopatia não ajudaram a prever o risco de AVC/AIT. As seguintes variáveis associaram-se a maior risco de AVC/AIT: TFG 1 (p = 0,025, OR 2,71, CI95% 1,10–6,69); GRACE MIH > 180 (p = 0,001, OR 4,09, CI95% 1,64–10,22); GRACE M6M > 150 (p = 0,001, OR 4,50, CI95% 1,80–6,27); GlicAd > 140 mg/dL (p = 0,001, OR 5,74, CI95% 1,87–17,58); MaxTrop > 42 ng/mL (p = 0,032, OR 2,64, CI95% 1,06–6,59). Regressão Logística obteve um modelo com os preditores GRACE M6M > 150 (OR 3,26, p = 0,014) e GlicAd > 7,7 mmol/L (OR 4,09, p = 0,017) que se ajustou bem aos dados (Hosmer-Lemeshow: p = 0,969). Discussão/conclusões: Em doentes com EAM, varáveis habitualmente úteis na predição do risco de MIH, como GlicAd e função renal, insuficiência cardíaca aguda e o score de GRACE mostraram valor como preditores de AVC/AIT durante um follow-up de 2 anos. Enquanto um valor do Score de GRACE M6M > 150 e GlicAd > 7,7 mmol/L se assumiram como preditores independentes de AVC/AIT, os factores de risco CV, a presença de CAD prévia e a extensão da coronariopatia não acrescentaram valor preditivo. Este estudo reforça a necessidade de uma prevenção secundária ainda mais agressiva em doentes com GRACE e GlicAD na admissão mais altos e com maior grau de disfunção renal. Keywords: Cerebrovascular disorders, Acute myocardial infarction, Risk prediction, Vascular events, Palavras-chave: Acidente vascular cerebral, Enfarte agudo do miocárdio, Predição de risco, Eventos vasculares

Atherosclerosis is a diffuse arterial disease, discontinuously distributed, that is not always linear in its pathophysiological significance. Starting with a paradigmatic clinical case, we review the hemodynamic techniques that are currently available for the assessment of this condition. We present the case of a male patient with known diffuse atherosclerotic disease, who had previously undergone angioplasty, in which angiography was repeated after acute pulmonary edema, with worsening left ventricular function and local ischemia documented by myocardial scintigraphy. The angiogram showed an intermediate lesion in the left anterior descending artery, not related to the ischemic territory. The significance of the lesion was evaluated anatomically with intravascular ultrasound, and functionally by determining fractional flow reserve with a pressure wire. We review the pathophysiology of the disease and the parameters used for overall evaluation, focusing on catheterization laboratory techniques and their indications and contribution to the diagnosis and treatment of coronary atherosclerosis. Resumo: A aterosclerose é uma doença arterial difusa, com distribuição descontínua, cujo significado fisiopatológico nem sempre é linear. A partir de um caso paradigmático faz-se a revisão das técnicas actualmente disponíveis na sala de hemodinâmica, para este fim. Trata-se de um paciente do sexo masculino, com aterosclerose coronária difusa conhecida, previamente submetido a intervenção percutânea, que realiza coronariografia após descompensação por edema agudo do pulmão, com deterioração da função ventricular esquerda e documentação de isquémia em cintigrafia de perfusão miocárdica. No cateterismo foi observada uma lesão de significado angiográfico intermédio na artéria descendente anterior, em território diferente do documentado por cintigrafia. Procedeu-se primeiro à avaliação do significado da lesão quer anatómico, através de ultrassonografia intravascular, quer funcional, através da determinação da reserva coronária com guia de pressão. Revemos a fisiopatologia desta doença, parâmetros da sua avaliação global, focando estas técnicas per-cateterismo, suas indicações e contribuição no diagnóstico e terapêutica da aterosclerose coronária. Keywords: Atherosclerosis, Pressure, wire, IVUS, Angiography, Palavras-chave: Aterosclerose, Guia de pressão, IVUS, Angiografia

Type-2 diabetic patients constitute a high-risk population for atherosclerosis. Primary prevention, although recommended, is not well funded. Our aim was to evaluate the degree of subclinical atherosclerosis, in asymptomatic diabetic patients, using coronary multi-slice computed tomography (MSCT) angiography.We prospectively studied 71 diabetic patients without any symptoms or documentation of atherosclerotic disease. Coronary MSCT angiography was performed in all patients and coronary artery calcium score (CACS) was evaluated. The number of diseased coronary segments was determined and classified as obstructive or nonobstructive and fibrolipid or calcified lesions. The mean follow-up was 29.5±6.6 months. Major adverse cardiovascular events were registered.The mean age was 59±10 years, 48% were female patients. The duration of diabetes was 12.5±8.7 years. CACS ranged from 0 to 1293 Agatston units (153±269.1). Image quality was generally good, allowing satisfactory evaluation of most of the coronary artery segments. CACS was 0 in 28 patients, but in nine patients MSCT angiography showed fibrolipid plaques. Obstructive coronary artery disease was present in 26.7% of the patients (5.6% with multivessel disease). During the follow-up period, six major adverse cardiovascular events were detected in patients, five of whom had a CACS more than 100 Agatston units.This study shows a high prevalence of silent atherosclerotic lesions in type-2 diabetic patients, reinforcing the importance of risk factor modification even when calcified disease is absent. Coronary MSCT angiography can be performed to identify the atherosclerotic burden and may be an important test in selecting the patients who are benefiting the most from primary prevention.

El ganglioneuroma es un tumor del sistema nervioso simpatico, y puede estar asociado con la hipersecrecion de sustancias vasoactivas responsables de diversos sintomas y signos, como la hipertension arterial. Los autores presentan un caso de ganglioneuroma y una revision de la literatura, centrandose en los aspectos mas importantes del diagnostico y la terapeutica. El paciente tenia crisis hipertensivas sintomaticas recurrentes, e hizo un estudio imaginologico que detecto una imagen nodular en la glandula adrenal derecha. Al ser los tumores neuroblasticos radiologicamente indistinguibles, se procedio a la extirpacion, lo que confirma el diagnostico mediante el analisis anatomopatologico. Este caso demuestra la variabilidad clinica de ganglioneuroma, la utilidad de la imagiologia y la importancia del estudio histologico.

Resumo: A varicela é uma doença infectocontagiosa comum na infância, ocorrendo pouco mais de 2% dos casos em adultos. Desde a década de 80 que a sua incidência nos adultos tem vindo a aumentar, dos quais apenas 7% são seronegativos1. A pneumonia a Varicella zoster, se bem que rara, constitui a complicação mais grave e mais frequente no adulto.Os autores apresentam um caso clínico ilustrativo de pneumonia a Varicella zoster num adulto fumador e imunocompetente e fazem uma breve revisão teórica sobre o tema.Rev Port Pneumol 2010; XVI (3): 493-505 Abstract: Varicella (chickenpox) is a common contagious infection of childhood, with fewer than 2% of the cases occurring in adults. Since the early 1980s the incidence of chickenpox in adults has been increasing and only 7% of them are seronegative for Varicella zoster antibodies. Pneumonia, although rare, is the most common and serious complication of chickenpox infection in adults.The authors present an illustrative case of varicella pneumonia in an immunocompetent adult with smoking habits and make a brief thematic review.Rev Port Pneumol 2010; XVI (3): 493-505 Palavras-chave: Varicela, pneumonia, ARDS, Key-words: Chickenpox, pneumonia, ARDS

Aims: To assess the effectiveness and safety of sirolimus-eluting stents (SES) in de novo native coronary lesions in small vessels (≤ 2.5 mm). Methods and results: PORTO was a multicentre, prospective registry, performed in 274 patients. Of these, 120 (43.8%) patients were diabetic. A total of 324 lesions were treated with 347 SES. The primary endpoint was 6-month in-stent late luminal loss (LL); secondary end-points included in-stent and in-segment binary restenosis (BR), target lesion revascularisation (TLR), target vessel revascularisation (TVR) and major adverse cardiac events (MACE) rate at six months and one-year. Pre-specified subgroup analysis included the comparison between diabetic and non-diabetic patients, and patients with stents of different diameter (2.25 mm vs. 2.5 mm) and length (≤ 18 mm vs. ≥ 23 mm). The mean (SD) reference vessel diameter of the treated segment was 2.08 (0.33) mm and lesion length 11.04 (6.0) mm. After six months, LL was 0.07 (0.37) mm. BR was 5.1% in-stent and 9.1% in-segment. At one year, TLR was 5.6% and TVR was 9.0%. MACE rate was 2.6% at six months and 8.6% at one year with 2.3% cardiac death and 1.5% non-fatal myocardial infarction. Stent thrombosis rate at one year was 0.8% per protocol. There were more MACE in diabetic patients (12.8%) than in non-diabetic (5.4%, p=0.046), but no other significant differences in clinical and angiographic parameters were noted between the subgroups analysed. Conclusions: The use of SES for lesions in very small coronary arteries proved to be safe and efficacious, irrespective of the size and length of the stents, with low restenosis and repeat revascularisation rates at one year.

Resumo: Os autores apresentam o caso de uma doente com os diagnósticos de miocardiopatia hipertrófica e estenose aórtica, na qual foi identificada a presença de um gradiente elevado ao nível do trato de saída do ventrículo esquerdo. O reconhecimento da patologia responsável pela sintomatologia foi desafiante, com envolvimento de várias técnicas de imagem, tendo sido fundamental a contribuição do ecocardiograma transesofágico. O diagnóstico final de estenose aórtica severa conduziu à referenciação para cirurgia de substituição valvular, com sucesso. A presença destas duas patologias em simultâneo num mesmo doente é conhecida, embora incomum. A sua combinação cria importantes desafios diagnósticos e terapêuticos, os quais serão objeto de discussão neste artigo. Abstract: The authors report the case of a patient diagnosed with both hypertrophic cardiomyopathy and aortic stenosis. Due to clinical deterioration, additional investigation was performed, and a high left ventricular outflow tract gradient was identified. Correct identification of the condition causing the symptoms was challenging, and involved several imaging techniques, the contribution of transesophageal echocardiography being crucial. The final diagnosis of severe aortic stenosis led to successful valve replacement surgery. The presence of these two conditions in the same patient has been documented, although it is uncommon. This association poses particular diagnostic and therapeutic challenges, which are discussed in this paper. Palavras‐chave: Estenose aórtica, Cardiomiopatia hipertrófica, Obstrução do trato de saída do ventrículo esquerdo, Ecocardiografia, Ecocardiografia Doppler, Keywords: Aortic valve stenosis, Hypertrophic cardiomyopathy, Left ventricular outflow tract obstruction, Echocardiography, Doppler echocardiography

Brugada syndrome, first described over 20 years ago, is characterized by a typical electrocardiographic pattern with coved-type ST-segment elevation in the right precordial leads and a high risk of sudden death in otherwise healthy young adults.The electrocardiographic pattern is sometimes intermittent, and fever is a possible trigger. The authors present the case of a 68-year-old woman who came to the emergency department with fever and syncope. A diagnosis of community-acquired pneumonia was made. The electrocardiogram performed when the patient had fever revealed a type 1 Brugada pattern, which disappeared after the fever subsided. After other causes of Brugada-like pattern were excluded, Brugada syndrome was diagnosed and a cardioverter-defibrillator was implanted.This case demonstrates that this entity can be diagnosed at more advanced ages and highlights the usefulness of electrocardiography in a febrile state. Resumo: A síndrome de Brugada, descrita há cerca de 20 anos, caracteriza-se eletrocardiograficamente por uma elevação convexa do segmento-ST nas derivações precordiais direitas e pelo elevado risco de morte súbita em jovens aparentemente saudáveis.Este padrão eletrocardiográfico é, por vezes, intermitente, sendo a febre um possível fator precipitante. Os autores apresentam o caso clínico de uma doente de 68 anos que recorre ao serviço de urgência por febre e síncope. Feito o diagnóstico de pneumonia adquirida na comunidade. O eletrocardiograma realizado em contexto de febre revelou um padrão de Brugada tipo 1, que desapareceu após resolução do quadro febril. Excluídas outras causas de padrão Brugada-like foi confirmado o diagnóstico de síndrome de Brugada e realizada implantação de cardioversor-desfibrilhador.Este caso ilustra a possibilidade do diagnóstico desta entidade poder ser feito numa faixa etária já avançada e reforça a utilidade da realização de um eletrocardiograma em contexto febril. Keywords: Brugada syndrome, Fever, Syncope, Palavras-chave: Síndrome de Brugada, Febre, Síncope

Carotid artery angioplasty with stenting is an effective treatment for carotid artery stenosis, but is frequently associated with acute transient hemodynamic changes. We present the case of a 73-year-old female patient with long-standing refractory hypertension who remained normotensive during a three-year follow-up after undergoing staged bilateral carotid angioplasty. Resumo: A angioplastia carotídea constitui uma opção terapêutica eficaz para o tratamento da doença carotídea aterosclerótica significativa, estando por vezes associada a alterações hemodinâmicas transitórias que influenciam a pressão arterial. Apresenta-se o caso clínico de uma mulher de 73 anos de idade com hipertensão arterial crónica refractária, que após ser submetida a angioplastia carotídea bilateral permaneceu normotensa durante um follow up de três anos. Keywords: Refractory hypertension, Bilateral carotid angioplasty, Palavras-chave: Hipertensão arterial refractária, Angioplastia carotídea bilateral

Resumo: A angioplastia carotídea constitui uma opção terapêutica eficaz para o tratamento da doença carotídea aterosclerótica significativa, estando por vezes associada a alterações hemodinâmicas transitórias que influenciam a pressão arterial. Apresenta-se o caso clínico de uma mulher de 73 anos de idade com hipertensão arterial crónica refractária, que após ser submetida a angioplastia carotídea bilateral permaneceu normotensa durante um follow up de três anos. Abstract: Carotid artery angioplasty with stenting is an effective treatment for carotid artery stenosis, but is frequently associated with acute transient hemodynamic changes. We present the case of a 73-year-old female patient with long-standing refractory hypertension who remained normotensive during a three-year follow-up after undergoing staged bilateral carotid angioplasty. Palavras-chave: Hipertensão arterial refractária, Angioplastia carotídea bilateral, Keywords: Refractory hypertension, Bilateral carotid angioplasty

The authors report the case of a patient diagnosed with both hypertrophic cardiomyopathy and aortic stenosis. Due to clinical deterioration, additional investigation was performed, and a high left ventricular outflow tract gradient was identified. Correct identification of the condition causing the symptoms was challenging, and involved several imaging techniques, the contribution of transesophageal echocardiography being crucial. The final diagnosis of severe aortic stenosis led to successful valve replacement surgery. The presence of these two conditions in the same patient has been documented, although it is uncommon. This association poses particular diagnostic and therapeutic challenges, which are discussed in this paper. Resumo: Os autores apresentam o caso de uma doente com os diagnósticos de miocardiopatia hipertrófica e estenose aórtica, na qual foi identificada a presença de um gradiente elevado ao nível do trato de saída do ventrículo esquerdo. O reconhecimento da patologia responsável pela sintomatologia foi desafiante, com envolvimento de várias técnicas de imagem, tendo sido fundamental a contribuição do ecocardiograma transesofágico. O diagnóstico final de estenose aórtica severa conduziu à referenciação para cirurgia de substituição valvular, com sucesso. A presença destas duas patologias em simultâneo num mesmo doente é conhecida, embora incomum. A sua combinação cria importantes desafios diagnósticos e terapêuticos, os quais serão objeto de discussão neste artigo. Keywords: Aortic valve stenosis, Hypertrophic cardiomyopathy, Left ventricular outflow tract obstruction, Echocardiography, Doppler echocardiography, Palavras-chave: Estenose aórtica, Cardiomiopatia hipertrófica, Obstrução do trato de saída do ventrículo esquerdo, Ecocardiografia, Ecocardiografia Doppler

The authors report the case of a 53-year-old man, with a long-standing history of mild mitral stenosis, admitted for worsening fatigue. Transthoracic echocardiography (limited by poor image quality) showed mitral annular calcification, leaflets that were difficult to visualize and an estimated mitral valve area of 1.8 cm2 by the pressure half-time method. However, elevated mean transmitral and right ventricle/right atrium gradients were identified (39 and 117 mmHg, respectively). This puzzling discrepancy in the echocardiographic findings prompted investigation by transesophageal echocardiography, which revealed an echogenic structure adjacent to the mitral annulus, causing severe obstruction (effective orifice area 0.7 cm2). The suspicion of supravalvular mitral ring was confirmed during surgery. Following ring resection and mitral valve replacement there was significant improvement in the patient's clinical condition and normalization of the left atrium/left ventricle gradient.Supravalvular mitral ring is an unusual cause of congenital mitral stenosis, characterized by an abnormal ridge of connective tissue on the atrial side of the mitral valve, which often obstructs mitral valve inflow. Few cases have been reported, most of them in children with concomitant congenital abnormalities. Diagnosis of a supravalvular mitral ring is challenging, since it is very difficult to visualize in most diagnostic tests. It was the combination of clinical and various echocardiographic findings that led us to suspect this very rare condition, enabling appropriate treatment, with excellent long-term results. Resumo: Os autores reportam o caso de um doente de 53 anos, com diagnóstico prévio de estenose mitral ligeira, admitido por agravamento do cansaço. O ecocardiograma (Eco) transtorácico, limitado pela janela acústica, mostrava válvula mitral com ecogenicidade aumentada a nível do anel, deficiente observação dos folhetos e área valvular estimada de 1,8 cm2 por tempo de hemipressão. No entanto, a identificação de elevados gradientes transmitral médio e ventrículo direito/aurícula direita (atingindo respetivamente 39 e 117 mmHg) intrigou os autores. No Eco transesofágico foi observada estrutura hiperecogénica sobre o anel mitral a condicionar obstrução grave (área do orifício efetivo de 0,7 cm2), o que levantou a suspeita de anel supramitral. Esta foi confirmada durante a cirurgia. Após a ressecção do anel e implantação de prótese mecânica, verificou-se uma franca melhoria clínica e normalização do gradiente aurícula esquerda (AE)/ventrículo esquerdo (VE).O anel supramitral é uma causa invulgar de estenose mitral congénita, caracterizado pela presença de uma membrana fibrosa adjacente à face auricular da válvula mitral. Existem poucos casos reportados na literatura, sendo a maioria descritos em idade pediátrica e em associação a outras anomalias congénitas. O diagnóstico é desafiante, dado que o anel raramente é visualizado nos exames complementares. Uma elevada suspeita clínica e a integração dos vários achados ecocardiográficos são aspetos fundamentais para a sua identificação, permitindo o tratamento adequado com bons resultados a longo prazo. Keywords: Supravalvular ring, Supravalvular mitral ring, Mitral stenosis, Congenital mitral stenosis, Supravalvular mitral stenosis, Palavras-chave: Anel supramitral, Anel mitral supravalvular, Estenose mitral, Estenose mitral congénita, Estenose mitral supravalvular

A 73-year-old woman was admitted to the emergency room with predominantly right-sided heart failure and anemia. Following clinical and imagiological evaluation, a diagnosis of pulmonary hypertension (PH) associated with Hereditary Hemorrhagic Telangiectasia (HHT) was confirmed. The initial response to bosentan plus sildenafil was good, including improvement in functional class and reduction of edema, allowing her to be discharged. Unfortunately, the patient died, due to her underlying condition, before the effects of the combination treatment could be fully assessed. PH should be considered in patients with HTT and screening for pulmonary hypertension should be performed in these patients and their relatives.

Background Mean platelet volume has been associated with stroke in patients with atrial fibrillation. However, its role as a predictor of left atrial stasis, assessed by transesophageal echocardiography, in patients with non-valvular atrial fibrillation has not yet been clarified. Methods Single center cross-sectional study comprising 427 patients admitted to the emergency department due to symptomatic atrial fibrillation and undergoing transesophageal echocardiogram evaluation for exclusion of left atrial appendage thrombus before cardioversion. All patients had a complete blood count performed in the 12 hours prior to transesophageal echocardiogram. Markers of left atrial stasis were sought: left atrial appendage thrombus, dense spontaneous echocardiographic contrast and low flow velocities in the left atrial appendage. The presence of at least one of the former markers of left atrial stasis was designated left atrial abnormality. Binary logistic multivariate analysis was used for obtaining models for the prediction of transesophageal echocardiogram endpoints. Results Left atrial appendage thrombus was found in 12.2%, dense spontaneous echocardiographic contrast in 29.7%, low flow velocities in 15.3% and left atrial abnormality in 34.2%. Mean platelet volume (exp β = 3.41 p = 0.048) alongside with previous stroke or transient ischemic attack (exp β = 5.35 p = 0.005) and troponin I (exp β = 5.07 p = 0.041) were independent predictors of left atrial appendage thrombus. Mean platelet volume was also incorporated in the predictive models of dense spontaneous echocardiographic contrast, low flow velocities and left atrial abnormality, adding predictive value to clinical, echocardiographic and laboratory variables. Conclusions These findings suggest that mean platelet volume may be associated with the presence of markers of left atrial stasis, reinforcing a likely cardioembolic mechanism for its association with stroke in patients with non-valvular atrial fibrillation.

AimsAlthough it is accepted that atrial fibrillation (AF) may be both the contributing factor and the consequence of pulmonary embolism (PE), data on the prognostic role of AF in patients with acute venous thromboembolism are scarce. Our aim was to study whether AF had a prognostic role in patients with acute PE.MethodsRetrospective cohort study involving 270 patients admitted for acute PE. Collected data: past medical history, analytic/gasometric parameters, admission ECG and echocardiogram, thoracic CT angiography. Patients followed for 6 months. An analysis was performed in order to clarify whether history of AF, irrespective of its timing, helps predict intrahospital, 1-month and 6-month all-cause mortality.ResultsPatients with history of AF, irrespective of its timing (n=57, 21.4%), had higher intrahospital (22.8% vs 13.1%, p=0.052, OR 2.07, 95% CI 0.98 to 4.35), 1-month (35.1% vs 16.9%, p=0.001, OR 3.16, 95% CI 1.61 to 6.21) and 6-month (45.6% vs 17.4%, pConclusionsThe presence of AF, irrespective of its timing, may independently predict mortality in patients with acute PE. These data should be tested and validated in prospective studies using larger cohorts.

The authors present a case of systemic amyloidosis with cardiac involvement. We discuss the need for a high level of suspicion to establish a diagnosis, diagnostic techniques and treatment options. Our patient was a 78-year-old man with chronic renal disease and atrial fibrillation admitted with acute decompensated heart failure of unknown cause. The transthoracic echocardiogram revealed severely impaired left ventricular function with phenotypic overlap between hypertrophic and restrictive cardiomyopathy. After an extensive diagnostic workup, which included an abdominal fat pad biopsy, the final diagnosis was amyloidosis. Resumo: Os autores apresentam um caso de amiloidose sistémica com envolvimento cardíaco e discutem a importância de um elevado índice de suspeição para o diagnóstico, os meios de diagnóstico e as opções terapêuticas à luz do conhecimento atual. Homem de 78 anos, com antecedentes de doença renal crónica e fibrilhação auricular, admitido por insuficiência cardíaca aguda de etiologia desconhecida. O ecocardiograma transtorácico mostrou ventrículo esquerdo não dilatado, com compromisso severo da função sistólica global com overlap fenotípico de miocardiopatia hipertrófica e restritiva. Após estudo complementar alargado, o diagnóstico definitivo de amiloidose foi obtido por biópsia da gordura abdominal. Keywords: Amyloidosis, Hypertrophic cardiomyopathy, Restrictive cardiomyopathy, Heart failure, Palavras-chave: Amiloidose, Miocardiopatia hipertrófica, Miocardiopatia restritiva, Insuficiência cardíaca

Congestive heart failure and left ventricular dysfunction in the setting of severe aortic stenosis are associated with a high mortality rate. Evidence on optimal medical therapy is scanty. Vasodilators were traditionally considered to be contraindicated in severe aortic stenosis, albeit this concept has recently been challenged. The use of levosimendan, which has positive inotropic, vasodilatory and cardioprotective properties seems attractive. We describe a small series of exceedingly ill patients with severe aortic stenosis and left ventricular dysfunction, in different clinical settings (acute heart failure, cardiogenic shock and difficult-to-wean ventilatory support), in which levosimendan was successfully used.

Despite the existence of several risk scores, the accurate prediction of the prognosis in pulmonary embolism (PE) remains a challenge. The Global Registry of Acute Coronary Events (GRACE) risk score has a high diagnostic performance for adverse outcomes in acute coronary syndrome. We aimed to assess the applicability and extend the use of the GRACE risk score to PE. A case-control study of 206 consecutive patients admitted with PE was performed. The GRACE, Geneva, Simplified Pulmonary Embolism Severity Index, Shock Index, and European Society of Cardiology risk scores were tested for the prediction of the primary end point: all-cause 30-day mortality. Comparisons between GRACE and the other risk scores were performed using receiver operating characteristic area under the curve and the integrated discrimination improvement index. All-cause 30-day mortality was observed in 18.9% of the patients. Unlike the other classifications, no adverse outcomes were observed in patients classified as low risk using the GRACE risk score (100% negative predictive value for GRACE risk score ≤113). The GRACE score showed greater discriminative performance than the Geneva score (area under the curve 0.623, 95% confidence interval [CI] 0.53 to 0.71), Shock Index (area under the curve 0.639, 95% CI 0.55 to 0.73), European Society of Cardiology (area under the curve 0.662, 95% CI 0.57 to 0.76), and Simplified Pulmonary Embolism Severity Index (area under the curve 0.705, 95% CI 0.61 to 0.80), although statistical significance was not reached. The integrated discrimination improvement index suggested a more appropriate risk classification with the GRACE score. In conclusion, our results have demonstrated that the GRACE risk score can accurately predict 30-day mortality in patients admitted for acute PE. Compared to previously proposed PE prediction rules, the GRACE risk score presented improved overall risk classification.

Hypertension is a major risk factor for cardiovascular disease, resulting in increased incidence of cerebrovascular events, ischaemic heart disease, heart failure, and renal impairment. Thus, it is one of the most important preventable causes of premature morbidity and mortality. Despite current knowledge on the management of hypertension and the availability of several effective antihypertensive medications, uncontrolled hypertension remains a common and challenging clinical problem. Resistant hypertension is a complex condition with multiple contributing factors and overlapping comorbidities. Although there is limited hard evidence regarding resistant hypertension, our understanding of this condition has improved recently. This article will present an overview of resistant hypertension and highlight recent publications about this topic.

Introduction: In non-valvular atrial fibrillation 90% of thrombi originate in the left atrial appendage (LAA). Percutaneous LAA closure has been shown to be non-inferior to warfarin for prevention of thromboembolism. Objective: To evaluate the initial experience of a single center in percutaneous LAA closure in patients with high thromboembolic risk and in whom oral anticoagulation was impractical or contraindicated or had failed. Methods: Patients with non-valvular atrial fibrillation and CHADS2 score ≥2 in whom oral anticoagulation was impractical or contraindicated or had failed underwent percutaneous LAA closure according to the standard technique. After the procedure, dual antiplatelet therapy was maintained for one month, followed by single antiplatelet therapy indefinitely. Patients were followed by clinical assessment and transthoracic and transesophageal echocardiography. Results: The procedure was performed in 22 of the 23 selected patients (95.7%), mean age 70±9 years, CHADS2 score 3.2±0.9 and CHA2DS2-VASC score 4.7±1.4. Intraprocedural device replacement was necessary only in the first patient, due to oversizing. The following periprocedural complications were observed: one femoral pseudoaneurysm, three femoral hematomas and two minor oropharyngeal bleeds, resolved by local hemostatic measures. During a 12±8 month follow-up a mild peri-device flow and a thrombus adhering to the device, resolved under with enoxaparin therapy, were identified. The rate of transient ischemic attack (TIA)/stroke was lower than expected according to the CHADS2 score (0 vs. 6.7±2.2%). Conclusions: In our initial experience, this procedure proved to be a feasible, safe and effective alternative for atrial fibrillation patients in whom oral anticoagulation is not an option. Only relatively minor complications were observed, with a lower than expected TIA/stroke rate. Resumo: Introdução: Na fibrilhação auricular não valvular, 90% dos trombos originam-se no apêndice auricular esquerdo. O seu encerramento percutâneo mostrou ser não inferior à varfarina na profilaxia do tromboembolismo. Objetivo: Avaliar a experiência inicial de um centro no encerramento percutâneo do apêndice auricular esquerdo em doentes com elevado risco tromboembólico sem possibilidade, com contraindicação ou falência da anticoagulação oral. Métodos: Doentes com fibrilhação auricular não valvular, score CHADS2 ≥ 2 sem possibilidade, com contraindicação ou falência da anticoagulação oral foram submetidos a encerramento percutâneo do apêndice auricular esquerdo de acordo com a técnica padrão. Após implantação, foi mantida dupla antiagregação durante um mês e simples indefinidamente. Realizado seguimento com avaliação clínica, ecocardiografia transtorácica e transesofágica. Resultados: O procedimento foi conseguido em 22 dos 23 doentes selecionados (95,7%): 70±9 anos, score CHADS2 de 3,2±0,9 e CHA2DS2-VASC de 4,7±1,4. Apenas no primeiro doente o dispositivo foi substituído por sobredimensionamento. Foram observadas as seguintes complicações periprocedimento: um pseudoaneurisma femoral, 3 hematomas femorais e 2 hemorragias da orofaringe, resolvidos com medidas locais. Durante o seguimento de 12±8 meses foram identificados um fluxo peridispositivo ligeiro e um trombo aderente ao dispositivo - que resolveu sob enoxaparina. A taxa de AVC/AIT foi inferior à esperada com base no score CHADS2 (0 versus 6,7±2,2%). Conclusões: Na nossa experiência inicial este procedimento mostrou ser uma alternativa exequível, segura e eficaz em doentes com fibrilhação auricular para os quais a anticoagulação oral não é uma opção. Foram identificadas complicações de baixa severidade, com uma taxa de AVC/AIT inferior à esperada. Keywords: Atrial fibrillation, Thromboembolism, Stroke, Left atrial appendage, Anticoagulation, Cardiac implant, Palavras-chave: Fibrilhação auricular, Tromboembolismo, Acidente vascular cerebral, Apêndice auricular esquerdo, Anticoagulação oral, Implante cardíaco