Cancer patients with acute coronary syndrome have non-superior bleeding risk compared to patients with similar characteristics - a propensity score analysis from the ProACS registry.

INTRODUCTION: The management of acute coronary syndrome (ACS) in malignancy is challenging due to higher bleeding risk. METHODS: We analyzed patients with cancer (active or in the previous five years) prospectively included in the ProACS registry between 2010 and 2019. Our aim was to assess safety (major bleeding, primary endpoint) and secondary efficacy endpoints (in-hospital mortality and combined in-hospital mortality, reinfarction and ischemic stroke) of ACS treatment. Propensity score matching analysis (1:1) was further performed to better understand predictors of outcomes. RESULTS: We found 934 (5%) cancer patients out of a total of 18 845 patients with ACS. Cancer patients had more events: major bleeding (2.9% vs. 1.5%), in-hospital mortality (5.8% vs. 3.4%) and the combined endpoint (7.4% vs. 4.9%). The primary endpoint was related to cancer diagnosis (OR 1.97), previous bleeding (OR 7.09), hemoglobin level (OR 4.94), atrial fibrillation (OR 3.50), oral anticoagulation (OR 3.67) and renal dysfunction. Mortality and the combined secondary endpoint were associated with lower use of invasive coronary angiography and antiplatelet and neurohormonal blocker therapy. After propensity score matching (350 patients), there were no statistically significant differences in endpoints between the populations. CONCLUSION: Bleeding risk was not significant higher in the cancer population compared to patients with similar characteristics, nor were mortality or ischemic risk. The presence of cancer should not preclude simultaneous ACS treatment.

Non-ST Elevation Myocardial Infarction With Occluded Culprit Artery: A Continuum of Mortality Risk Across the Spectrum of Acute Myocardial Infarction.

BACKGROUND: Non-ST segment elevation myocardial infarction (NSTEMI) patients presenting with occluded culprit artery (OCA) may be at higher risk for worse outcomes. We sought to compare in-hospital (IH) mortality between patients presenting with NSTEMI with and without OCA, and ST-segment elevation myocardial infarction (STEMI). METHODS: This retrospective analysis studied 14,037 patients enrolled in the Portuguese National Registry of Acute Coronary Syndromes. Three groups were defined: (A) STEMI (n = 8616); (B) OCA-NSTEMI (n = 1309); and (C) non-OCA NSTEMI (n = 4112). Baseline characteristics, therapeutic strategies, and outcomes were compared. Multivariate analysis was performed to assess the risk of IH all-cause mortality across the prespecified groups. RESULTS: Twenty-four percent of NSTEMI patients presented with OCA. The left circumflex artery was more frequently the culprit artery in group B (12.4% A vs 34.5% B vs 26.0% C; P<.001) and this group was also less likely to receive percutaneous revascularization (95.2% A vs 69.7% B vs 83.2% C; P<.001). The incidence of left ventricular systolic dysfunction was higher in group A and lower in group C (19.9% A vs 12.2% B vs 8.1% C; P<.001). The adjusted risk of IH mortality was significantly higher in group A when compared with group B (3.9% A vs 1.8% B; odds ratio, 2.34; 95% confidence interval, 1.34-4.07; P<.01) and in group B when compared with group C (1.8% B vs 0.9% C; odds ratio, 2.25; 95% confidence interval, 1.17-4.35; P=.02). CONCLUSION: OCA-NSTEMI patients had worse IH outcomes than non-OCA NSTEMI patients and better IH outcomes than STEMI patients, suggesting the existence of a continuum of increased risk of IH mortality across these groups.

Estratégias de Revascularização em Doentes com Infarto Agudo do Miocárdio em Choque Cardiogênico - Resultados do Registo Português de Síndromes Coronárias Agudas / Revascularization Strategies in Patients with Acute Myocardial Infarction and Cardiogenic Shock: Results from the Portuguese Registry on Acute Coronary Syndromes

Resumo Fundamento: Em doentes com infarto agudo do miocárdio (IAM), choque cardiogênico (CC) e doença multivaso (DMV) persistem dúvidas sobre a intervenção nas artérias não responsáveis. Objetivos: 1) caracterizar a amostra de doentes com IAM, CC e DMV incluídos no Registo Nacional Português de Síndromes Coronárias Agudas (RNSCA); 2) comparar os eventos associados a diferentes estratégias de revascularização; e 3) identificar preditores de mortalidade intra-hospitalar nesta amostra. Métodos: Estudo observacional retrospetivo de doentes com IAM, CC e DMV incluídos no RNSCA entre 2010 e 2018. Compararam-se duas estratégias de revascularização: completa durante o procedimento índice (grupo 1); e completa diferida ou incompleta durante o internamento (grupo 2-3). O endpoint primário foi a ocorrência de reinfarto ou morte intra-hospitalar. A significância estatística foi definida por um valor p < 0,05. Resultados: Identificaram-se 127 doentes com IAM, CC e DMV (18,1% no grupo 1 e 81,9% no grupo 2-3), com idade média de 70 ± 12 anos e 92,9% com IAM com supradesnivelamento do segmento ST. O endpoint primário ocorreu em 47,8% dos doentes do grupo 1 e em 37,5% do grupo 2-3 (p = 0,359). As taxas de mortalidade intra-hospitalar, reinfarto, acidente vascular cerebral e hemorragia major foram também semelhantes nos dois grupos. Os preditores de mortalidade intra-hospitalar nesta amostra foram a presença na admissão de disfunção ventricular esquerda (OR 16,8), bloqueio completo de ramo direito (OR 7,6) e anemia (OR 5,2), (p ≤ 0,02). Conclusões: Entre os doentes com IAM, CC e DMV, incluídos no RNSCA, não se verificou diferença significativa entre revascularização completa no evento índex e completa diferida ou incompleta durante o internamento, relativamente à ocorrência de morte intra-hospitalar ou reinfarto. (Arq Bras Cardiol. 2021; 116(5):867-876)

Abstract Background: In patients with acute myocardial infarction (MI), cardiogenic shock (CS), and multivessel disease (MVD) questions remain unanswered when it comes to intervention on non-culprit arteries. Objective: This article aims to 1) characterize patients with MI, CS and MVD included in the Portuguese Registry on Acute Coronary Syndromes (ProACS); 2) compare different revascularization strategies in the sample; 3) identify predictors of in-hospital mortality among these patients. Methods: Observational retrospective study of patients with MI, CS and MVD included in the ProACS between 2010 and 2018. Two revascularization strategies were compared: complete during the index procedure (group 1); and complete or incomplete during the index hospitalization (groups 2-3). The primary endpoint was a composite of in-hospital death or MI. Statistical significance was defined by a p-value <0.05. Results: We identified 127 patients with MI, CS, and MVD (18.1% in group 1, and 81.9% in groups 2-3), with a mean age of 7012 years, and 92.9% of the sample being diagnosed with ST-segment elevation MI (STEMI). The primary endpoint occurred in 47.8% of the patients in group 1 and 37.5% in group 2-3 (p = 0.359). The rates of in-hospital death, recurrent MI, stroke, and major bleeding were also similar. The predictors of in-hospital death in this sample were the presence of left ventricle systolic dysfunction on admission (OR 16.8), right bundle branch block (OR 7.6), and anemia (OR 5.2) (p ≤ 0.02 for both). Conclusions: Among patients with MI, CS, and MVD included in the ProACS, there was no significant difference between complete and incomplete revascularization during the index hospitalization regarding the occurrence of in-hospital death or MI. (Arq Bras Cardiol. 2021; 116(5):867-876)

Revascularization Strategies in Patients with Acute Myocardial Infarction and Cardiogenic Shock: Results from the Portuguese Registry on Acute Coronary Syndromes. / Estratégias de Revascularização em Doentes com Infarto Agudo do Miocárdio em Choque Cardiogênico ­ Resultados do Registo Português de Síndromes Coronárias Agudas.

BACKGROUND: In patients with acute myocardial infarction (MI), cardiogenic shock (CS), and multivessel disease (MVD) questions remain unanswered when it comes to intervention on non-culprit arteries. OBJECTIVE: This article aims to 1) characterize patients with MI, CS and MVD included in the Portuguese Registry on Acute Coronary Syndromes (ProACS); 2) compare different revascularization strategies in the sample; 3) identify predictors of in-hospital mortality among these patients. METHODS: Observational retrospective study of patients with MI, CS and MVD included in the ProACS between 2010 and 2018. Two revascularization strategies were compared: complete during the index procedure (group 1); and complete or incomplete during the index hospitalization (groups 2-3). The primary endpoint was a composite of in-hospital death or MI. Statistical significance was defined by a p-value <0.05. RESULTS: We identified 127 patients with MI, CS, and MVD (18.1% in group 1, and 81.9% in groups 2-3), with a mean age of 7012 years, and 92.9% of the sample being diagnosed with ST-segment elevation MI (STEMI). The primary endpoint occurred in 47.8% of the patients in group 1 and 37.5% in group 2-3 (p = 0.359). The rates of in-hospital death, recurrent MI, stroke, and major bleeding were also similar. The predictors of in-hospital death in this sample were the presence of left ventricle systolic dysfunction on admission (OR 16.8), right bundle branch block (OR 7.6), and anemia (OR 5.2) (p ≤ 0.02 for both). CONCLUSIONS: Among patients with MI, CS, and MVD included in the ProACS, there was no significant difference between complete and incomplete revascularization during the index hospitalization regarding the occurrence of in-hospital death or MI. (Arq Bras Cardiol. 2021; 116(5):867-876).

FUNDAMENTO: Em doentes com infarto agudo do miocárdio (IAM), choque cardiogênico (CC) e doença multivaso (DMV) persistem dúvidas sobre a intervenção nas artérias não responsáveis. OBJETIVOS: 1) caracterizar a amostra de doentes com IAM, CC e DMV incluídos no Registo Nacional Português de Síndromes Coronárias Agudas (RNSCA); 2) comparar os eventos associados a diferentes estratégias de revascularização; e 3) identificar preditores de mortalidade intra-hospitalar nesta amostra. MÉTODOS: Estudo observacional retrospetivo de doentes com IAM, CC e DMV incluídos no RNSCA entre 2010 e 2018. Compararam-se duas estratégias de revascularização: completa durante o procedimento índice (grupo 1); e completa diferida ou incompleta durante o internamento (grupo 2-3). O endpoint primário foi a ocorrência de reinfarto ou morte intra-hospitalar. A significância estatística foi definida por um valor p < 0,05. RESULTADOS: Identificaram-se 127 doentes com IAM, CC e DMV (18,1% no grupo 1 e 81,9% no grupo 2-3), com idade média de 70 ± 12 anos e 92,9% com IAM com supradesnivelamento do segmento ST. O endpoint primário ocorreu em 47,8% dos doentes do grupo 1 e em 37,5% do grupo 2-3 (p = 0,359). As taxas de mortalidade intra-hospitalar, reinfarto, acidente vascular cerebral e hemorragia major foram também semelhantes nos dois grupos. Os preditores de mortalidade intra-hospitalar nesta amostra foram a presença na admissão de disfunção ventricular esquerda (OR 16,8), bloqueio completo de ramo direito (OR 7,6) e anemia (OR 5,2), (p ≤ 0,02). CONCLUSÕES: Entre os doentes com IAM, CC e DMV, incluídos no RNSCA, não se verificou diferença significativa entre revascularização completa no evento índex e completa diferida ou incompleta durante o internamento, relativamente à ocorrência de morte intra-hospitalar ou reinfarto. (Arq Bras Cardiol. 2021; 116(5):867-876).

Dual antiplatelet therapy in myocardial infarction with non-obstructive coronary artery disease - insights from a nationwide registry.

INTRODUCTION AND OBJECTIVES: Dual antiplatelet therapy (DAPT) is a mainstay for myocardial infarction (MI) therapy. However, in patients with myocardial infarction with non-obstructive coronary artery disease (MINOCA), clear recommendations are lacking in the literature. This study aims to identify the cases in which DAPT is currently prescribed at discharge for MINOCA. METHODS: The authors analyzed a cohort of patients from a multicenter national registry enrolling patients who suffered their first MI between 2010 and 2017, and underwent coronary angiography revealing absence of stenosis ≥50%. Individual antithrombotic therapy was identified. A logistic regression analysis was applied to search for predictors of DAPT. RESULTS: From a total of 16 237 patients analyzed, 709 (4.4%) were categorized as MINOCA. Mean age was 64±13 years, 46.3% (n=409) were females. 390 (55.0%) of MINOCA patients were discharged on DAPT. Males (OR 1.67, CI 95 [1.05-2.38], p=0.027), active smokers (OR=1.82, CI 95 [1.05-3.16], p=0.033), previous percutaneous intervention (OR 3.18, CI 95 [1.48-6.81], p=0.003), ST elevation MI (OR 2.70, CI 95 [1.59-4.76], p<0.001) and sinus rhythm at admission (OR=3.94, CI 95 [2.07-7.48], p<0.001) were independent predictors of DAPT use. CONCLUSION: In this nationwide registry, DAPT was prescribed at discharge in 55% of MINOCA patients. Beyond sinus rhythm, the variables presented as independent predictors for DAPT use identify subgroups of patients who are classified as more prone to thrombotic events. The issue of how to handle antithrombotic agents in MINOCA patients is a topic open for discussion.

The sex gap in hypertrophic cardiomyopathy.

INTRODUCTION AND OBJECTIVES: Key sex differences have been explored in multiple cardiac conditions. However, sex impact in hypertrophic cardiomyopathy outcome is unclear. We aimed to characterize sex impact in overall and cardiovascular (CV) mortality in a nationwide hypertrophic cardiomyopathy registry. METHODS: We analyzed 1042 adult patients, 429 (41%) women, from a national registry of hypertrophic cardiomyopathy, with mean age at diagnosis 53±16 years and a mean follow-up of 65±75 months. At baseline, women were older (56±16 vs 51±15 years; P <.001), more symptomatic (56.4%, vs 51.7%; P <.001) and had more heart failure (42.0% vs 24.2%. P <.001), diastolic dysfunction (75.2% vs 64.1% P=.001), moderate/severe mitral regurgitation (33.4% vs 21.7%; P=.003), and higher B-type natriuretic peptide levels (920 [366-2412] mg/dL vs 487 [170-1087] mg/dL; P <.001). Women underwent fewer stress tests and cardiac magnetic resonance. RESULTS: Kaplan-Meier survival curves showed higher overall (8.4% vs 5.0%; P=.026) and CV mortality (5.5% vs 2.2%; P=.004) in women. Cox proportional hazard regression showed that female sex was an independent predictor of overall (HR, 2.05; 95%CI, 1.11-3.78; P=.021) and CV mortality (HR, 3.16; 95%CI, 1.25-7.99; P=.015). Women had more heart failure-related death (2.6% vs 0.8%, P=.024). Despite similar sudden cardiac death (SCD) risk, women received fewer implantable cardioverter-defibrillators (10.9% vs 15.6%; P=.032) and, in patients without cardioverter-defibrillators, SCD occurred more commonly in women (1.8% vs 0.4%; P=.031). CONCLUSIONS: In this nationwide registry, female sex was an independent predictor of overall and CV-related death, with more heart failure-related death. Despite similar SCD risk, women were undertreated with implantable cardioverter-defibrillators. These data highlight the need for an improved clinical approach in women with HCM.

Impact of previous coronary artery bypass grafting in patients presenting with an acute coronary syndrome: Current trends and clinical implications.

BACKGROUND: Among patients presenting with an acute coronary syndrome, those with previous coronary artery bypass grafting are a particular subset. AIMS: The purpose of this study was to investigate the prognostic impact of previous coronary artery bypass grafting in acute coronary syndrome patients and to identify the current trends in their clinical management. METHODS: We performed a cohort analysis of patients prospectively enrolled in the Portuguese Registry of acute coronary syndrome between 2010-2019 with known previous coronary artery bypass grafting status. The co-primary endpoints were in-hospital and one-year mortality. RESULTS: A total of 19,334 (962 coronary artery bypass grafting and 18,372 non-coronary artery bypass grafting) and 9402 (479 coronary artery bypass grafting and 8923 non-coronary artery bypass grafting) patients were included in the analyses of in-hospital and mid-term outcomes, respectively. Coronary artery bypass grafting patients were older and had a higher incidence of comorbidities. They were less likely to undergo invasive angiography (74.9 vs 84.6%, p<0.001), but were equally likely to receive dual antiplatelet therapy (91.0 vs 90.8%, p=0.823). In-hospital mortality was similar between groups (3.6 vs 3.4%, p=0.722). Unadjusted one-year mortality was higher in the coronary artery bypass grafting group (hazard ratio 1.48, 95% confidence interval 1.09-2.01, p=0.012), but similar in both groups after propensity-matching and multivariate analysis (hazard ratio 0.63, 95% confidence interval 0.37-1.09, p=0.098). CONCLUSIONS: Among patients with acute coronary syndrome, a previous history of coronary artery bypass grafting was associated with a high burden of comorbidities and a high-risk profile but was not an independent predictor of adverse events. Treatment decisions should be made on a case-by-case basis, and should not be based on previous coronary artery bypass grafting status alone.

New-Onset Atrial Fibrillation in St-Segment Elevation Myocardial Infarction: Predictors and Impact on Therapy And Mortality / Fibrilação Auricular de Novo no Infarto Agudo do Miocárdio com Supradesnível do Segmento ST: Preditores e Impacto na Terapêutica e Mortalidade

Abstract Backgrund: New-onset atrial fibrillation complicating acute myocardial infarction represents an important challenge, with prognostic significance. Objective: To study the incidence, impact on therapy and mortality, and to identify predictors of development of new-onset atrial fibrillation during hospital stay for ST-segment elevation myocardial infarction. Methods: We studied all patients with ST-elevation myocardial infarction included consecutively, between 2010 and 2017, in a Portuguese national registry and compared two groups: 1 - no atrial fibrillation and 2 - new-onset atrial fibrillation. We adjusted a logistic regression model data analysis to assess the impact of new-onset atrial fibrillation on in-hospital mortality and to identify independent predictors of its development. A p value < 0.05 was considered significant. Results: We studied 6325 patients, and new-onset atrial fibrillation was found in 365 (5.8%). Reperfusion was successfully accomplished in both groups with no difference regarding type of reperfusion. In group 2, therapy with beta-blockers and angiotensin-conversion enzyme (ACE) inhibitors/angiotensin receptor blockers (ARBs) was less frequent, 20.6% received anticoagulation at discharge and 16.1% were on triple therapy. New-onset atrial fibrillation was associated with more in-hospital complications and mortality. However, it was not found as an independent predictor of in-hospital mortality. We identified age, prior stroke, inferior myocardial infarction and complete atrioventricular block as independent predictors of new-onset atrial fibrillation. Conclusion: New-onset atrial fibrillation remains a frequent complication of myocardial infarction and is associated with higher rate of complications and in-hospital mortality. Age, prior stroke, inferior myocardial infarction and complete atrioventricular block were independent predictors of new onset atrial fibrillation. Only 36.7% of the patients received anticoagulation at discharge.

Resumo Fundamento: A fibrilação auricular de novo no contexto de infarto agudo do miocárdio representa um importante desafio com potencial impacto prognóstico. Objetivo: Determinar a incidência, impacto na terapêutica e mortalidade, e identificar possíveis preditores do aparecimento de fibrilação auricular de novo durante o internamento por infarto agudo do miocárdio com supradesnivelamento do segmento ST. Métodos: Estudamos todos os pacientes com infarto agudo do miocárdio com supradesnivelamento do segmento ST inseridos consecutivamente de 2010 a 2017 num registro nacional português e comparamos dois grupos: 1 - sem fibrilação auricular; 2- com fibrilação auricular de novo. Efetuamos análise com modelo de regressão logística para avaliar o impacto de fibrilação auricular de novo na mortalidade intra-hospitalar e identificar preditores independentes para o seu aparecimento. Para teste de hipóteses, considerou-se significativo p < 0,05. Resultados: Estudamos 6325 pacientes, dos quais 365 (5.8%) apresentaram fibrilação auricular de novo. Não houve diferença no número de pacientes reperfundidos nem na estratégia de reperfusão. No grupo 2, terapêutica com betabloqueadores e IECA/ARA foi menos frequente, 20.6% tiveram alta sob anticoagulação oral e 16.1% sob terapêutica tripla. A fibrilação auricular de novo associou-se a maior incidência de complicações e mortalidade intra-hospitalar, mas não foi preditor independente de mortalidade intra-hospitalar. Identificamos idade, acidente vascular cerebral prévio, infarto inferior e bloqueio auriculoventricular completo como preditores independentes de fibrilação auricular de novo. Conclusões: A fibrilação auricular de novo continua sendo uma complicação frequente do infarto agudo do miocárdio, estando associada a aumento das complicações e mortalidade intra-hospitalar. Apenas 36.7% desses pacientes teve alta sob anticoagulação.

New-Onset Atrial Fibrillation in St-Segment Elevation Myocardial Infarction: Predictors and Impact on Therapy And Mortality.

BACKGRUND: New-onset atrial fibrillation complicating acute myocardial infarction represents an important challenge, with prognostic significance. OBJECTIVE: To study the incidence, impact on therapy and mortality, and to identify predictors of development of new-onset atrial fibrillation during hospital stay for ST-segment elevation myocardial infarction. METHODS: We studied all patients with ST-elevation myocardial infarction included consecutively, between 2010 and 2017, in a Portuguese national registry and compared two groups: 1 - no atrial fibrillation and 2 - new-onset atrial fibrillation. We adjusted a logistic regression model data analysis to assess the impact of new-onset atrial fibrillation on in-hospital mortality and to identify independent predictors of its development. A p value < 0.05 was considered significant. RESULTS: We studied 6325 patients, and new-onset atrial fibrillation was found in 365 (5.8%). Reperfusion was successfully accomplished in both groups with no difference regarding type of reperfusion. In group 2, therapy with beta-blockers and angiotensin-conversion enzyme (ACE) inhibitors/angiotensin receptor blockers (ARBs) was less frequent, 20.6% received anticoagulation at discharge and 16.1% were on triple therapy. New-onset atrial fibrillation was associated with more in-hospital complications and mortality. However, it was not found as an independent predictor of in-hospital mortality. We identified age, prior stroke, inferior myocardial infarction and complete atrioventricular block as independent predictors of new-onset atrial fibrillation. CONCLUSION: New-onset atrial fibrillation remains a frequent complication of myocardial infarction and is associated with higher rate of complications and in-hospital mortality. Age, prior stroke, inferior myocardial infarction and complete atrioventricular block were independent predictors of new onset atrial fibrillation. Only 36.7% of the patients received anticoagulation at discharge.

Genetic Variants Are Not Rare in ICD Candidates with Dilated Cardiomyopathy: Time for Next-Generation Sequencing?

BACKGROUND: Sudden cardiac death (SCD) risk stratification in dilated cardiomyopathy (DCM) has been based on left ventricular ejection fraction (LVEF), even though SCD may occur with LVEF > 35%. Family history of unexplained SCD, especially in the young, raises concern about potential inheritable risk factors. It remains largely unknown how genetic tests can be integrated into clinical practice, particularly in the selection of implantable cardioverter defibrillator (ICD) candidates. We aimed to assess the diagnostic yield of genetic testing in DCM patients with a class I recommendation for ICD implantation, based on current guidelines. METHODS: We included ambulatory stable adult patients with idiopathic or familial DCM with previously implanted ICD. Molecular analysis included 15 genes (LMNA, MYH7, MYBPC3, TNNT2, ACTC1, TPM1, CSRP3, TCAP, SGCD, PLN, MYL2, MYL3, TNNI3, TAZ, and LDB3) using next-generation sequencing. RESULTS: We evaluated 21 patients, 12 (57%) males and 9 (43%) with familial DCM, including 3 (14%) with a family history of premature unexplained SCD. Mean age at DCM diagnosis was 40 ± 2 years, and mean age at ICD implantation was 50 ± 12 years. LVEF was 27 ± 9%, and LV end-diastolic diameter was 65 ± 7 mm. Genetic variants were found in six (29%) patients, occurring in 5 genes: TPM1, TNNT2, MYH7, PLN, and MYBPC3. The majority were classified as variants of uncertain significance. Family history of SCD was present in both patients with PLN variants. CONCLUSION: In patients with DCM and ICD, genetic variants could be identified in a significant proportion of patients in several genes, highlighting the potential role of genetics in DCM SCD risk stratification.

Beta-blockers for post-acute coronary syndrome mid-range ejection fraction: a nationwide retrospective study.

BACKGROUND: Patients with mid-range ejection fraction (40-49%) are in focus due to the newly defined entity of heart failure with mid-range ejection fraction. Acute coronary syndromes are a major aetiology for heart failure with mid-range ejection fraction. We aim to evaluate which therapeutic decisions are associated with inhospital survival benefit in post-acute coronary syndrome patients categorised according to the ejection fraction. METHODS AND RESULTS: The authors analysed a cohort of a multicentre national registry enrolling acute coronary syndrome patients between 2010 and 2016, classified according to their ejection fraction before hospital discharge. Patients with previously known heart failure or with no ejection fraction evaluation were excluded. A total of 9429 patients were included and categorised in three groups: (a) ejection fraction of 50% or greater (n=6113, 65%); (b) ejection fraction of 40-49% (n=1926, 20%); and (c) ejection fraction less than 40% (n=1390, 15%). The primary endpoint was inhospital mortality. To eliminate confounding factors, a multivariate logistic regression analysis was conducted, including acute coronary syndrome type, baseline characteristics, pharmacological treatment, clinical data, laboratory data and coronary anatomy when known. The overall inhospital mortality was 2.8% (n=263): 0.9% (n=53) in group 1, 2.4% (n=37) in group 2 and 11.4% (n=159) in group 3. After multivariate analysis, an invasive strategy had a positive impact in all groups, inhospital beta-blocker administration had a positive impact for groups 2 and 3, and angiotensin-converting enzyme inhibitor/angiotensin receptor blocker and spironolactone had a positive impact on group 3. CONCLUSION: Post-acute coronary syndrome mid-range ejection fraction patients represent an intermediate risk group in which beta-blocker administration was associated with inhospital survival benefit. An invasive strategy was a survival predictor for all groups, regardless of ejection fraction category.

Genetic characterization and genotype-phenotype associations in a large cohort of patients with hypertrophic cardiomyopathy - An ancillary study of the Portuguese registry of hypertrophic cardiomyopathy.

BACKGROUND: We present an ancillary study of the Portuguese Registry of Hypertrophic Cardiomyopathy (PRo-HCM). This is one of the largest HCM genetic studies based on a registry. METHODS AND RESULTS: Collected genetic variants were re-analysed for pathogenicity. Demographic, clinical, imaging and outcome data were analysed for associations with genotype, focusing on comparisons between patients with (G+) vs without (G-) a pathogenic/likely pathogenic (P/LP) variant in one the 9 main causal sarcomeric genes. From the 1042 patients in the registry, 528 (51%) had genetic testing. 152 (28%) were G+ and 98 pts. (19%) had variants of unknown significance. From the patients with the 9 mentioned genes sequenced (424 pts), 14.6% had P/LP variants in MYBPC3, 8.7% MYH7, 4.5% TNNT2, 1.7% TNNI3. Patients were 51 ±â€¯16 years-old, 59% males. Genotype was associated with the following: birthplace (p = 0.005); age (p < 0.001); family history of HCM (p < 0.0005); hypertension (p < 0.0005); chest pain (p = 0.015); pattern of hypertrophy (p = 0.006); left ventricular hypertrophy on the ECG (p < 0.0005); family history of sudden cardiac death (SCD) (p = 0.002). G+ patients more frequently had more than one risk factor for SCD (p = 0.002) and a higher ESC-SCD risk score (p = 0.003). In survival analysis, G+ was associated with SCD (p = 0.017) and MYH7+ with LV systolic dysfunction (p = 0.038). CONCLUSION: Half of the registry patients had genetic testing. Sarcomere-positive patients had distinct demographics, ECG, imaging characteristics and family history and are at increased risk of SCD. The presence of a MYH7 mutation was associated with evolution towards LV systolic dysfunction.

The SHIFT model combines clinical, electrocardiographic and echocardiographic parameters to predict sudden cardiac death in hypertrophic cardiomyopathy.

INTRODUCTION: Limitations have been pointed out in the clinical risk prediction model for sudden cardiac death (SCD) of the European Society of Cardiology (ESC), which is recommended for hypertrophic cardiomyopathy (HCM) patients. The aim of this study was to determine the SCD risk of the HCM patients enrolled in a Portuguese nationwide registry and to develop a new SCD risk prediction model applicable to our population. METHODS AND RESULTS: The cohort consisted of 1022 patients (mean age 53.2±16.4 years, 59% male) enrolled in a Portuguese national HCM registry. During the follow-up period (median five years), 19 patients (1.9%) died suddenly or had aborted SCD or appropriate implantable cardioverter-defibrillator (ICD) shock therapy. Through a Cox proportional hazards model, four variables were independently associated with SCD or equivalent: unexplained Syncope, Heart failure signs, Interventricular septum thickness ≥19 mm and FragmenTed QRS complex. These predictors were included in the SHIFT model and individual risk probabilities of SCD at five years were estimated. This model was internally validated using bootstrapping. The C-index of the SHIFT model was 0.81 (95% CI: 0.77-0.83) and the C-index of the ESC model (performed in a subgroup of 349 HCM patients) was 0.77 (95% CI: 0.73-0.81) (p=0.246). CONCLUSION: The SHIFT model may potentially provide prognostic value and contribute to the clinical decision-making process for ICD implantation for primary prevention of SCD.

Left main and/or three-vessel disease in patients with non-ST-segment elevation myocardial infarction and low-risk GRACE score: Prevalence, clinical outcomes and predictors.

INTRODUCTION: A low-risk GRACE score identifies patients with a lower incidence of major cardiac events, however it can erroneously classify patients with severe coronary artery disease as low-risk. We assessed the prevalence, clinical outcomes and predictors of left main and/or three-vessel disease (LM/3VD) in non-ST-elevation acute myocardial infarction (NSTEMI) patients with a GRACE score of ≤108 at admission. METHODS: Using data from the Portuguese Registry on Acute Coronary Syndromes, 1196 patients with NSTEMI and a GRACE score of ≤108 who underwent coronary angiography were studied. Independent predictors of LM/3VD and its impact on in-hospital complications and one-year mortality were retrospectively analyzed. RESULTS: LM/3VD was present in 18.2% of patients. Its prevalence was higher in males and associated with hypertension, diabetes, previous myocardial infarction, heart failure and peripheral arterial disease (PAD). Although there were no differences in in-hospital complications, these patients had higher mortality (0.9 vs. 0.0%) and more major adverse cardiac and cerebrovascular events (MACCE) (4.1 vs. 2.5%, p=0.172), and higher one-year mortality (2.4 vs. 0.5%, p=0.005). Independent predictors of LM/3VD were age (OR 1.03; 95% CI 1.01-1.0, p=0.003), male gender (OR 2.56; 95% CI 1.56-4.17, p<0.001), heart rate (1.02; 95% CI 1.01-1.03, p<0.001), PAD (OR 3.21; 95% CI 1.47-7.00, p<0.001) and heart failure (OR 3.38; 95% CI 1.02-11.15, p=0.046). CONCLUSIONS: LM/3VD was found in one in five patients. These patients had a tendency for higher in-hospital mortality and more MACCE, and higher one-year mortality. Simple clinical variables could help predict this severe coronary anatomy.

Awareness of Fabry disease in cardiology: A gap to be filled.

INTRODUCTION: In adults, unexplained left ventricular hypertrophy is usually due to sarcomeric hypertrophic cardiomyopathy (HCM). Fabry disease (FD) is rare but may mimic sarcomeric HCM, and has an adverse prognosis in the absence of specific treatment. We aimed to assess cardiologists' awareness of FD based on data from the Portuguese Registry of Hypertrophic Cardiomyopathy. METHODS: A total of 811 index patients, aged 55 ± 16 years, 486 (59.9%) male, were included. Three groups were characterized: A - 128 patients, 74 (57.8%) male, with pathogenic or likely pathogenic mutation(s) in sarcomeric genes; B - 234 patients, 146 (62.4%) male, with negative genetic testing; and C - 449 patients, 266 (59.2%) male, no genetic testing performed. The groups were compared in terms of whether FD was excluded in the registry. Potential red flags for FD were also analyzed and compared between groups. RESULTS: Patients in group A were younger and more frequently had familial HCM (A - 53.9% vs. B - 20.1% vs. C - 18.3%; p <0.001). FD was recorded as excluded in 217 (26.8%), similar in all groups; GLA gene testing was performed in only 50/217 patients (A - 48.6%, B - 25.7%, p = 0.019; C - 13.4%, p = 0.036 for B vs. C), mostly in women (p <0.001) in groups B and C. Alpha-galactosidase A (α-Gal A) activity was assessed in 39/217 (18%) patients, with no difference between groups, but more often in men (p = 0.005). Among patients with potential red flags for FD, only 46.7% underwent specific tests (GLA gene testing and/or α-Gal A activity). When GLA genotyping was performed no mutations were identified. CONCLUSIONS: There is a need to improve cardiologists' alertness for the identification of FD among the Portuguese HCM population.

The Portuguese Registry of Hypertrophic Cardiomyopathy: Overall results.

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

Prior exposure to aspirin in acute coronary syndrome patients: a cardiovascular risk marker or a predictor of adverse outcome? A contemporary data of a national registry.

Despite the known protective cardiovascular effect of aspirin, former studies identified its prior exposure to an acute coronary syndrome (ACS) as an independent risk factor for adverse events. However, those studies did not reflect contemporary approaches. In the current study, we determine whether patients exposed to aspirin before an ACS have a worse cardiovascular risk profile and if it predicts higher risk of recurrent cardiovascular events or mortality. A cohort of patients enrolled in a national registry of ACS was analyzed according to prior exposure to aspirin. A propensity score standardized patients according to baseline comorbidities. Multivariable COX regression analysis was performed in unmatched and matched populations for a primary endpoint (composite of all-cause mortality and/or cardiovascular rehospitalization) and two secondary endpoints (all-cause mortality and cardiovascular rehospitalization, separately) at 1-year follow-up. Among 5533 ACS patients, 1763 were previously exposed to aspirin. They were older and had more comorbidities; contemporary approaches, both coronary angiography and percutaneous coronary angioplasty were less likely to be performed. Before matching the population, prior exposure to aspirin was an independent predictor of primary composite endpoint (p = 0.002) and cardiovascular rehospitalization as the secondary endpoint (p = 0.001). There were no statistically significant differences between both groups in the multivariable model for the primary or secondary endpoints after matching. Previous exposure to aspirin identified ACS patients with worse baseline characteristics, establishing its role as a cardiovascular risk marker. However, our data do not support including aspirin pretreatment in risk stratification scores as an adverse prognostic variable.

Importância do diagnóstico endodôntico frente à displasia cemento-óssea periapical: revisão da literatura / Importance of endodontic diagnosis regarding periapical cemento-osseous dysplasia: literature review

Introdução: a displasia cemento-óssea periapical (DCOP) é uma lesão idiopática benigna mais prevalente na região de incisivos centrais inferiores, em mulheres negras, na faixa etária dos 30 aos 50 anos. Apresenta características radiográficas que podem levar o cirurgião-dentista a um diagnóstico e plano de tratamento equivocados, por ser confundida com periapicopatias. Objetivo: o objetivo do presente artigo foi, por meio de uma revisão de literatura, descrever essa patologia. Métodos: essa revisão foi feita por meio de buscas em duas das principais bases de dados mundiais: PubMed e SciELO. Para isso, foram usados os descritores "periapical cementoosseus dysplasia" e "displasia cemento-óssea periapical", com o objetivo de se avaliar o conteúdo sobre essa temática na literatura atual. Resultados: foram coletados 24 artigos científicos que obedeciam aos seguintes critérios de inclusão: ser uma revisão de literatura ou caso clínico; escrito em língua portuguesa ou inglesa, nos períodos de 1989 a 2016; contemplando a etiologia, características clínicas e radiográficas, diagnóstico, plano de tratamento e prognóstico referentes à displasia cemento-óssea periapical. Conclusão: é importante para o profissional reconhecer os aspectos relevantes da DCOP, a fim de elucidar o diagnóstico diferencial e tratamento e, assim, evitar procedimentos iatrogênicos, tais como terapias endodônticas desnecessárias.

ProACS risk score: An early and simple score for risk stratification of patients with acute coronary syndromes.

INTRODUCTION: There are barriers to proper implementation of risk stratification scores in patients with acute coronary syndromes (ACS), including their complexity. Our objective was to develop a simple score for risk stratification of all-cause in-hospital mortality in a population of patients with ACS. METHODS: The score was developed from a nationwide ACS registry. The development and internal validation cohorts were obtained from the first 31829 patients, randomly separated (60% and 40%, respectively). The external validation cohort consisted of the last 8586 patients included in the registry. This cohort is significantly different from the other cohorts in terms of baseline characteristics, treatment and mortality. Multivariate logistic regression analysis was used to select four variables with the highest predictive potential. A score was allocated to each parameter based on the regression coefficient of each variable in the logistic regression model: 1 point for systolic blood pressure ≤116 mmHg, Killip class 2 or 3, and ST-segment elevation; 2 points for age ≥72 years; and 3 points for Killip class 4. RESULTS: The new score had good discriminative ability in the development cohort (area under the curve [AUC] 0.796), and it was similar in the internal validation cohort (AUC 0.785, p=0.333). In the external validation cohort, there was also excellent discriminative ability (AUC 0.815), with an adequate fit. CONCLUSIONS: The ProACS risk score enables easy and simple risk stratification of patients with ACS for in-hospital mortality that can be used at the first medical contact, with excellent predictive ability in a contemporary population.

Risk factor paradox in the occurrence of cardiac arrest in acute coronary syndrome patients. / Paradoxo dos fatores de risco na ocorrência de parada cardiorrespiratória em pacientes com síndrome coronária aguda.

OBJECTIVE:: To compare patients without previously diagnosed cardiovascular risk factors) and patients with one or more risk factors admitted with acute coronary syndrome. METHODS:: This was a retrospective analysis of patients admitted with first episode of acute coronary syndrome without previous heart disease, who were included in a national acute coronary syndrome registry. The patients were divided according to the number of risk factors, as follows: 0 risk factor (G0), 1 or 2 risk factors (G1 - 2) and 3 or more risk factors (G ≥ 3). Comparative analysis was performed between the three groups, and independent predictors of cardiac arrest and death were studied. RESULTS:: A total of 5,518 patients were studied, of which 72.2% were male and the mean age was 64 ± 14 years. G0 had a greater incidence of ST-segment elevation myocardial infarction, with the left anterior descending artery being the most frequently involved vessel, and a lower prevalence of multivessel disease. Even though G0 had a lower Killip class (96% in Killip I; p < 0.001) and higher ejection fraction (G0 56 ± 10% versus G1 - 2 and G ≥ 3 53 ± 12%; p = 0.024) on admission, there was a significant higher incidence of cardiac arrest. Multivariate analysis identified the absence of risk factors as an independent predictor of cardiac arrest (OR 2.78; p = 0.019). Hospital mortality was slightly higher in G0, although this difference was not significant. By Cox regression analysis, the number of risk factors was found not to be associated with mortality. Predictors of death at 1 year follow up included age (OR 1.05; p < 0.001), ST-segment elevation myocardial infarction (OR 1.94; p = 0.003) and ejection fraction < 50% (OR 2.34; p < 0.001). CONCLUSION:: Even though the group without risk factors was composed of younger patients with fewer comorbidities, better left ventricular function and less extensive coronary disease, the absence of risk factors was an independent predictor of cardiac arrest.