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Neurohormonal blockade (NB)/modulation is the combination of two renin-angiotensin-aldosterone system inhibitors (RAASi) with a beta blocker. It is the core therapy for heart failure with reduced ejection fraction (HFrEF). While improving long term prognosis, it also induces hyperkalemia (serum KWe reviewed the medical records of HFrEF patients attending a HF clinic at a tertiary Portuguese hospital during 2018 (n=240). The number of patients not tolerating maximal neurohormonal blockade/modulation due to hyperkalemia was determined. The incidence and characteristics of hyperkalemia episodes were also assessed.Only six patients (3%) achieved maximal doses of neurohormonal blockade/modulation. Hyperkalemia was the limiting factor in 48 (20%) patients. A total of 185 hyperkalemia episodes occurred in 100 (42%) patients. Forty-five (24%) episodes were moderate or severe (serum KIn daily clinical practice, hyperkalemia is frequent and limits neurohormonal blockade/modulation by leading to the withholding or reducing of the intensity of RAAS inhibition. Considering the negative prognostic impact associated with sub-optimal neurohormonal blockade/modulation, addressing hyperkalemia is an important issue when treating HFrEF patients.

Background and aim Frailty Phenotype (FP) is very common in Heart Failure (HF) and both syndromes worsen one another. The aim of this study is to first describe FP in a sample of Portuguese HF patients, and to analyse its association with nutritional and clinical statuses, namely muscle mass, obesity and functional class. Methods and results In this cross-sectional study, a sample of 136 heart failure outpatients (24-81 years, 33.8% women) was randomly selected from the appointments’ listings of a HF and Transplant clinic in a Portuguese University Hospital. FP was assessed according to Fried et al.; muscle mass was estimated from mid-upper arm muscle circumference; weight status was assessed using body mass index; HF functional classes were registered. The association between participants’ characteristics and FP categories was analysed using logistic ordinal regression. The frequency of pre-frailty and frailty is 57.4% and 15.4%, respectively. Within frail individuals, 52.4% were under the age of 65. In multivariable analysis, frailty was positively associated with being 70 or older (OR = 3.44) and obese (OR = 2.66), and negatively associated with muscle mass (OR = 0.77) and with being at class I (OR = 0.14) or class II (OR = 0.29) of HF functional classes. Conclusion Muscle mass seems to be an important predictor of frailty in HF patients and should be taken into account when designing intervention plans that allow for reverting or modifying frailty and pre-frailty. Younger patients should be monitored for the presence and evolution of FP.

Background: Inflammatory responses play an important role in the pathophysiology of cardiogenic shock (CS). The aim of this study was to investigate the kinetics of procalcitonin (PCT), C-reactive protein (CRP), and interleukin-6 (IL-6) in CS and to assess their relation to clinical presentation, other biochemical variables, and prognosis. Methods: Levels of PCT, CRP and IL-6 were analyzed in serial plasma samples (0-120h) from 183 patients in the CardShock study. The study population was dichotomized by PCTmax >= and < 0.5 mu g/L, and IL-6 and CRPmax above/below median. Results: PCT peaked already at 24 h [median PCTmax 0.71 mu g/L (IQR 0.24-3.4)], whereas CRP peaked later between 48 and 72 h [median CRPmax 137mg/L (59-247)]. PCT levels were significantly higher among non-survivors compared with survivors from 12 h on, as were CRP levels from 24 h on (p < 0.001). PCTmax >= 0.5 mu g/L (60% of patients) was associated with clinical signs of systemic hypoperfusion, cardiac and renal dysfunction, acidosis, and higher levels of blood lactate, IL-6, growth-differentiation factor 15 (GDF-15), and CRPmax. Similarly, IL-6 > median was associated with clinical signs and biochemical findings of systemic hypoperfusion. PCTmax >= 0.5 mu g/L and IL-6 > median were associated with increased 90-day mortality (50% vs. 30% and 57% vs. 22%, respectively; p < 0.01 for both), while CRPmax showed no prognostic significance. The association of inflammatory markers with clinical infections was modest. Conclusions: Inflammatory markers are highly related to signs of systemic hypoperfusion in CS. Moreover, high PCT and IL-6 levels are associated with poor prognosis. (C) 2020 Elsevier B.V. All rights reserved.

Renin-angiotensin-aldosterone system (RAAS) inhibitors are a cornerstone in the treatment of heart failure with reduced ejection fraction (HFrEF). Sacubitril/valsartan modulates the neurohormonal axis by inhibiting both angiotensin receptors and neprilysin, and improves neurohormonal balance more than blocking the RAAS alone.The PARADIGM-HF trial validated this new treatment option for patients with HFrEF. Sacubitril/valsartan was also more effective than enalapril in slowing disease progression by decreasing the risk of worsening heart failure requiring hospitalization or emergency admission and the need for intensified therapy, heart failure devices or cardiac transplantation. More than 70% of patients included in PARADIGM-HF were in NYHA class II, and overall, the results indicate that sacubitril/valsartan should be started in the earliest symptomatic stages of the disease.As PARADIGM-HF has excellent robustness for a cardiovascular trial, sacubitril/valsartan has been included as a new treatment option with a strong level of recommendation in the main international guidelines.This expert task force proposes a practical guide to the use of this new drug that has been endorsed by the Working Group on Heart Failure of the Portuguese Society of Cardiology. Resumo: Os inibidores do sistema renina-angiotensina-aldosterona são uma das pedras basilares no tratamento da insuficiência cardíaca com fração de ejeção reduzida. O sacubitril/valsartan promove a modulação neuro-hormonal, bloqueando os recetores da angiotensina e inibindo a neprilisina, e produz um maior equilíbrio neuro-hormonal, mais do que o bloqueio do sistema renina-angiotensina-aldosterona isoladamente. O estudo PARADIGM-HF validou essa nova opção para o tratamento de doentes com insuficiência cardíaca e fração de ejeção reduzida, em alternativa ao IECA/ARA.O sacubitril/valsartan demonstrou ser mais eficaz do que o enalapril em retardar a progressão da doença, diminuindo o risco de agravamento da insuficiência cardíaca através da diminuição da necessidade de hospitalização e da menor necessidade de intensificação terapêutica, dispositivos ou transplante cardíaco. Mais de 70% dos doentes incluídos no estudo PARADIGM-HF estavam em classe II da NYHA, suportando a utilização do sacubitril/valsartan precocemente após o início dos sintomas.Como o estudo PARADIGM-HF apresentou uma robustez sem precedentes para um estudo cardiovascular, o sacubitril/valsartan foi incluído como uma nova opção de tratamento nas Recomendações internacionais mais relevantes, com um elevado nível de evidência (I-B).Este grupo de peritos em Insuficiência Cardíaca vem propor uma orientação prática para a utilização deste novo fármaco, subscrita pelo Grupo de Estudos de Insuficiência Cardíaca da Sociedade Portuguesa de Cardiologia. Keywords: Sacubitril/valsartan, PARADIGM-HF, Practical guidance, Heart failure with reduced ejection fraction, Palavras-chave: Sacubitril/valsartan, PARADIGM-HF, Guia prático, Insuficiência cardíaca com fração de ejeção reduzida

Introduction: Dilated cardiomyopathy (DCM) is a disease of the heart muscle characterized by ventricular dilatation and impaired systolic function. Familial forms account for 30-50% of cases. Autosomal dominant inheritance is the predominant pattern of transmission. Causal genetic variants have been identified in several genes and molecular diagnosis has implications for genetic counseling and risk stratification. Objective: We aimed to estimate the frequency of genetic variants and the molecular basis of DCM in Portugal. Methods: We performed a multicenter study of unrelated patients, recruited between 2013 and 2014. Variants in 15 genes were screened using PCR with direct sequencing (next-generation sequencing with at least 30-fold coverage combined with Sanger sequencing). Results: A total of 107 patients were included, 64 (60%) men, mean age at diagnosis 38±13 years, with 48 (45%) familial cases. In total, 31 rare variants in eight genes (mainly in MYBPC3, TNNT2 and LMNA) were identified, in 28 patients (26%). Only four variants had been previously described in association with DCM, 11 with hypertrophic cardiomyopathy, and nine variants were novel. Four variants were likely pathogenic and the remainder were of uncertain significance. We found no major differences in the main clinical and imaging characteristics between patients with or without rare variants and patients with likely pathogenic variants. Conclusions: Our results reflect the complexity and diversity of DCM genetics. For better interpretation of the pathogenicity of the variants found and their causative roles in DCM, molecular cascade screening of families is imperative. Further insight into genotype-phenotype correlations and risk stratification is desirable. Resumo: Introdução: A miocardiopatia dilatada (MCD) é uma doença do músculo cardíaco caracterizada por dilatação ventricular e compromisso da função sistólica. As formas familiares são responsáveis por 30 a 50% dos casos. O padrão de hereditariedade predominante é o autossómico dominante. Variantes genéticas causais foram identificadas em vários genes e o diagnóstico molecular tem implicações para o aconselhamento genético e estratificação de risco. Objetivo: Avaliar a base molecular da MCD em Portugal. Métodos: Estudo multicêntrico de doentes não relacionados, recrutados entre 2013 e 2014. Foram analisados 15 genes, através da técnica de PCR com sequenciação direta (NGS com pelo menos uma cobertura de 30 vezes combinada com sequenciação de Sanger). Resultados: Foram incluídos 107 pacientes, 64 (60%) homens, idade média ao diagnóstico de 38 ± 13 anos, com 48 (45%) casos familiares. Foram identificadas 31 variantes raras, em oito genes, (principalmente MYBPC3, TNNT2 e LMNA) em 28 pacientes (26%). Apenas quatro variantes tinham sido previamente descritas em associação com MCD, 11 com miocardiopatia hipertrófica e nove variantes eram novas. Quatro variantes foram classificadas como provavelmente patogénicas e as restantes de significado incerto. Não encontrámos diferenças significativas nas principais características clínicas e imagiológicas entre doentes com ou sem variantes raras e doentes com variantes provavelmente patogénicas. Conclusões: Estes resultados refletem a complexidade e diversidade genética da MCD. Para uma melhor interpretação da patogenicidade das variantes e potencial causalidade, o rastreio molecular das famílias é imperativo. Uma visão mais aprofundada das correlações genótipo-fenótipo e da estratificação de risco é desejável. Keywords: Dilated cardiomyopathy, Genetic variant, Next-generation sequencing, Palavras-chave: Miocardiopatia dilatada, Variante genética, Sequenciação massiva paralela

Background High amounts of coronary artery calcium limit image quality and diagnostic accuracy of multidetector computed tomography (MDCT) angiography (CTA) regarding the assessment of obstructive coronary artery disease (CAD). CT myocardial perfusion imaging may represent an opportunity to overcome this limitation. Objective To explore the additive value of CT myocardial perfusion to CTA depending on the patient's calcium score and in comparison to the reference standard of invasive coronary angiography plus fractional flow reserve (FFR) measurement. Methods Symptomatic patients with intermediate pretest probability of CAD were prospectively recruited and underwent both cardiac MDCT (64-slice scanner, retrospectively-gated stress–rest protocol) and invasive coronary angiography including FFR assessment. We defined hemodynamically significant CAD by the presence of occlusive or subocclusive (99%) stenosis, >50% stenosis in left main or FFR≤0.80. Stress CT myocardial perfusion imaging was performed in all patients in addition to CTA. The additive value of CT myocardial perfusion to rule in or rule out the presence of hemodynamically relevant stenosis on a per-patient basis was assessed and analyzed relative to the patient's calcium score. Results 95 patients were included in the analysis (62 ± 8.2 years, 68%males). Hemodynamically significant CAD was present in 42 patients. Sixty-four patients had a fully evaluable CTA examination. Per-patient, CTA alone had a sensitivity, specificity and AUC of 100%, 59% and 0.79 respectively (77% patients correctly classified). Adding CT myocardial perfusion to evaluate uninterpretable vessel territories in CTA in 66 patients with a calcium score>100 yielded a sensitivity of 88%, a specificity of 74% and an AUC of 0.81 (81% patients correctly classified), and in 52 patients with a calcium score>400 sensitivity was 91%, specificity 70%, and AUC 0.80 (82% patients correctly classified, p = 0.733 versus using perfusion imaging in all patients). Conclusions From a pragmatic standpoint, limiting the use of CT perfusion to individuals with a calcium score above 400 might be a feasible strategy to optimize the diagnostic accuracy of CT imaging for diagnosis of obstructive CAD.

Introduction and objective: The prognostic value of myocardium deformation measurements in chronic heart failure (CHF) is still poorly addressed. The purpose of this study was to evaluate the correlation of left ventricular (LV) global longitudinal strain (GLS) with clinical and prognostic indicators in patients with CHF and systolic dysfunction. Methods: Ambulatory patients with CHF and LV ejection fraction (LVEF)

Objectives This study sought to compare computed tomography delayed enhancement (CTDE) against cardiac magnetic resonance (CMR) late gadolinium enhancement (LGE) for detection of ischemic scar and to test the additive value of CTDE as part of a comprehensive multidetector computed tomography (MDCT) stress–rest protocol including computed tomography perfusion (CTP) and computed tomography angiography (CTA) for the diagnosis of significant coronary artery disease (CAD). Background CTDE has been recently described as a promising tool for noninvasive detection of myocardial scar, similarly to CMR-LGE techniques. Despite its theoretical potential as an adjunctive tool to improve MDCT accuracy for detection of CAD, its clinical performance has not been validated. Methods One hundred five symptomatic patients with suspected CAD (age 62.0 ± 8.0 years, 67% men) underwent MDCT, CMR, and x-ray invasive coronary angiography. The MDCT protocol consisted of calcium scoring, stress CTP under adenosine 140 μg/kg/min, rest CTP + CTA, and a low-dose radiation prospective scan for detection of CTDE. CMR-LGE was used as the reference standard for assessment of scar. Functionally significant CAD was defined as the presence of ≥90% stenosis/occlusion or fractional flow reserve measurements ≤0.80 in vessels >2 mm. Results CTDE had good accuracy (90%) for ischemic scar detection with low sensitivity (53%) but excellent specificity (98%). Positive and negative predictive values were 82% and 91%, respectively. On a patient-based model, MDCT protocol without integration of CTDE results had a sensitivity, specificity, and positive and negative predictive values of 90%, 81%, 80%, and 90%, respectively, for the detection of functionally significant CAD. Addition of CTDE results did not improve MDCT performance (90%, 77%, 77%, and 90%, respectively). Conclusions CTDE has moderate accuracy for detection of ischemic scar in patients with suspected CAD. Integration of CTDE into a comprehensive MDCT protocol including stress–rest CTP and CTA does not improve MDCT accuracy for detection of significant CAD in intermediate-to-high pre-test probability populations.

Transcatheter aortic valve implantation (TAVI) techniques have been presenting good procedural success and favorable clinical outcomes. However, optimal management of aortic valve disease in elderly patients depends on quality of life (QoL) improvement. In this study we aimed to evaluate changes in QoL in patients referred for TAVI.Prospective analysis of 74 consecutive patients (34 male), aged 81.6 ± 8 years with symptomatic severe aortic valve stenosis (AS) ineligible for conventional aortic valve replacement, referred to TAVI in one tertiary center. For the assessment of QoL, the Minnesota Living with Heart Failure Questionnaire (MLHFQ) was used before the procedure and at 6.5 months. The mortality was 9.5% at 30 days and 20.2% at 6.5 months follow-up. Fifty three (71.6%) patients completed MLHFQ at baseline and at follow-up. All patients showed good hemodynamic results and no signs of prosthesis dysfunction were observed on transthoracic echocardiography. The New York Heart Association (NYHA) class (2.9 ± 0.4 to 1.4 ± 0.7; p0.001), and the MLHFQ scores [overall (37.0 ± 14.7 vs. 14.4 ± 10.1; p0.001), physical dimension (23.2 ± 9.5 vs. 8.6 ± 5.9; p0.001) and emotional dimension (5.4 ± 4.2 vs. 2.6 ± 3.0; p0.001)] were significantly improved 6.5 months after TAVI. Patients with peripheral vascular disease (PVD) had an inferior improvement in QoL caused by a lower enhancement in physical dimension MLHFQ score (mean difference: -17.0 ± 10.2 vs. -10.1 ± 11.5; p=0.036).TAVI significantly improves symptoms and QoL in patients with severe AS and high surgical risk. Patients with PVD might be expected to have a less impressive improvement in QoL after TAVI.

Familial dilated cardiomyopathy (FDCM) is characterized by genetic heterogeneity, incomplete age-dependent penetrance, and a multifactorial pathogenesis ([1][1]). Diagnosis is still dependent on clinical criteria and familial investigation ([2][2]). On the other hand, several abnormalities have been

We evaluated left ventricular function by echocardiography in a prospective study that included 98 consecutive human immunodeficiency virus (HIV)-infected patients and 40 HIV-seronegative normal controls. When compared with controls, HIV patients showed increased isovolumic relaxation time (101+/-18 ms versus 71+/-10 ms; p

We evaluated the effect of chronic Pneumocystis carinii pneumonia (PCP) prophylaxis, with a once a month dose of 300 mg of inhalatory pentamidine isethionate, on QT interval duration. We included 22 human immunodeficiency virus (HIV)-infected patients: 11 were on this medication and 11 were not. The two groups were matched for age, sex and HIV infection stage. No patient had any clinical condition or was under any medication known to affect the duration of the QT interval. The heart rate-corrected QT (QTc) was obtained by averaging the observations of three independent observers. QTc duration was similar in both groups. The time separating pentamidine administration and the performance of the ECG did not influence the results, neither did the duration of inhalatory pentamidine therapy. Our results suggest that inhalatory pentamidine does not prolong the QT interval duration and so, as opposed to what has been reported concerning intravenous pentamidine therapy, does not seem to induce an increased risk of torsades de pointes.

We prospectively studied, with 24-h Holter monitoring, 21 consecutive human immunodeficiency virus (HIV) infected patients, at all stages of the infection, in order to assess their dysrhythmic profile. Three (14.3%) patients presented one isolated run of supraventricular tachycardia, with10 beats, that was considered clinically irrelevant. No patient presented other clinically relevant supraventricular or ventricular tachy or bradydysrhythmias. One (4.8%) patient presented intermittent Mobitz type I second-degree AV block, two (9.5%) patients paroxistic 2:1 AV block and one (4.8%) patient presented a bifascicular block on the 12-lead ECG that persisted during the ambulatory recording. This study suggests that clinically relevant cardiac tachydysrhythmias are rare in our population of HIV infected patients. On the other hand we found an unexpectedly high incidence of cardiac impulse conduction disturbances.