Pulmonary Artery Aneurysm Associated with Sarcoidosis in a 75-year-old with Heart Failure: The Challenges of Diagnosis and Management.

Introduction: Pulmonary artery aneurysm (PAA) is a rare abnormality of pulmonary vasculature. It can be idiopathic or secondary to various pathologies, frequently with multiple factors leading to its formation. We report the case of a man with concomitant sarcoidosis and PAA. Case description: A 75-year-old male with a diagnosis of pulmonary sarcoidosis was referred to the Cardiology department due to heart failure with reduced left ventricular ejection fraction (LVEF). The transthoracic echocardiogram revealed mildly reduced LVEF, aortic root and pulmonary artery (PA) dilatation, and no signs of pulmonary hypertension (PH). Cardiac magnetic resonance imaging was performed, revealing mild left ventricular dilation, LVEF of 40%, main PA dilation (43 mm) and a pattern of late gadolinium enhancement suggestive of cardiac sarcoidosis. At follow-up, a thoracic computed tomography (CT) angiography scan revealed ascending aorta ectasia and giant main PA aneurysm (60 mm). A right heart catheterisation was performed, and a mean PA pressure of 34 mmHg was obtained. Given the clinical context, the patient was considered to have PH due to lung disease and left heart disease, and PAA was possible due to vascular granulomatous involvement by sarcoidosis. Conclusion: PAA is a rare finding and mostly occurs in the setting of PH. Sarcoidosis is a granulomatous disease that mostly affects the lungs, but the sarcoid involvement of great vessels has been described. In this clinical case, the probable cause for the PA fragility leading to aneurysm formation remains sarcoid vascular infiltration, regarding the discrepancy between the PA dimensions and mildly elevated PA pressure. LEARNING POINTS: Pulmonary artery aneurysm is a rare abnormality of pulmonary vasculature that can be idiopathic or a consequence of pulmonary hypertension, congenital heart disease, infection, vasculitis or collagenopathies.Due to the low incidence of this disease, there are no guidelines for its diagnosis, management or follow-up, and treatment is based on the underlying aetiology, aneurysm dimensions and occurrence of symptoms.Sarcoidosis is a multisystem disorder of unknown aetiology characterised by non-caseating granulomas that mostly involve the lungs, but can also affect skin, eyes, and lymph nodes. Sarcoid involvement of great vessels has been rarely described.

Rheumatoid Arthritis and Pericardial Effusion - A Unique Major Manifestation.

Introduction: Pericardial effusion is common in the setting of rheumatoid arthritis (RA); however, it is rarely its first manifestation. Case description: An 82-year-old male presented with abdominal pain, vomiting and fever. Blood analysis revealed elevated systemic inflammatory markers, and an abdominal computed tomography scan revealed non-specific alveolar condensation of the right pulmonary base and pericardial effusion subsequently quantified as medium size by transthoracic echocardiography. A large aetiological panel was requested, with the autoimmunity study revealing high levels of rheumatoid factor (RF) and anti-citrullinated cyclic peptide (anti-CCP) antibodies. Since the patient did not present articular involvement, the initial hypothesis was pericardial effusion due to pneumonia and no specific treatment for RA was started. At follow-up, the pericardial effusion recurred and a pericardiocentesis was performed. The pericardial fluid analysis was sterile, and no malignant cells were identified. A new serological study confirmed high levels of RF and anti-CCP antibodies, and immunomodulatory treatment was initiated. After one year, the pericardial effusion recurred due to non-compliance with immunomodulatory therapy. A surgical pleuro-pericardial window was performed, and the cytological study of the pericardial patch revealed submesothelial thickening and foci of perivascular lymphocytic infiltrate. The patient remained asymptomatic. Discussion: After exclusion of a large spectrum of infectious and non-infectious causes and the relapse after suspension of immunomodulatory treatment, the most probable aetiology for the pericardial effusion remains RA. Conclusion: Pericardial syndromes can be the first manifestation of AR even in the absence of articular symptoms and this disease must be considered in the aetiological investigation. LEARNING POINTS: The occurrence of pericardial effusion in the setting of rheumatoid arthritis (RA) is a usual finding but this form of extra-articular manifestation is possibly the first and only presentation of the disease.In the case of recurrent pericardial effusion, the diagnosis of RA must be considered in the aetiological investigation even in the absence of more common manifestations of the disease.

Portuguese Heart Failure Prevalence Observational Study (PORTHOS) rationale and design - A population-based study.

INTRODUCTION AND OBJECTIVES: Current epidemiological data on heart failure (HF) in Portugal derives from studies conducted two decades ago. The main aim of this study is to determine HF prevalence in the Portuguese population. Using current standards, this manuscript aims to describe the methodology and research protocol applied. METHODS: The Portuguese Heart Failure Prevalence Observational Study (PORTHOS) is a large, three-stage, population-based, nationwide, cross-sectional study. Community-dwelling citizens aged 50 years and older will be randomly selected via stratified multistage sampling. Eligible participants will be invited to attend a screening visit at a mobile clinic for HF symptom assessment, anthropomorphic assessment, N-terminal pro-B-type natriuretic peptide (NT-proBNP) testing, one-lead electrocardiogram (ECG) and a sociodemographic and health-related quality of life questionnaire (EQ-5D). All subjects with NT-proBNP ≥125 pg/mL or with a prior history of HF will undergo a diagnostic confirmatory assessment at the mobile clinic composed of a 12-lead ECG, comprehensive echocardiography, HF questionnaire (KCCQ) and blood sampling. To validate the screening procedure, a control group will undergo the same diagnostic assessment. Echocardiography results will be centrally validated, and HF diagnosis will be established according to the European Society of Cardiology HF guidelines. A random subsample of patients with an equivocal HF with preserved ejection fraction diagnosis based on the application of the Heart Failure Association preserved ejection fraction diagnostic algorithm will be invited to undergo an exercise echocardiography. CONCLUSIONS: Through the application of current standards, appropriate methodologies, and a strong research protocol, the PORTHOS study will determine the prevalence of HF in mainland Portugal and enable a comprehensive characterization of HF patients, leading to a better understanding of their clinical profile and health-related quality of life.

conTemporary reflectiOns regarding heart failure manaGEmenT - How to ovERcome the PorTuguese barriers (TOGETHER-PT).

INTRODUCTION AND OBJECTIVES: Heart failure (HF) is a complex clinical syndrome that is a significant burden in hospitalisations, morbidity, and mortality. Although a significant effort has been made to better understand its consequences and current barriers in its management, there are still several gaps to address. The present work aimed to identify the views of a multidisciplinary group of health care professionals on HF awareness and literacy, diagnosis, treatment and organization of care, identifying current challenges and providing insights into the future. METHODS: A steering committee was established, including members of the Heart Failure Study Group of the Portuguese Society of Cardiology (GEIC-SPC), the Heart Failure Study Group of the Portuguese Society of Internal Medicine (NEIC-SPMI) and the Cardiovascular Study Group (GEsDCard) of the Portuguese Association of General and Family Medicine (APMGF). This steering committee produced a 16-statement questionnaire regarding different HF domains that was answered to by a diversified group of 152 cardiologists, internists, general practitioners, and nurses with an interest or dedicated to HF using a five-level Likert scale. Full agreement was defined as ≥80% of level 5 (fully agree) responses. RESULTS: Globally, consensus was achieved in all but one of the 16 statements. Full agreement was registered in seven statements, namely 3 of 4 statements for patient education and HF awareness and 2 in 4 statements of both HF diagnosis and healthcare organization, with proportions of fully agree responses ranging from 82.9% to 96.7%. None of the HF treatment statements registered full agreement but 3 of 4 achieved ≥80% of level 4 (agree) responses. CONCLUSION: This document aims to be a call-to-action to improve HF patients' quality of life and prognosis, by promoting a change in HF care in Portugal.

Triple-Valve Infective Endocarditis: A Case with Unusual Features.

Infective endocarditis is a condition associated with high morbidity and mortality, usually with univalvular involvement. We describe the case of a 76-year-old woman with triple-valve endocarditis due to Streptococcus gallolyticus, complicated by perivalvular suppurative lesions, acute heart failure and acute kidney injury. Unfortunately, the patient died despite antibiotic therapy and emergent surgery. This case highlights uncommon triple-valve involvement in the absence of risk factors, posing a diagnostic and therapeutic challenge. LEARNING POINTS: Native triple-valve endocarditis is extremely rare, especially in the absence of predisposing conditions.Streptococcus gallolyticus has been associated with endocarditis as well colonic and hepatobiliary pathology, so gastrointestinal endoscopy is important as bacteraemia frequently precedes gastrointestinal symptoms, allowing prompt diagnosis.In multivalvular involvement, early surgery is often required, and timely recognition and treatment before complications develop may be decisive for prognosis.

SGLT-2 inhibitors: A step forward in the treatment of heart failure with reduced ejection fraction.

Heart failure (HF) is a major health problem with a significant impact on morbidity, mortality, quality of life and healthcare costs. Despite the positive impact of disease-modifying therapies developed over the last four decades, HF mortality and hospitalization remain high. We aim at reviewing the evidence supporting the use of sodium-glucose co-transporter-2 (SGLT-2) inhibitors, as a novel strategy for HF with reduced ejection fraction (HFrEF) treatment. The consistent observation of a reduction in HF hospitalizations in type-2 diabetes cardiovascular safety trials EMPA-REG OUTCOME, CANVAS, DECLARE-TIMI 58 and VERTIS raised the hypothesis that SGLT-2 inhibitors could have an impact in HF treatment. This hypothesis was first confirmed in 2019 with the DAPA-HF publication showing that dapagliflozin on top of optimized HFrEF therapy, reduced HF-hospitalizations and cardiovascular mortality. This was reinforced by the EMPEROR-Reduced publication in 2020 showing that empagliflozin on top of optimized HFrEF therapy, reduced HF-hospitalizations. Both studies established SGLT-2 inhibitors as a fourth pillar of HFrEF prognosis-modifying therapy, in addition to the gold standard triple neurohormonal modulation/blockade.

Development and validation of a liquid chromatography method using UV/fluorescence detection for the quantitative determination of metabolites of the kynurenine pathway in human urine: Application to patients with heart failure.

Recent evidence indicates the relevant role of the tryptophan (TRP) metabolites in the pathophysiology of cardiovascular diseases via inflammatory and oxidative stress mechanisms. Therefore, quantification of TRP and its metabolites in biological samples can be a powerful tool to elucidate the disease mechanisms. The aim of this work was to develop and validate a liquid chromatography with ultraviolet (UV) and fluorescence detection (FD) (LCUV/FD) method for the quantification of TRP and its metabolites (L-kynurenine (KYN) and kynurenic acid (KA)) in urine samples from heart failure (HF) patients. Biochemical parameters and inflammatory markers were quantified, and data correlated with urinary concentrations of TRP and its metabolites. Optimized chromatographic conditions were achieved using a Luna® 3 µm PFP(2) analytical column, a mobile phase of 20 mM of ammonium formate in ultra-pure water (with 0.01 % of formic acid), acetonitrile and ethanol (95/2/3, v/v/v), a flow rate of 0.7 mL/min and a column oven temperature set at 25 °C. The method was validated according to the European Medicines Agency (EMA) guidelines and showed to be linear (r2 >0.99), accurate (82-116%) and precise (%RSD below 15 %). The limits of quantification varied between 50 and 125 ng/mL. The method was applied to the quantification of TRP, KYN and KA in healthy volunteers and male HF patients. The results obtained through this pilot study (small group of patients) showed a relationship between biochemical parameters, inflammatory markers and changes in the concentration of TRP, KYN and KA. The KYN/TRP and KA/KYN ratios were calculated. Results support the hypothesis that KYN/TRP ratio is related with enzymatic activity and that KA/KYN ratio can be a good neuroprotection indicator. The potential of the LCUV/FD method for the monitoring of the selected compounds in cardiac patients was also demonstrated.

Acute Aortic Regurgitation Due to Endocarditis Caused by Disseminated Gonococcal Infection: A Case Report.

ABSTRACT: A 23-year-old man presented with cough and progressive shortness of breath. Echocardiogram showed a biscupid aortic valve with a large vegetation causing severe regurgitation. Blood cultures were positive for Neisseria gonorrhoeae sensitive to cefotaxime and penicillin. Despite direct antibiotherapy, the patient required cardiac surgery with aortic valve replacement.

From left ventricular ejection fraction to cardiac hemodynamics: role of echocardiography in evaluating patients with heart failure.

In clinical practice heart failure (HF) patients are generally classified on the basis of left ventricular (LV) ejection fraction. This approach, however, has important limitations. According to the definition of HF as a clinical syndrome that results from any impairment of LV filling or ejection of blood, a more articulated hemodynamic categorization of HF patients taking into account both LV forward flow and filling pressure would be desirable. However, the reliability of hemodynamic measures using echocardiographic techniques, which are the most used in current clinical practice for evaluation of HF patients, needs to be clarified. The aim of this article, therefore, is to verify whether echocardiography has acceptable feasibility, accuracy and reproducibility for the noninvasive evaluation of LV hemodynamics. This evaluation is necessary to progress to a hemodynamic characterization of HF patients that would ultimately overcome the HF classification based on ejection fraction.

Importance of three-dimensional speckle tracking in the assessment of left atrial and ventricular dysfunction in patients with myotonic dystrophy type 1.

INTRODUCTION AND AIM: Myotonic dystrophy type 1 (DM1) is a multisystem disease in which cardiac involvement is common. The aim of this study was to identify early changes in left atrial (LA) mechanics and left ventricular (LV) systolic function in patients with myotonic dystrophy type 1 using three-dimensional (3D) speckle tracking echocardiography (3D-STE). METHODS: This observational study included 25 patients with DM1 and 25 healthy volunteers. We assessed LA and LV global strain parameters using 3D-STE. RESULTS: Patients with DM1 showed significantly lower longitudinal LA strain (22.85%±5.06 vs. 26.82%±5.15; p=0.008 in univariate analysis and p=0.026 in multivariate analysis) and global LV longitudinal strain (-13.55%±1.82 vs. -16.11%±1.33; p<0.001 in univariate analysis and p<0.001 in multivariate analysis), which was not observed with LA area tracking (p=0.412) or LV global circumferential strain (p=0.879), global radial strain (p=0.058), area tracking (p=0.092) or twist (p=0.992). CONCLUSION: LA and LV global longitudinal strain is significantly decreased in patients with DM1, which may be an early marker of subclinical dysfunction in these patients.

Interpretation of B-type natriuretic peptides in the era of angiotensin receptor-neprilysin inhibitors. / Interpretação dos peptídeos natriuréticos tipo B na era dos antagonistas da neprilisina/recetores da angiotensina (ARNIs).

Assessment of serum levels of natriuretic peptides, especially the amino-terminal portion (NT-proBNP) and the carboxy-terminal portion (BNP) of pro-B-type natriuretic peptide, has had a highly significant clinical impact on the diagnosis and prognostic stratification of patients with heart failure (HF). They are now an instrument with recognized value in this context and several studies have demonstrated their value in tailoring therapy for these patients. Following the recent advent of angiotensin receptor-neprilysin inhibitors (ARNIs), there is a need to review how these two biomarkers are interpreted in HF. The use of ARNIs is associated with a reduction in NT-proBNP but an increase in BNP levels. The authors of this concise article review the interpretation of natriuretic peptide levels in the light of the most recent evidence.

Caracterización epidemiológica del zika en mujeres atendidas en el distrito 09D01 de Guayaquil / Epidemiological characterization of zika in women attended in the district 09D01 of Guayaquil

Introducción. El virus del Zika es un flavivirus que es transmitido por mosquitos principalmente del género Aedes. Aproximadamente el 80 % de las personas infectadas no presenta síntomas. Objetivo. Caracterizar epidemiológicamente la enfermedad del Zika, en mujeres atendidas en el distrito 09D01 durante enero a junio del 2017. Metodología. Fue de diseño no experimental, descriptivo, analítica de corte transversal en mujeres atendidas en la consulta externa. Resultados. Se obtuvo el grupo mayormente afectado de 30 - 39 años con 30,41 %, 20 - 29 años con 29,90 %, entre otros; pero en las embarazadas, fue el grupo etario de 20 ­ 29 años con 47,42 %, seguido del 30 ­ 39 años de edad con 40,21 %, entre otros. En el caso de las no embarazadas se observó en edades de 0 a 9 años, 10 a 19 años y en más de 50 años. En cuanto a las embarazadas, mayormente fueron en el segundo trimestre con 48,44 %. Los resultados de laboratorio dio positivo el 98,97 % de los casos con PCR y nexo epidemiológico 1,03 %; siendo el Hospital Materno Infantil y Centro de Salud N.° 3 de mayor detección. Conclusiones. El comportamiento de la enfermedad del Zika influye en las mujeres atendidas en el distrito 09D01, presentándose a cualquier edad y especialmente en las mujeres embarazadas en el segundo trimestre de gestación; con características sintomáticas variables. Por lo tanto, es significativo anotar que se debe continuar observando el debutar clínico en el futuro próximo, pero sigue siendo importante y de confianza el diagnóstico molecular para su confirmación.

Introduction. The Zika virus is a flavivirus that is transmitted by mosquitoes mainly of the genus Aedes. Approximately 80 % of infected people have no symptoms. Objective. Epidemiological characterization of Zika's disease in women treated in the district 09D01 during January to June of 2017. Methodology. It was of non-experimental, descriptive, cross-sectional analytic design in women attended at the outpatient clinic. Results. It was obtained the group most affected of 30 - 39 years with 30,41 %, 20 - 29 years with 29,90 %, among others; but in the pregnant women, it was the 20- to 29-year-old age group with 47,42 % followed by 30-39 years of age with 40,21 %, among others. In the case of non-pregnant women, it was observed in the ages of 0 to 9 years, 10 to 19 years and in more than 50 years. As for the pregnant women, they were mostly in the second quarter with 48,44 %. Laboratory results were positive in 98,97 % of the cases with PCR and epidemiological link 1,03 %; being the hospital Maternal Infantile and Health Center N.° 3 of greater detection. Conclusions. The behavior of Zika'sdisease influences the women treated in the district 09D01, presenting at any age and especially in pregnant women in the second trimester of gestation; with variable symptomatic characteristics. Therefore, it is significant to note that clinical debut should continue to be observed in the near future, but the molecular diagnosis for confirmation remains important and reliable.