Filamin A C-terminal fragment modulates Orai1 expression by inhibition of protein degradation.

Filamin A (FLNA) is an actin-binding protein that has been reported to interact with STIM1 modulating the activation of Orai1 channels. Cleaving of FLNA by calpain leads to a C-terminal fragment that is involved in a variety of functional and pathological events, including pro-oncogenic activity in different types of cancer. Here we show that full-length FLNA is downregulated in samples from colon cancer patients as well as in the adenocarcinoma cell line HT-29.

Risk Factors for Chronic Kidney Disease in Adult Patients with Congenital Heart Disease and Its Relationship with Cardiovascular Mortality.

: Patients with congenital heart disease (CHD) show risk factors for chronic kidney disease (CKD) and it is well known that CKD has a large negative impact on survival. : Observational and prospective cohort study. Adult CHD patients and controls were matched for age and sex.

C-Reactive Protein and Long-Term Prognosis in Adult Patients with Congenital Heart Disease.

: Prognostic biomarkers may provide information about the patient's cardiovascular outcomes. However, there are doubts regarding how high-sensitivity C-reactive protein (hs-CRP) impacts patients with congenital heart disease (CHD). The main objective is to evaluate whether high hs-CRP levels predict a worse prognosis in patients with CHD.

Crystalluria in adolescent and adult patients with congenital heart disease.

Introduction: Crystalluria is a frequent finding in normal individuals and in patients suffering from urolithiasis. As nephrolithiasis has been associated with cardiovascular risk factors and most congenital heart disease (CHD) patients reach adulthood, the objective of this study is to determine the presence of crystalluria and if it influences their cardiovascular outcome.

Methods: Case-control and observational prospective study design of patients with CHD older than 14 years with a stable CHD verified with imaging tests and a control population.

Impact of face masks during the treadmill exercise tests.

Introduction And Objective: There is little literature on the use of face masks in a treadmill test (TMT) during the COVID-19 pandemic. The objective of this study is to analyze the impact of face masks during a TMT performed during the prepandemic (without face mask) and postpandemic (with face mask) era.

Methods: Retrospective observational unicentric study of patients undergoing TMT.

A Common Variant at the 3'untranslated Region of the CCL7 Gene (rs17735770) Is Associated With Decreased Susceptibility to Coronary Heart Disease.

Monocytes participate in the development of atherosclerosis through the action of cytokines and other inflammatory mediators. Among them, CCR2 and its ligands, CCL2 and CCL7 play an important role, so the main objective of this work was to determine whether genetic variants affecting their activity were associated with cardiovascular disease. A cohort of 519 patients that have suffered coronary events was analyzed under a propensity score-matching protocol selecting a homogeneous set of cases and controls, according to age, sex, smoking status, dyslipidemia, arterial hypertension and type 2 diabetes as risk factors.

Clinical and Pharmacological Parameters Determine Relapse During Clopidogrel Treatment of Acute Coronary Syndrome.

The therapeutic efficacy of clopidogrel as an antiplatelet drug varies among individuals, being the mainstream hypothesis that its bioavailability depends on the individual genetic background and/or interactions with other drugs. A total of 477 patients receiving double antiaggregation therapy with aspirin and clopidogrel, after suffering a first event, were followed for 1 year to record relapse, as a surrogate end point to measure their therapeutic response, as defined by presenting with an acute coronary event (unstable angina, ST-segment-elevation myocardial infarction, or non-ST-segment-elevation myocardial infarction), stent thrombosis/restenosis, or cardiac mortality. Anthropometric, clinical, and pharmacological variables along with CYP2C19 genotypes were analyzed for their association with the disease relapse phenotype.

Genealogy of patients with congenital heart disease in isolated populations.

In isolated populations rare genetic diseases are important and relatively frequent. The objective of this study is to determine the geographical aggregates of maternal and paternal ancestors of patients with congenital heart disease (CHD) to determine whether there is an association between the different areas and types of cardiac defects. Descriptive, observational, and cross-sectional study of patients with CHD obtained consecutively in a single adult CHD unit between January 2018 and December 2019 in Gran Canaria (Canary Islands, Spain).

Mental well-being among patients with congenital heart disease and heart failure during the COVID-19 pandemic.

Patients with congenital heart disease (CHD) show increasing survival. We evaluated the influence of COVID-19 confinement on the mental well-being of patients with CHD. Descriptive, cross-sectional, observational epidemiological study in a cohort of 242 patients with CHD over 14 years old recruited consecutively from a single adolescent and adult CHD outpatient unit.

Blood test assessment of liver ultrasound findings in patients with Fontan surgery.

Hepatic complications are common in patients with Fontan surgery. The objective of this observational study is to compare demographic, clinical and blood test data in patients older than 14 years old with a Fontan procedure (cases) and asymptomatic patients with single non-operated restrictive ventricular septal defect (VSD) (controls) and to determine whether there are differences in blood collection and liver disease scores according to the liver ultrasound findings in the group of Fontan patients. The liver findings were classified as mild (normal or heterogeneous echogenicity) and significant (nodular surface, small hyperechoic nodules or hepatocarcinoma).

Gamma-glutamyl transferase and cardiovascular events in patients with congenital heart disease.

Introduction: Serum gamma-glutamyl transferase activity (GGT) seems to predict cardiovascular events in different populations. However, no data exist on patients with congenital heart disease (CHD).

Methods: Observational, analytic, prospective cohort study design involving CHD patients and a control population to determine the effect of GGT levels on survival.

COVID-19 quarantine and depression in patients with congenital heart disease.

COVID-19 outbreak has brought tremendous psychological pressure to the general population, especially to those with associated cardiovascular disease. An online Patient Health Questionnaire (PHQ-9) survey on consecutive congenital heart disease (CHD) patients, was carried out to determine depression during the Spanish coronavirus disease (COVID-19) quarantine. Two-hundred forty-two out of 407 (59%) CHD patients answered the survey, 123 (51%) had mild defects, 88 (36%) moderate and 31 (13%) great defects, most of them between 18 and 24 years old and 51% were male.

Evaluation of quality of life in patients with congenital heart disease: an observational case control study.

Quality of life (QoL) has become an important issue in patients with congenital heart disease (CHD). Accordingly, the focus has shifted from mere survival to a better QoL. The objective of this study is to assess QoL, by examining the World Health Organization QoL-bref (WHOQoL-BREF) questionnaire, in patients with CHD and a control population of a same geographic area matched for age, sex, cardiovascular risk factors and educational level.

The effect of physical activity on quality of life and serum glucose and cholesterol levels in patients with congenital heart disease.

As physical activity contributes to quality of life and health, we evaluated its association, as measured by the Global physical activity (GPAQ) questionnaire, on the quality of life (QoL) and serum glucose and cholesterol levels of patients with congenital heart disease (CHD). This cross-sectional study was carried out in 200 adult patients with CHD (17 to 58 years old), of whom 45 had simple defects, 122 moderate defects and 33 great anatomical complexity defects. Physiological complexity was defined as stage A in 74 patients, stage B in 29, stage C in 86 and stage D in 11.

Proteinuria versus albuminuria in 24-hour urine collection: prevalence and clinical outcome in non-hypoxemic adult patients with congenital heart disease.

Congenital heart disease (CHD) patients, especially cyanotic ones, usually have renal function impairment. However, little information exists in non-cyanotic CHD patients. The objective of this study is to determine renal failure in non-hypoxemic CHD patients by measuring the amount of protein and albumin released in urine over a 24-hour period and determining the glomerular filtration rate (GFR).

COVID-19 in Adults With Congenital Heart Disease.

Background: Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications.

Objectives: This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes.

Methods: Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide.

Mediterranean diet adherence in patients with congenital heart disease.

The Mediterranean diet, based on a rural life where people ate what they grew, has shown cardiovascular benefits. Cross-sectional study of congenital heart disease (CHD) patients recruited consecutively from a single hospital outpatient clinic with the aim of determining their adherence to the Mediterranean diet according to the PREDIMED questionnaire. CHD complexity was categorized as simple, moderate, or great and demographic, clinical and blood test data were recorded.

Malnutrition is independently associated with an increased risk of major cardiovascular events in adult patients with congenital heart disease.

Background And Aims: Malnutrition is found frequently during chronic diseases, and its prevalence and relation to disease outcome in adult patients with congenital heart disease (CHD) remains unknown.

Methods And Results: A cohort of 393 consecutive stable congenital heart disease (CHD) patients was followed up in a single dedicated clinical unit. Demographic, clinical and laboratory parameters, along with a nutritional risk index (NRI), were studied, as well as major acute cardiovascular events (MACE), defined as arterial thrombotic events, heart failure requiring hospitalization or cardiovascular and non-cardiovascular mortality.

Red blood cell distribution width in addition to N-terminal prohormone of B-type natriuretic peptide concentration improves assessment of risk of cardiovascular events in adult patients with congenital heart disease.

Background: Red blood cell distribution width (RDW) is a predictor of adverse outcomes in patients with heart disease.

Aim: To establish predictors of high RDW values in patients with congenital heart disease (CHD), and their relationship with cardiovascular events.

Methods: Overall, 561 patients with stable CHD who attended a single outpatient clinic and a matched control population of 2128 patients were studied.

Predicting sudden cardiac death in adults with congenital heart disease.

Objectives: To develop, calibrate, test and validate a logistic regression model for accurate risk prediction of sudden cardiac death (SCD) and non-fatal sudden cardiac arrest (SCA) in adults with congenital heart disease (ACHD), based on baseline lesion-specific risk stratification and individual's characteristics, to guide primary prevention strategies.

Methods: We combined data from a single-centre cohort of 3311 consecutive ACHD patients (50% male) at 25-year follow-up with 71 events (53 SCD and 18 non-fatal SCA) and a multicentre case-control group with 207 cases (110 SCD and 97 non-fatal SCA) and 2287 consecutive controls (50% males). Cumulative incidences of events up to 20 years for specific lesions were determined in the prospective cohort.

Prevalence and predictors of psychological distress in congenital heart disease patients.

Objective: To determine psychological distress in congenital heart disease (CHD) patients.

Methods: Cross-sectional study among consecutive CHD patients recruited from a single hospital outpatient clinic to determine anxiety and depression according to the Hospital Anxiety and Depression Scale (HADS) questionnaire.

Results: One hundred and sixty-nine CHD patients [29 (19-39) years old, 100 (59%) males] were studied.

Anterior spinal artery syndrome and aortic dissection.

Background: Anterior spinal artery syndrome, usually resulting in flaccid paraplegia, is a rare but disastrous complication that can occur after surgery of aortic aneurysms and aortic dissections. Spinal cord infarct as the initial clinical presentation of aortic dissection is a very rare finding.

Case Report: A 42-year-old male patient who comes to the emergency department due to severe chest pain associated with presyncope and paraplegia of the lower limbs in the context of type A aortic dissection.