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Background/Objectives : 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. Methods : Observational and prospective cohort study. Adult CHD patients and controls were matched for age and sex. Results : In total, 434 CHD patients (cases) and 820 controls were studied. The median age in the CHD patients was 30 (18-62) years and 256 (59%) were male. A total of 51%, 30%, and 19% of patients with CHD had mild, moderate, and great complexity defects, respectively. The body mass index [1.07 (1.01-1.13), p = 0.022)], diabetes mellitus [3.57 (1.07-11.97), p = 0.039], high NT-pro-BNP levels [1.00 (1.00-1.01), p = 0.021], and low serum iron concentrations [0.98 (0.97-0.99), p = 0.001] predicted high hs-CRP levels (≥0.3 mg/dL) in patients with CHD. During a follow-up time of 6.81 (1.17-10.46) years, major cardiovascular events (MACE) occurred in 40 CHD patients, showing the Kaplan-Meier test demonstrated a worse outcome among patients with hs-CRP levels above 0.3 mg/dL ( p = 0.012). Also, hs-CRP showed statistical significance in the univariate Cox regression survival analysis. However, after adjusting for other variables, this significance was lost and the remaining predictors of MACE were age [HR 1.03 (1.01-1.06), p = 0.001], great complexity defects [HR 2.46 (1.07-5.69), p = 0.035], and an NT pro-BNP cutoff value for heart failure > 125 pg/mL [HR 7.73 (2.54-23.5), p < 0.001]. Conclusions : Hs-CRP obtained statistical significance in the univariate survival analysis. However, this significance was lost in the multivariate analysis in favor of age, CHD complexity, and heart failure.

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., Results: 214 patients with CHD [median age 21 (17-35) years and 41 (19%) males] and 345 controls were studied and followed up. None of them had symptoms of renal calculi. Nine (4%) patients with CHD and 24 (7%) patients in the control group showed crystalluria (p = 0.180), all of them composed of calcium oxalate. No significant differences were seen in age, sex, body mass index, CHD complexity, cardiovascular risk factors, NYHA functional class, cyanosis, and medical treatment between CHD patients with and without crystalluria. In relation to survival, 18 patients with CHD had a major acute cardiovascular event (MACE) (3 strokes, 2 myocardial infarction, 9 cardiovascular death and 4 non cardiovascular mortality) during the follow up time [7.3 (4.4-8.5) years] without significant differences in the Kaplan-Meier analysis (p = 0.358) between patients with and without crystalluria., Conclusion: No significant differences were found between CHD and control patients in relation to crystalluria and it had no impact on the occurrence of cardiovascular events in the medium term follow up of patients with CHD., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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. The inclusion criterion were being over 16years old and having performed at least one TMT in the prepandemic and postpandemic period., Results: One thousand six hundred fifty-five patients were included in the study. Nine hundred thirty-five (56.5%) were men and 720 (43.5%) women. The mean age was 57.3±14.9 and the mean follow-up time was 15.4 months. Fifty-three percent patients had arterial hypertension, 20% dyslipidemia, 12% diabetes mellitus, 8% smoking habit, 19% personal history of ischemic heart disease, 5% COPD, 8% bronchial asthma, and 8% atrial fibrillation. In almost all the variables studied in PE, including the appearance of ventricular arrhythmias, no significant differences were found, neither by age nor sex, except for the existence of a very slight decrease in exercise capacity with mask use in older patients (>65years)., Conclusions: The use of surgical or FFP2 face masks during the TMT did not affect functional capacity, blood pressure, heart rate, or increased ventricular arrhythmias., (Copyright © 2022 Elsevier España, S.L.U. All rights reserved.)

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. Only 75 patients (15%) suffered a relapse, which occurred during the first 6 months of therapy, with a peak at 4.5 months. An initial univariate analysis identified that patients in the relapse group were significantly older (67.4 ± 11.0 vs 61.6 ± 12.3 years old) and presented with diffuse coronary disease, insulin-dependent type 2 diabetes mellitus dyslipidemia, and arterial hypertension. A poor clinical response to the platelet antiaggregation regime also occurred more frequently among patients taking acenocoumarol and calcium channel blockers, along with aspirin and clopidogrel, while no association was found according to CYP2C19 genotypes. A retrospective multivariate analysis indicated that patients belonging to the nonresponder phenotype to treatment with aspirin and clopidogrel were older, presented with diffuse coronary disease, a group largely overlapping with type 2 insulin-dependent diabetes mellitus, and were taking dihidropyrimidinic calcium channel blockers., (© 2021, The American College of Clinical Pharmacology.)

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. While dyslipidemia and arterial hypertension were more prevalent among patients with angina pectoris, current smoking status and elevated inflammatory markers, including total leukocyte and monocyte counts, were more likely associated with acute coronary events. Propensity score matching analysis, performed to eliminate the influence of these risk factors and highlight genetic modifiers, revealed that a single nucleotide variant, rs17735770 at the 3'untranslated region of the CCL7 gene transcript, was associated with decreased cardiovascular risk in a group represented mostly by men, with an average age of 57, and without significant differences in traditional risk factors. Furthermore, the presence of this variant altered the local mRNA structure encompassing a binding site for miR-23ab, resulting in increased translation of a reporter gene in a miR23 independent fashion. The rs17735770 genetic variant led to increased expression of CCL7, a potential antagonist of CCR2 at inflammatory sites, where it could play a meaningful role during the evolution of atherosclerosis., Competing Interests: AD-C was employed by SECUGEN SL, Madrid, Spain and designed all the primers and performed all the sequencing associated with this project at SECUGEN SL, a service paid for by the authors. However, SECUGEN SL was not involved in the study design, collection, analysis, interpretation of data, the writing of this article or the decision to submit it for publication. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Medina-Gil, Pérez-García, Saavedra-Santana, Díaz-Carrasco, Martínez-Quintana, Rodríguez-González, Ramírez, Riaño, Garay-Sánchez and Tugores.)

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., Results: A total of 589 CHD patients (58% males, 29 ± 14 years old) and 2745 matched control patients were followed up. A total of 69 (12%) CHD patients had a major acute cardiovascular event (MACE) during the follow-up time (6.1 [0.7-10.4] years). Patients with CHD and a GGT >60 U/L were significantly older, more hypertensive and dyslipidemic, had a worse NYHA functional class and a greater anatomical complexity than CHD patients with a GGT ≤60 U/L. The binary logistic regression analysis showed that age, a great CHD anatomical complexity, and having atrial fibrillation/flutter were the predictive factors of higher GGT levels (>60 U/L). The Kaplan-Meier analysis showed that patients with CHD and a GGT concentration above 60 UL showed the lowest probability of survival compared to that of CHD with GGT ≤60 U/L and controls irrespective of their GGT concentrations (p < .001). Similarly, the multivariable Cox regression analysis found an independent association between higher GGT levels (>60 U/L) and a worse prognosis (HR 2.44 [1.34-4.44], p = .003) among patients with CHD., Conclusion: Patients with CHD showed significant higher GGT levels than patients in the control group having those with higher GGT concentrations (>60 U/L) the worst survival., (© 2021 Stichting European Society for Clinical Investigation Journal Foundation. Published by John Wiley & Sons Ltd.)

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). 74 patients (14 patients with a Fontan procedure and 60 patients with a restrictive VSD) were included in the study. Median age was 18 (14-45) years old and 41 patients were males. Fontan patients had significantly lower platelet count, lower mean platelet volume (MPV) and lower glucose levels than patients with single non-operated restrictive VSD. On the contrary, Fontan patients showed higher liver enzymes [aspartate aminotransferase (AST) and alanine aminotransferase (ALT), gamma-glutamyl transferase (GGT)], N-terminal pro-brain natriuretic peptide (NT-pro-BNP) and thyroid-stimulating hormone (TSH) concentrations than patients with restrictive VSD. 4 out of 14 (29%) patients with Fontan operation and significant liver ultrasound findings showed greater number of cardiac surgeries, lower MPV values and higher GGT and TSH levels than Fontan patients with mild findings. In conclusion, Fontan patients showed higher liver enzymes (AST, ALT and GGT) than controls and Fontan patients with significant liver ultrasound findings had higher GGT and TSH concentrations than Fontan patients with mild findings., Competing Interests: None., (AJCD Copyright © 2021.)

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. Patients were sent an online questionnaire to determine clinical, demographic and the 12-element general health questionnaire (GHQ-12) data during the COVID-19 quarantine. 242 out of 407 (59%) patients with CHD, to whom the questionnaire was sent, responded to the survey. 98 (42%) patients were between 14 and 24 years old and 133 (58%) were over 25 years old. Of the total, 119 (51%) were male. 123 (51%), 88 (36%) and 31 (13%) patients with CHD had mild, moderate, and severe anatomical complexity respectively. 11 (4.5%) out of 242 patients with CC presented heart failure (HF) symptoms, requiring 18% of them admission to the hospital emergency department during the pandemic (P=0.002). In relation to the GHQ-12 questionnaire, patients with CHD and HF enjoyed less their daily activities (81% vs. 51%, P=0.043) and had less self-confidence (46% vs. 18%, P=0.041) than those without HF symptoms. In conclusion, patients with CHD and HF, during the COVID-19 quarantine, presented a lower capacity to enjoy daily activities and self-confidence than CHD without HF symptoms., Competing Interests: None., (AJCD Copyright © 2021.)

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). To be included in the study, at least one of the grandparents (maternal or paternal) should be born in Gran Canaria. 258 out of 353 CHD patients met the inclusion criteria. 58% of CHD patients were male and the median age was of 28 (21-40) years old. The most frequent types of CHD were cardiac septal defects (76 patients), right side cardiac outflow tract anomalies (74 patients) and left side cardiac outflow tract anomalies (58 patients). 13% of the patients had a family history of CHD, 11% showed consanguinity and 7% had an associated polymalformative syndrome. 20% of the four ancestors were born in the same municipality and a significant association was seen between two areas of Gran Canaria, orographically related, and right-side cardiac outflow tract anomalies (P<0.001). In conclusion in patients with tetralogy of Fallot and/or pulmonary valve stenosis/atresia an ancestry's geographic aggregation was seen., Competing Interests: This work is supported by a competitive, peer reviewed grant from the Distribuidora Industrial, S.A. (DISA) (2018) and the Fundación Canaria Instituto de Investigación Sanitaria de Canarias (FIISC) of the Canary Islands Health Service (PIFUN 40/18)., (AJCD Copyright © 2021.)

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). Prospective study of consecutive outpatient non-hypoxemic CHD patients followed up in a single tertiary referral hospital. Demographic, clinical, blood test and 24-hour urine collection were recorded. 264 CHD patients, 22 (18-343) years old and 160 (61%) males, were followed up during 9.2 (5.9-11.1) years. 137 (52%), 96 (36%) and 31 (18%) CHD patients had mild, moderate, and great anatomical CHD defects. 44 (17%) and 32 (12%) CHD patients showed proteinuria (≥ 150 mg/24 hours) and albuminuria (> 30 mg/24 hours) respectively. 35 out of 44 (79%) CHD patients with proteinuria (≥ 150 mg/24 hours) showed normal to mild albuminuria levels (< 30 mg/24 hours). Variables associated with proteinuria were male sex, body mass index, auricular fibrillation/flutter, arterial hypertension, diabetes mellitus and being under angiotensin-converting enzyme (ACE) inhibitor and an angiotensin receptor blocker (ARB), loop diuretics or anti-aldosterone treatment. Major adverse cardiovascular events (MACE), defined as cardiovascular and non-cardiovascular deaths, stroke, myocardial infarction and heart failure requiring hospitalization, occurred in 16 (6%) patients during the follow up time. Multivariate Cox regression analysis showed that older patients, patients with a great CHD complexity and patients with proteinuria [6.99 (1.90-24.74), P=0.003] had a significant higher risk of MACE. Proteinuria is frequent among non-hypoxemic CHD patients and occurs mostly in those with a GFR above 60 ml/min/1.73 m 2 and normal to mild albuminuria levels. Having proteinuria, but not albuminuria, was independently associated with a worse outcome., Competing Interests: None., (AJCD Copyright © 2021.)

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. 154 patients with CHD recruited from a single hospital outpatient clinic and 250 healthy controls were studied between October 2018 and April 2019. Median age in patients with CHD was 27 (20-34) years and 62% were male. 32, 90 and 32 patients with CHD showed mild, moderate, and complex defects, respectively. 131 (53%) controls referred having felt or experienced negative feelings such as bad mood, despair, stress, or depression compared to 53 (34%) patients in the CHD group (P<0.001). Despite needing more medical treatment (P<0.001), patients with CHD enjoyed more their lives (P<0.001), felt their lives made more sense (P<0.001), were happier with themselves (P=0.006) and with their personal relationships (P=0.020), had a greater support from friends (P=0.031) and felt safer in their daily lives (P=0.004) than patients in the control group. Similarly, patient with CHD felt their environment was healthier, had more opportunities for leisure and were happier with their access to healthcare system (P<0.005) than controls. On the contrary, except the need for more medical treatment in patients with great CHD defects (P=0.019), no significant differences were seen in the WHOQoL-BREF survey according to the anatomical complexity. In conclusion, overall patients with CHD scored better in the WHOQoL-BREF questionnaire, especially in the psychological and environment domains, than patients in the control group., Competing Interests: None., (AJCD Copyright © 2021.)

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. The energy expenditure was below 600 Metabolic Equivalent of Task (MET)-minutes per week in 56 (28%) patients, while 144 (72%) were above 600 MET-minutes per week. Physically inactive patients with CHD were significantly more dyslipidemic than active ones, but no significant differences in serum glucose and cholesterol levels were observed. Logistic regression analysis showed that physical activity was associated with a better QoL rating [0.28 (0.10-0.17), P=0.014] and health satisfaction [0.24 (0.09-0.62), P=0.003]. Physically active patients with CHD scored 7.7 and 8.9 points higher, on a 100-point scale, in the physical and social relationships domains respectively, than physically inactive ones. No significant differences were seen in the psychological and the environment domains associated with physical activity. Additionally, a worse New York Heart Association (NYHA) functional class (≥ 2) was identified as a risk factor for dissatisfaction with health [OR 7.48, 95% CI (1.55-47.14), P=0.020], having a significantly negative impact of 8.5 and 7.6, on a 100-point scale, in the physical and psychological domains respectively. In conclusion, physically active patients with CHD had a better QoL assessment, were more satisfied with their health and scored higher in the physical and social relationships domains., Competing Interests: None., (AJCD Copyright © 2021.)

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. Patients were dichotomized to no or mild (PHQ-9 < 10) and moderate to severe (≥ 10) depressive symptoms. Thirty-four (14%) patients showed a PHQ-9 ≥ 10 and 10 of them (29%) were under anxiolytic or antidepressant treatment during the quarantine. During the study period, 9 (4%) patients had COVID-19 symptoms. Patients with a NYHA above 2 (P=0.025), living in houses without garden or balcony (P=0.014), needing psychological/psychiatric evaluation/medication in the previous 12 months or being under anxiolytic/antidepressant treatment during the confinement had, significantly, a PHQ-9 score ≥ 10 (P < 0.001). Being under anxiolytic/antidepressant treatment during the coronavirus pandemic [OR 3.92 (95% CI 1.05-14.66), P=0.043] and having previous psychological/psychiatric evaluation in the previous 12 months to the quarantine [OR 3.82 (95% CI 1.16-12.54), P=0.027] were the only variables that reached statistical significance, in the multivariable analysis, as predictors of a pathological PHQ-9 questionnaire (score ≥ 10). In conclusion depression was frequent during the COVID-19 quarantine among CHD patients, with only a third of them being under anxiolytic or antidepressant treatment. Needing psychological/psychiatric evaluation/treatment during the previous 12 months to the lockdown was a predictive factor for an abnormal PHQ-9 score., Competing Interests: None., (AJCD Copyright © 2021.)

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. The median age of the patients was 23 years (17-35) and 225 (57%) were males. Median plasma albumin concentration was 4.5 (4.2-4.7) g/dL, the body mass index was 23 (21-27) kg/m 2 , the NRI was 112 (106-118), and 33 (8%) patients showed malnutrition (NIR<100). A worse NYHA functional class (II and III), total cholesterol and serum glucose levels were significant risk factors associated with malnutrition (NRI<100) in CHD patients. During a median follow-up of 8 (5-10) years, 39 (10%) CHD patients suffered a MACE. Multivariable Cox regression analysis showed that older patients (years) [HR 1.06 (1.04-1.09), p < 0.001], CHD patients with great anatomical complexity [HR 4.24 (2.17-8.27), p < 0.001] and those with a lower NRI [HR 0.95 (0.93-0.98), p = 0.001] had a significant worse MACE-free survival, being the NRI a better predictor of MACE than albumin concentration., Conclusions: A low NRI is independently associated with a significant increased risk of MACE in CHD patients., Competing Interests: Declaration of competing interest No conflicts of interest., (Copyright © 2020 The Italian Diabetes Society, the Italian Society for the Study of Atherosclerosis, the Italian Society of Human Nutrition and the Department of Clinical Medicine and Surgery, Federico II University. Published by Elsevier B.V. All rights reserved.)

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. Risk model and its 5-year risk predictions were derived by logistic regression modelling, using separate development (18 centres: 144 cases and 1501 controls) and validation (two centres: 63 cases and 786 controls) datasets., Results: According to the combined SCD/SCA cumulative 20 years incidence, a lesion-specific stratification into four clusters-very-low (<1%), low (1%-4%), moderate (4%-12%) and high (>12%)-was built. Multivariable predictors were lesion-specific cluster, young age, male sex, unexplained syncope, ischaemic heart disease, non-life threatening ventricular arrhythmias, QRS duration and ventricular systolic dysfunction or hypertrophy. The model very accurately discriminated (C-index 0.91; 95% CI 0.88 to 0.94) and calibrated (p=0.3 for observed vs expected proportions) in the validation dataset. Compared with current guidelines approach, sensitivity increases 29% with less than 1% change in specificity., Conclusions: Predicting the risk of SCD/SCA in ACHD can be significantly improved using a baseline lesion-specific stratification and simple clinical variables., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

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. 200 CHD patients, median age 28 (16-54) years old and 120 (60%) males were studied. 45 (22.5%), 122 (61%) and 33 (16.5%) CHD patients had simple, moderate, and great complexity defects respectively. PREDIMED score was classified as low (score 0-5), intermediate (6-9) or high (> 9). 146 (83%) CHD patients showed a suboptimal Mediterranean diet adherence (PREDIMED score < 9) with less than half of patients eating enough vegetables, fruits, legumes, fish or nuts but with a high intake of butter/margarine, commercial sweets and carbonated beverages. No significant differences were seen between sex, body mass index, cardiovascular risk factors, CHD complexity or the educational level and the PREDIMED scores. Only being married was associated with a significant lower Mediterranean diet adherence (P=0.019). Meanwhile, no statistical significance was observed between serum glucose, creatinine, uric acid, albumin, LDL cholesterol, HDL cholesterol or triglycerides levels according to the PREDIMED classification (low, intermediate or high adherence). Conclusions: CHD patients have a low adherence to the Mediterranean diet with a low intake of vegetables, fruits, legumes, and fish., Competing Interests: None., (AJCD Copyright © 2020.)

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. Exclusion criteria were renal failure, anaemia, receiving iron therapy and cyanosis. Blood tests included glucose, creatinine, iron, apoferritin, liver enzymes and a complete blood count. C-reactive protein and N-terminal prohormone of B-type natriuretic peptide (NT-pro-BNP) concentrations were also measured in patients with CHD. Major adverse cardiac events (MACE) were defined as cardiovascular/total mortality, arterial thrombotic events, arrhythmias, major bleedings, pulmonary embolism or heart failure needing hospital admission., Results: The median age in patients with CHD was 23 (17-36) years and the median follow-up time was 5.8 (3.2-8.7) years; 103 (4.8%) controls and 40 (7.1%) patients with CHD had an RDW>15% (P=0.032). During follow-up, MACE were reported in 48 patients. CHD of great complexity, cardiovascular risk factors, low haemoglobin concentration and high NT-pro-BNP concentration were risk factors for an RDW>15%. Kaplan-Meier analysis showed a significantly worse cardiovascular outcome in patients with CHD with an RDW>15% (P<0.001). The multivariable survival analysis determined that age, CHD of great complexity, high NT-pro-BNP concentration and an RDW>15% were independent predictive factors for MACE., Conclusion: RDW and NT-pro-BNP concentration are independent analytical predictors of MACE in patients with CHD., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

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. A total of 25% and 9% of CHD patients showed anxiety and depression symptoms, respectively. Patients with an HADS score ≥ 8 had a significantly worse New York Heart Association (NYHA) functional class, needed more psychological support, had more mental health history, and took more anxiolytic/antidepressant medication than the CHD patients with an HADS score below 8. A worse NYHA functional class [OR, 1.88 (1.01-3.52)] proved to be a predictor of a borderline/abnormal HADS score., Conclusion: Psychological distress has a high prevalence among CHD patients and having an NYHA Class II and III is a significant predictor of an HADS score ≥ 8., (© 2020 Wiley Periodicals, Inc.)

Introduction: As congenital patients get older, it can be hypothesised that cardiovascular risk factors increase., Methods: Retrospective study of congenital heart disease (CHD) patients attended between January 2008 and September 2018. Cardiovascular risk factors, myocardial infarction, stroke, peripheral vascular disease, and analytical data such as serum glucose and lipid profile were determined., Results: Eight hundred and eighteen CHD patients and 1955 control patients matched for age and sex were studied. CHD patients were distributed in simple (462 patients), moderate (228 patients) and great (128 patients) complexity. Median age in CHD patients was 33 (25-41) years old and 56% were male. CHD patients were significantly more hypertensive and diabetic but less dyslipidemic and smokers than patients in the control group. Twenty-seven (3.3%) CHD patients had an arterial thrombotic event: 3 coronary, 22 neurological and 2 peripheral vascular disease. No significant differences were seen in the incidence of myocardial infarction between the control and the CHD groups. However CHD patients had a significant higher incidence of arterial thrombotic events (coronary, neurological and peripheral vascular events) at the expense of strokes and transient ischaemic attacks (22 vs 2 events in CHD and control patients, respectively). Also, no significant differences were seen in age, sex, BMI, arterial hypertension, diabetes mellitus, dyslipidemia, smoking habit, serum glucose, total and LDL cholesterol, statin treatment, myocardial infarction and arterial thrombotic events according to CHD complexity. Being older and having arterial hypertension, diabetes mellitus, dyslipidemia and smoking habit were more frequent among CHD patients with arterial thrombosis., Conclusions: Congenital heart diseases are more hypertensive and diabetic but less dyslipemic and smokers than patients in the control group. CHD patients have a higher incidence of neurological events but not of myocardial infarction in relation to the control population., (© 2019 John Wiley & Sons Ltd.)

Aneurysms of the pulmonary arteries and trunk are rare entities. The Waterston shunt is a palliative procedure for children with cyanotic CHD due to obstruction of the pulmonary outflow. Described complications are distortion of the pulmonary artery and pulmonary arterial hypertension. We report a patient with a giant right pulmonary artery aneurysm in relation to a Waterston shunt.

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., Conclusions: Recognizing this atypical clinical presentation of aortic dissection and knowing how to approach it is critical for an early diagnosis and to minimize the risk of spinal cord ischemia during surgery., (Copyright: © 2019 Permanyer.)

Background: Platelet activation is linked with thrombosis, inflammation or heart failure., Objective: To establish clinical and analytical factors that may favor high mean platelet volume (MPV) and to determine if MPV levels favor major adverse cardiovascular events (MACE)., Methods: Stable CHD patients and a control population matched for age, sex and cardiovascular factors., Results: 658 CHD patients and 2092 controls were studied. Median age in CHD patients was 33 (25-41) year old with 56% of them being male. No significant differences were seen between MPV values and cardiac complexity (p = 0.308) nor between MPV values in the CHD and control groups (p = 0.911). CHD patients had significant lower platelet count and MPV levels than patients in the control group. In the binary logistic regression analysis NT-pro-BNP levels above 125 pg/ml, thrombocytopenia and having atrial fibrillation/flutter reached statistical significance as predictors of MPV levels above 11 fL. The Kaplan-Meier survival analysis showed no significance between MPV levels higher than 11 fL and MACE, cardiovascular mortality and thrombotic events in a median time follow-up of 6.7(1.5-10.6) years., Conclusions: Atrial fibrillation/flutter, heart failure and thrombocytopenia are predictors of high MPV levels. A MPV above 11 fL is not associated with MACE at a median follow-up time.

Introduction: Despite anemia in acquired heart disease being a common problem, little is known in patients with congenital heart disease (CHD)., Methods: In total, 544 consecutive stable noncyanotic CHD patients were studied to determine demographic, clinical, and analytic parameters. Anemia was defined as a condition in which hemoglobin concentration was <13 g/dL in male individuals and <12 g/dL in female individuals., Results: In total, 49 (9%) CHD patients had anemia. Patients with complex anatomy had the highest prevalence of anemia (33%). The median hemoglobin concentration was 14.4 (13.5 to 15.6) mg/dL. Of the total anemic CHD patients, 21 of 49 (43%) were microcytic (mean corpuscular volume <84 fL) and 46 of 49 (94%) had a mean corpuscular volume under 95 fL. Oral anticoagulation, oral antiaggregation, diuretic treatment, and having valve prostheses or cardiovascular risk factors, such as arterial hypertension or diabetes mellitus, did not reach statistical significance between anemic and nonanemic CHD patients. Multivariate analyses determined as risk factors for anemia a worse New York Heart Association functional class (patients in class >II/IV) (odds ratio [OR], 8.37; 95% confidence interval [CI], 1.69-41.35), N-terminal proB-type natriuretic peptide levels >125 pg/mL (OR, 7.90; 95% CI, 2.88-21.69), and apoferritn levels below 15 ng/mL (OR, 0.21; 95% CI, 0.09-0.50). The Kaplan-Meier survival analysis showed no significant differences in mortality between anemic and nonanemic CHD patients (P=0.143)., Conclusions: The incidence of anemia in CHD patients is similar to that of the normal population and iron deficiency anemia accounts for most of the cases. There were no significant differences in mortality between CHD patients with and without anemia.

Introduction: Hyperuricemia has been associated with cardiovascular risk factors but it remains controversial if uric acid is an independent predictor of cardiac mortality., Methods: A total of 503 CHD patients (457 nonhypoxemic and 46 hypoxemic) and 772 control patients fulfilled inclusion criteria. Demographic, clinical, and analytical data [serum uric acid and 24h urine uric acid levels, N-terminal pro-B-type natriuretic peptide (NT-pro-BNP), and C-reactive-protein (CRP) concentrations] were studied. Survivals curves to determine cardiac death and arterial thrombosis in CHD patients were also examined., Results: Noncyanotic and cyanotic CHD patients had significant higher serum uric acid concentration (5.2 ± 1.5 vs 4.9 ± 1.3mg/dL, P = .007 and 6.7 ± 2.1 vs 4.9 ± 1.3mg/dL, P < .001, respectively) and gout (1% vs 0%, P = .003 and 4% vs 0%, P < .01, respectively) than the control population. Among CHD patients, hyperuricemic patients were significant older and with overweight, used more diuretics, were more cyanotic and had higher serum creatinine, NT-pro-BNP and CRP concentrations than nonhyperuricemic. In the multivariable analysis, the body mass index (BMI) (OR 1.09; 95% CI 1.01-1.18), cyanosis (OR 6.2; 95 CI 1.5-24.6), serum creatinine concentration (OR 49; 95% CI 44-538), and being under diuretic treatment (OR 4.5; 95% CI 1.4-14.5) proved to be risk factors for hyperuricemia in CHD patients. The Kaplan-Meier events free survival curves, during a 5.2 ± 2.7 years follow-up of up time, showed that hyperuricemic CHD patients had significant higher cardiovascular death (P = .002). However, after applying the Cox regression analysis uric acid levels lost its statistical significance. No significant differences were seen in relation to thrombotic events between CHD patients with and without hyperuricemia., Conclusions: CHD patients, noncyanotic and cyanotic, have higher serum uric acid levels and gout than patients in the general population. BMI, renal insufficiency, cyanosis, and the use of diuretics were risk factor for hyperuricemia among CHD patients., (© 2018 Wiley Periodicals, Inc.)

Introduction: CYP2C19 is a major isoform of cytochrome P450 that metabolizes a number of drugs and is involved in the glucocorticoids synthesis. CYP2C19 polymorphisms have been associated with the genetic risk for type 2 diabetes., Methods: Five hundred and three patients with an acute coronary event were studied to assess the association between the CYP2C19 activity (CYP2C19*2, CYP2C19*3 and CYP2C19*17 variants) and the type of acute coronary syndrome, cardiovascular risk factors (arterial systemic hypertension, diabetes mellitus, dyslipidemia and smoking), analytical parameters and the extent and severity of coronary atherosclerosis., Results: Genotype distribution in our series was similar to that expected in the Caucasian population. Among the traditional cardiovascular risk factors, very poor metabolizer patients (*2/*2, *3/*3 or *2/*3) had a greater tendency to present diabetes mellitus needing insuline (P=.067). Conversely, when we compared very poor, poor and normal metabolizers vs. rapid and ultrarapid metabolizers we found significant differences in those diabetic patients under insulin treatment (64 patients [18%] vs. 17 patients [11%]; P=.032). On the contrary, analytical parameters, systemic arterial hypertension, dyslipidemia, smoking or the personal/family history of coronary artery disease did not reach statistical significance regardless of CYP2C19 activity. Similarly, the number and the type of coronary disease (thrombotic, fibrotic or both) did not differ between patients with different CYP2C19 enzyme activity., Conclusion: Patients with an acute coronary event and a very poor, poor and normal CYP2C19 metabolizer genotype have a higher prevalence of diabetes mellitus needing insuline than patients with the rapid and ultrarapid metabolizers CPY2C19 genotype., (Copyright © 2017 Elsevier España, S.L.U. All rights reserved.)

Introduction: Dextro-transposition of the great arteries (d-TGA) patients is at high risk of developing right ventricular dysfunction and tricuspid regurgitation in adulthood. Determining the relation between echocardiographic parameters, N-terminal pro-brain natriuretic peptide (NT-pro-BNP) levels and the New York Heart Association (NYHA) functional class may help determining the best time to operate them., Methods: Patients with simple d-TGA operated in infancy with an atrial switch procedure (Mustard or Senning operation) were followed up in our Adult Congenital Heart Disease Unit. Analytical, echocardiographic, and clinical parameters were determined to evaluate the correlation between right echocardiographic ventricular function, NT-pro-BNP levels, and NYHA functional class., Results: Twenty-four patients with d-TGA were operated in infancy of whom 17 alive patients had simple d-TGA. Nine patients had NT-pro-BNP levels lower than 200 pg/mL and eight patients were above 200 pg/mL. Patients with lower hemoglobin concentration, higher right ventricular diameter or under diuretic treatment showed significant higher NT-pro-BNP levels (above 200 pg/dL). The Spearman test showed a positive correlation between basal right ventricular diameter and tricuspid regurgitation with pro NT BNP levels (correlation coefficient of .624; P=.017 and .490; P=.046, respectively) and a negative correlation with the right ventricle fractional area change (-.508, P=.045). No correlation was seen between NT-pro-BNP levels and the rest of echocardiographic parameters or the NYHA functional class., Conclusion: NT-pro-BNP levels showed a positive correlation with basal right ventricular diameter and tricuspid regurgitation but not with NYHA association functional class in d-TGA patients., (© 2017, Wiley Periodicals, Inc.)

Marfan syndrome is an autosomal dominant disorder of the connective tissue caused by mutations in the fibrillin-1 ( FBN1 ) gene. Mutations affecting cysteine residues within the epidermal growith factor-like calcium-binding domains (EGF&#95;CA) of FBN1 are associated with Marfan syndrome features and, especially, with ectopia lentis. We report a novel substitution, affecting the first cysteine of an EGF&#95;CA-binding module encoded by exon 63 of FBN1 (C2571Y), in a patient presenting with typical Marfan syndrome features but without ectopia lentis. The involvement of this particular carboxi-terminal domain in bone morphogenetic protein signaling is evidenced by patterning defects in the apendicular skeleton shown by the gain of a phalange at digit 1 and the fusion of some wrist bones. Although the mutation appeared as sporadic, detailed analysis revealed that the asymptomatic father was a gonosomal mosaic, and that aproximately 25% of his body cells carry the mutation. Based on this and previous evidence on the origin of sporadic mutations, we would like to stress the importance of detailed parental genetic screening, so the risk of recurrence may be evaluated.

Patients after a Fontan operation exhibit some degree of fibrosis which are primarily located in a sinusoidal or centrilobular pattern. Cirrhosis may develop years later and the incidence of cancer is up to 5%. That is why some authors recommend that patients who are >10 years out from their Fontan operation should undergo cardiac assessment as well as the determination of alpha-fetoprotein level, the realization of liver imaging tests or even liver biopsy to stay ahead of neoplastic transformation.

Introduction: Paraoxonase 1 (PON1) plays a major role in the oxidation of low density lipoprotein and in the prevention of coronary atherogenesis. In this context, coding region polymorphisms of PON1 gene, responsible for the enzyme activity, has become of interest as a marker for atherogenesis., Methods: A study and follow-up was conducted on 529 patients with an acute coronary event in order to assess the association between the PON1 Q192R (rs662;A/G) polymorphism, the type of acute coronary syndrome, cardiovascular risk factors (arterial hypertension, diabetes mellitus, dyslipidaemia, and smoking), the extent and severity of coronary atherosclerosis, and the medium-term clinical follow-up., Results: The QQ genotype was found in 245 (46.3%) patients, with 218 (41.2%) patients showing the QR genotype, and 66 (14.5%) patients had the RR genotype. No significant differences were found between the QQ and QR/RR genotypes as regards the clinical characteristics, the analytical data, and the angiographic variables. Similarly, Kaplan-Meier survival analysis showed no significant differences in presenting with a new acute coronary event (p=0.598), cardiac mortality (p=0.701), stent thrombosis (p=0.508), or stent re-stenosis (p=0.598) between QQ and QR/RR genotypes during the follow-up period (3.3±2.2 years)., Conclusions: In patients with an acute coronary syndrome, the PON1 Q192R genotypes did not influence the risk of suffering a new acute coronary event during the medium-term follow-up., (Copyright © 2016 Sociedad Española de Arteriosclerosis. Publicado por Elsevier España, S.L.U. All rights reserved.)

Hyperuricemia is defined as serum uric acid level of more than 7 mg/dL and blood levels of uric acid are causally associated with gout, as implicated by evidence from randomized clinical trials using urate lowering therapies. Uric acid as a cardiovascular risk factor often accompanies metabolic syndrome, hypertension, diabetes, dyslipidemia, chronic renal disease, and obesity. Despite the association of hyperuricemia with cardiovascular risk factors, it has remained controversial as to whether uric acid is an independent predictor of cardiovascular disease. To settle this issue, and in the absence of large randomized controlled trials, Mendelian randomization analysis in which the exposure is defined based on the presence or absence of a specific allele that influences a risk factor of interest have tried to shed light on this., Competing Interests: The authors have no conflicts of interest to declare.

Background: Peripheral arterial disease and coronary artery disease are frequently associated. The percutaneous approach may sometimes involve additional difficulties to the coronary artery disease., Clinical Case: The case is presented on an 82 year-old male patient with multiple cardiovascular risk factors, a Leriche syndrome and axillobifemoral bypass, who was admitted to hospital due to an inferior myocardial infarction. The procedure approach (radial, brachial, or femoral access routes for percutaneous coronary treatment) and associated complications from the procedure are discussed., Conclusion: Although technical improvements and/or treatment of peripheral vascular lesions may allow percutaneous coronary intervention, individual risk and benefit in each patient must be assessed., (Copyright © 2015 Academia Mexicana de Cirugía A.C. Publicado por Masson Doyma México S.A. All rights reserved.)

Unlabelled: Introduction Hypoxaemic congenital heart disease (CHD) patients are at higher risk of complications. The aim of this study was to compare and follow-up blood and 24-hour urine analytical data in hypoxaemic and non-hypoxaemic CHD patients., Methods: The inclusion criteria for this study were as follows: patients older than 14 years of age with a structural CHD with or without associated hypoxaemia., Results: In total, 27 hypoxaemic and 48 non-hypoxaemic CHD patients were included in order to compare blood and 24-hour urine analytical data. Among hypoxaemic patients, 13 (48.1%) were male, two (7.4%) had diabetes mellitus, one of whom was a smoker, one (3.7%) had systemic arterial hypertension, and 11 (40.7%) showed pulmonary arterial hypertension. The mean follow-up time was 3.1±1.9 years. Hypoxaemic CHD patients showed higher proteinuria concentrations (g/24 hours) (0.09 (0.07; 0.46) versus 0.08 (0.07; 0.1), p=0.054) and 24-hour albumin excretion rate (µg/min) (16.5 (11.2; 143.5) versus 4.4 (0.0; 7.6), p<0.001) compared with non-hypoxaemic CHD patients; however, no significant differences were found in the proteinuria levels and in the 24-hour albumin excretion rate in CHD patients with associated hypoxaemia, both at baseline and at follow-up. When divided into groups, hypoxaemic patients with palliative shunts showed significantly higher proteinuria concentrations compared with hypoxaemic patients not operated on or with Fontan procedures (p=0.01). No significant differences were seen in 24-hour proteinuria and 24-hour albumin excretion rate during the follow-up of patients with palliative shunts., Conclusions: Hypoxaemic CHD patients have significant higher 24-hour proteinuria concentration and 24-hour albumin excretion rate compared with non-hypoxaemic CHD patients. Among hypoxaemic CHD patients, those with palliative shunts showed the highest 24-hour proteinuria concentrations.

Coronary pseudoaneurysms are rare and usually occur after percutaneous procedures, spontaneous dissections, aortocoronary bypass, or chest trauma. Although most of the cases are casual, other patients present as acute myocardial infarction, fistula formation or cardiac tamponade. We report the case of a coronary pseudoaneurysm after percutaneous treatment of a coronary perforation., (Copyright © 2014 Sociedad Española de Arteriosclerosis. Published by Elsevier España. All rights reserved.)

Coarctation of the aorta, repaired in childhood, is a congenital heart disease frequently seen in adulthood. In these patients there is a higher incidence of hypertension during daily activities and exercise despite a correct surgical repair. Since hypertension may only be seen by ambulatory blood pressure monitoring (occult arterial hypertension) we should also look for indirect signs, such as the development of left ventricular hypertrophy.

Introduction: Subclinical hypothyroidism or mild thyroid failure is a common problem in patients without known thyroid disease., Methods: Demographic and analytical data were collected in 309, of which 181 were male and 128 were female, congenital heart disease (CHD) patients. CHD patients with thyroid-stimulating hormone above 5.5 mIU/L were also followed up from an analytical point of view to determine changes in serum glucose, cholesterol, N-terminal pro b-type natriuretic peptide, and C-reactive protein concentrations., Results: Of the CHD patients, 35 (11.3%) showed thyroid-stimulating hormone concentration above 5.5 mIU/L. Of them, 27 were followed up during 2.4±1.2 years - 10 were under thyroid hormone replacement treatment, and 17 were not. Of the 27 patients (25.9%), 7 with subclinical hypothyroidism had positive anti-thyroid peroxidase, and 3 of them (42.8%) with positive anti-thyroid peroxidase had Down syndrome. Down syndrome and hypoxaemic CHD patients showed higher thyroid-stimulating hormone concentrations than the rest of the congenital patients (p<0.001). No significant differences were observed in serum thyroxine, creatinine, uric acid, lipids, C-reactive protein, or N-terminal pro b-type natriuretic peptide concentrations before and after the follow-up in those CHD patients with thyroid-stimulating hormone above 5.5 mIU/L whether or not they received levothyroxine therapy., Conclusions: CHD patients with subclinical hypothyroidism showed no significant changes in serum thyroxine, cholesterol, C-reactive protein, or N-terminal pro b-type natriuretic peptide concentrations whether or not they were treated with thyroid hormone replacement therapy.