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. 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.

Impacto del uso de la mascarilla durante la realización de la prueba de esfuerzo en tapiz rodante / Impact of face masks during the treadmill exercise tests

Introducción y objetivos: Existe poca literatura sobre el uso de la mascarilla en la realización de una prueba de esfuerzo (PE) durante la pandemia de COVID-19. El objetivo de este estudio es analizar el impacto del uso de la mascarilla durante la realización de una PE en un grupo de pacientes que han realizado al menos una PE con y sin mascarilla.MétodosEstudio observacional retrospectivo unicéntrico de pacientes sometidos a una PE con tapiz rodante. El criterio de inclusión fue tener más de 16años y haber realizado al menos una PE en época prepandemia (sin mascarilla) y pospandemia (con mascarilla).ResultadosUn total de 1.655 pacientes fueron incluidos en el estudio; 935 (56,5%) eran varones y 720 (43,5%) eran mujeres. La edad media fue de 57,3±14,9 años y el tiempo medio de seguimiento fue de 15,4 meses. El 53% de los pacientes presentaron antecedentes personales de hipertensión arterial; el 20%, dislipemia; el 12%, diabetes mellitus; el 8%, hábito tabáquico; el 19%, cardiopatía isquémica; el 5%, EPOC; el 8%, asma bronquial, y el 8%, fibrilación auricular. En la casi totalidad de las variables estudiadas en la PE, incluida la aparición de arritmias ventriculares, no se evidenciaron diferencias significativas, ni por edad ni por sexo, salvo la existencia de una muy leve disminución en la capacidad de ejercicio con el uso de mascarilla en los pacientes de mayor edad (>65años).ConclusionesEl uso de mascarillas quirúrgicas o FFP2 durante la PE no afectó a la capacidad funcional, a la presión arterial, a la frecuencia cardiaca ni incrementó las arritmias ventriculares. (AU)

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.MethodsRetrospective 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.ResultsOne 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).ConclusionsThe use of surgical or FFP2 face masks during the TMT did not affect functional capacity, blood pressure, heart rate, or increased ventricular arrhythmias. (AU)

Impact of face masks during the treadmill exercise tests. / Impacto del uso de la mascarilla durante la realización de la prueba de esfuerzo en tapiz rodante.

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.

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. 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.

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. 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.

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. 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.

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). 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.

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. 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.

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). 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.

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. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined. RESULTS: From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p = 0.001), whereas anatomic complexity or defect group were not. CONCLUSIONS: COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity.

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). 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 m2 and normal to mild albuminuria levels. Having proteinuria, but not albuminuria, was independently associated with a worse outcome.

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. 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.

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. 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.

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. 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.

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. 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/m2, 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.

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. 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.

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. 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.

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. 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.