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BACKGROUND: Eisenmenger syndrome is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of Eisenmenger syndrome patients. METHODS: In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters. RESULTS: We studied 1098 patients (median age, 34.4 years; range, 16.1-84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4-5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24-1.59; P<0.001), pretricuspid shunt (HR, 1.56; 95% CI, 1.02-2.39; P=0.041), oxygen saturation at rest (HR, 0.53/10%; 95% CI, 0.43-0.65; P<0.001), presence of sinus rhythm (HR, 0.53; 95% CI, 0.32-0.88; P=0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59-3.66; P<0.001) remained significant predictors of death. CONCLUSIONS: There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.
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Complejo de Eisenmenger/diagnóstico , Complejo de Eisenmenger/mortalidad , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores , Ecocardiografía , Complejo de Eisenmenger/terapia , Electrocardiografía , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Consumo de Oxígeno , Fenotipo , Pronóstico , Modelos de Riesgos Proporcionales , Medición de Riesgo , Factores de Riesgo , Índice de Severidad de la Enfermedad , Prueba de Paso , Adulto JovenRESUMEN
Monitoring the effect of dabigatran (Pradaxa®) is challenging. The aim of this study was to evaluate if thrombelastography reaction time (TEG® R) could detect the anticoagulant effect of dabigatran showing a correlation between TEG® R, Hemoclot Thrombin Inhibitor (HTI) assay and Ecarin Clotting Time (ECT) in patients with non-valvular atrial fibrillation (NVAF). Blood samples from 35 AF patients receiving either 110 mg (n 19) or 150 mg (n 16) dabigatran twice daily were analyzed with TEG®, HTI and ECT 2-3 h after dabigatran intake. All patients had prolonged TEG® R. The patients receiving dabigatran 110 mg ×2 had a TEG® R mean 14.2 min (range 9.1-25), a mean dabigatran concentration measured by HTI of 268.5 ng/mL (range 54-837 ng/mL) and by ECT of 355.7 ng/mL (range 40-1020 ng/mL). The corresponding numbers for patients receiving dabigatran 150 mg ×2 were TEG® R mean of 12.5 min (range 9.2-23.2 min), mean dabigatran concentration of 179.2 ng/mL by HTI (range 26-687 ng/mL) and by ECT 225.1 ng/mL (range 42-1020 ng/mL). The two dosage groups had comparable anticoagulation demonstrated by equally prolonged TEG® R (p = .909), HTI (p = .707) and ECT (p = .567). No difference in creatinine levels in the two dosage groups was observed (p = .204) though patients with dabigatran concentration >400 ng/mL had significantly higher creatinine levels (p = .001). Large individual variation of the anticoagulant response was observed. Some patients had TEG® R values up to three times upper normal limit with immediate risk of bleeding. Our data indicate that TEG® R reflected dabigatran levels in NVAF patients and that TEG® R correlated to HTI and ECT.
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Anticoagulantes/uso terapéutico , Antitrombinas/uso terapéutico , Fibrilación Atrial/sangre , Fibrilación Atrial/tratamiento farmacológico , Dabigatrán/uso terapéutico , Endopeptidasas/uso terapéutico , Tromboelastografía , Adulto , Anciano , Anciano de 80 o más Años , Fibrilación Atrial/fisiopatología , Pruebas de Coagulación Sanguínea , Demografía , Relación Dosis-Respuesta a Droga , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
AIMS: Eisenmenger syndrome (ES) is associated with considerable morbidity and mortality. Therapeutic strategies have changed during the 2000s in conjunction with an emphasis on specialist follow-up. The aim of this study was to determine the cause-specific mortality in ES and evaluate any relevant changes between 1977 and 2015. METHODS AND RESULTS: This is a retrospective, descriptive multicentre study. A total of 1546 patients (mean age 38.7 ± 15.4 years; 36% male) from 13 countries were included. Cause-specific mortality was examined before and after July 2006, 'early' and 'late', respectively. Over a median follow-up of 6.1 years (interquartile range 2.1-21.5 years) 558 deaths were recorded; cause-specific mortality was identified in 411 (74%) cases. Leading causes of death were heart failure (34%), infection (26%), sudden cardiac death (10%), thromboembolism (8%), haemorrhage (7%), and peri-procedural (7%). Heart failure deaths increased in the 'late' relative to the 'early' era (P = 0.032), whereas death from thromboembolic events and death in relation to cardiac and non-cardiac procedures decreased (P = 0.014, P = 0.014, P = 0.004, respectively). There was an increase in longevity in the 'late' vs. 'early' era (median survival 52.3 vs. 35.2 years, P < 0.001). CONCLUSION: The study shows that despite changes in therapy, care, and follow-up of ES in tertiary care centres, all-cause mortality including cardiac remains high. Patients from the 'late' era, however, die later and from chronic rather than acute cardiac causes, primarily heart failure, whereas peri-procedural and deaths due to haemoptysis have become less common. Lifelong vigilance in tertiary centres and further research for ES are clearly needed.
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Complejo de Eisenmenger/mortalidad , Adolescente , Adulto , Distribución por Edad , Anciano , Análisis de Varianza , Causas de Muerte/tendencias , Femenino , Humanos , Masculino , Persona de Mediana Edad , Análisis de Supervivencia , Adulto JovenRESUMEN
BACKGROUND: Robust data on changes in pulmonary valve replacement (PVR) procedural volume and predictors of bioprosthetic pulmonary valve (BPV) durability in patients with tetralogy of Fallot (TOF) are scarce. OBJECTIVES: This study sought to assess temporal trends in PVR procedural volume and BPV durability in a nationwide, retrospective TOF cohort. METHODS: Data were obtained from patient records. Robust linear regression was used to assess temporal trends in PVR procedural volume. Piecewise exponential additive mixed models were used to estimate BPV durability, defined as the time from implantation to redo PVR with death as a competing risk, and to assess risk factors for reduced durability. RESULTS: In total, 546 PVR were performed in 384 patients from 1976 to 2021. The annual number of PVR increased from 0.4 to 6.0 per million population (P < 0.001). In the last decade, the transcatheter PVR volume increased by 20% annually (P < 0.001), whereas the surgical PVR volume did not change significantly. The median BPV durability was 17 years (Q1: 10-Q3: 10 years-not applicable). There was no significant difference in the durability of different BPV after adjustment for confounders. Age at PVR (HR: 0.78 per 10 years from <1 year; 95% CI: 0.63-0.96; P = 0.02) and true inner valve diameter (9-17 mm vs 18-22 mm HR: 0.40; 95% CI: 0.22-0.73; P = 0.003 and 18-22 mm vs 23-30 mm HR: 0.59; 95% CI: 0.25-1.39; P = 0.23) were associated with reduced BPV durability in multivariate models. CONCLUSIONS: The PVR procedural volume has increased over time, with a greater increment in transcatheter than surgical PVR during the last decade. Younger patient age at PVR and a smaller true inner valve diameter predicted reduced BPV durability.
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Implantación de Prótesis de Válvulas Cardíacas , Insuficiencia de la Válvula Pulmonar , Válvula Pulmonar , Tetralogía de Fallot , Humanos , Niño , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/cirugía , Tetralogía de Fallot/diagnóstico por imagen , Tetralogía de Fallot/cirugía , Estudios Retrospectivos , Implantación de Prótesis de Válvulas Cardíacas/efectos adversos , Resultado del Tratamiento , Insuficiencia de la Válvula Pulmonar/diagnóstico por imagen , Insuficiencia de la Válvula Pulmonar/etiología , Insuficiencia de la Válvula Pulmonar/cirugíaRESUMEN
OBJECTIVES: To assess temporal changes in the surgical management of patients with tetralogy of Fallot including the timing of interventions, surgical techniques, reinterventions and survival in a nationwide cohort. METHODS: Patients with tetralogy of Fallot in Denmark were divided into 3 eras based on their year of birth: early (1977-1991), intermediate (1992-2006) and late (2007-2021). RESULTS: The cohort consisted of 745 patients. Median follow-up was 21.2 years (13.7-30.5). There was a temporal trend towards less shunt palliation (-0.3% per year, 95% CI -0.05 to -0.1). Median age at intracardiac repair was 2.9 years (1.8-5.0), 0.8 years (0.5-1.3) and 0.5 years (0.4-0.7) (P < 0.001) in the early, intermediate and late era, respectively. There was a temporal trend towards less valve-sparing repair (-0.7% per year, 95% CI -0.5 to -1.0) and more repair with transannular patches (0.7% per year, 95% CI 0.5-1.0). Survival at 10 years was 79% (64-76), 90% (87-93) and 95% (92-98) (P < 0.001) and pulmonary valve replacement within the first 10 years after intracardiac repair was performed in 3% (1-6), 12% (8-16) and 21% (13-29) (P < 0.001) in the early, intermediate and late era, respectively. CONCLUSIONS: There was a temporal trend towards less shunt palliation and intracardiac repair at a younger age with more use of transannular patches. While survival throughout childhood and adolescence has improved, more patients undergo pulmonary valve replacement during the first 10 years after intracardiac repair.
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Procedimientos Quirúrgicos Cardíacos , Válvula Pulmonar , Tetralogía de Fallot , Adolescente , Humanos , Lactante , Niño , Preescolar , Tetralogía de Fallot/cirugía , Estudios de Cohortes , Válvula Pulmonar/cirugía , Procedimientos Quirúrgicos Cardíacos/métodos , Reoperación , Dinamarca/epidemiología , Resultado del Tratamiento , Estudios RetrospectivosRESUMEN
Background: At the beginning of the COVID-19 pandemic, professionals in charge of particularly vulnerable populations, such as adult congenital heart disease (ACHD) patients, were confronted with difficult decision-making. We aimed to assess changes in risk stratification and outcomes of ACHD patients suffering from COVID-19 between March 2020 and April 2021. Methods and results: Risk stratification among ACHD experts (before and after the first outcome data were available) was assessed by means of questionnaires. In addition, COVID-19 cases and the corresponding patient characteristics were recorded among participating centres. Predictors for the outcome of interest (complicated disease course) were assessed by means of multivariable logistic regression models calculated with cluster-robust standard errors. When assessing the importance of general and ACHD specific risk factors for a complicated disease course, their overall importance and the corresponding risk perception among ACHD experts decreased over time. Overall, 638 patients (n = 168 during the first wave and n = 470 during the subsequent waves) were included (median age 34 years, 52% women). Main independent predictors for a complicated disease course were male sex, increasing age, a BMI >25 kg/m2, having ≥2 comorbidities, suffering from a cyanotic heart disease or having suffered COVID-19 in the first wave vs. subsequent waves. Conclusions: Apart from cyanotic heart disease, general risk factors for poor outcome in case of COVID-19 reported in the general population are equally important among ACHD patients. Risk perception among ACHD experts decreased during the course of the pandemic.
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Background Little is known about the cause of death (CoD) in patients with transposition of the great arteries palliated with a Mustard or Senning procedure. The aim was to describe the CoD for patients with the Mustard and Senning procedure during short- (<10 years), mid- (10-20 years), and long-term (>20 years) follow-up after the operation. Methods and Results This is a retrospective, descriptive multicenter cohort study including all Nordic patients (Denmark, Finland, Norway, and Sweden) who underwent a Mustard or Senning procedure between 1967 and 2003. Patients who died within 30 days after the index operation were excluded. Among 968 patients with Mustard/Senning palliated transposition of the great arteries, 814 patients were eligible for the study, with a mean follow-up of 33.6 years. The estimated risk of all-cause mortality reached 36.0% after 43 years of follow-up, and the risk of death was highest among male patients as compared with female patients (P=0.004). The most common CoD was sudden cardiac death (SCD), followed by heart failure/heart transplantation accounting for 29% and 27%, respectively. During short-, mid-, and long-term follow-up, there was a change in CoD with SCD accounting for 23.7%, 46.6%, and 19.0% (P=0.002) and heart failure/heart transplantation 18.6%, 22.4%, and 46.6% (P=0.0005), respectively. Conclusions Among patients corrected with Mustard or Senning transposition of the great arteries, the most common CoD is SCD followed by heart failure/heart transplantation. The CoD changes as the patients age, with SCD as the most common cause in adolescence and heart failure as the dominant cause in adulthood. Furthermore, the risk of all-cause mortality, SCD, and death attributable to heart failure or heart transplantation was increased in men >10 years after the Mustard/Senning operation.
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Procedimientos Quirúrgicos Cardíacos , Insuficiencia Cardíaca , Transposición de los Grandes Vasos , Adolescente , Adulto , Arterias , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Causas de Muerte , Estudios de Cohortes , Muerte Súbita Cardíaca/epidemiología , Muerte Súbita Cardíaca/etiología , Femenino , Estudios de Seguimiento , Insuficiencia Cardíaca/etiología , Humanos , Masculino , Estudios RetrospectivosRESUMEN
Over the last decades, remarkable advances in survival in patients with congenital heart disease (CHD) have been reported. Currently, 90% of infants born with CHD can expect to reach adulthood. Moderate and severe CHD is associated with increased perioperative mortality. To ensure optimal management of CHD patients undergoing non-cardiac surgery, preoperative risk assessment is pivotal, along with a multidisciplinary approach and collaboration across hospitals. The objective of this review is to provide a simple model to identify CHD patients at risk prior to non-cardiac surgery.
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Cardiopatías Congénitas , Adulto , Humanos , Lactante , Medición de RiesgoRESUMEN
AIMS: Patients with adult congenital heart disease (ACHD) are a potentially vulnerable patient cohort in case of COVID-19. Some cardiac defects may be associated with a poor COVID-19 outcome. Risk estimation in ACHD is currently based on expert opinion. The aim of this study was to collect clinical outcome data and to identify risk factors for a complicated course of COVID-19 in patients with ACHD. METHODS: Twenty-five ACHD centres in nine European countries participated in the study. Consecutive patients with ACHD diagnosed with COVID-19 presenting to one of the participating centres between 27 March and 6 June 2020 were included. A complicated disease course was defined as hospitalisation for COVID-19 requiring non-invasive or invasive ventilation and/or inotropic support, or a fatal outcome. RESULTS: Of 105 patients with a mean age of 38±13 years (58% women), 13 had a complicated disease course, of whom 5 died. In univariable analysis, age (OR 1.3, 95% CI 1.1 to 1.7, per 5 years), ≥2 comorbidities (OR 7.1, 95% CI 2.1 to 24.5), body mass index of >25 kg/m2 (OR 7.2, 95% CI 1.9 to 28.3) and cyanotic heart disease (OR 13.2, 95% CI 2.5 to 68.4) were associated with a complicated disease course. In a multivariable logistic regression model, cyanotic heart disease was the most important predictor (OR 60.0, 95% CI 7.6 to 474.0). CONCLUSIONS: Among patients with ACHD, general risk factors (age, obesity and multiple comorbidities) are associated with an increased risk of complicated COVID-19 course. Congenital cardiac defects at particularly high risk were cyanotic lesions, including unrepaired cyanotic defects or Eisenmenger syndrome.
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OBJECTIVE: The optimal timing for transplantation is unclear in patients with Eisenmenger syndrome (ES). We investigated post-transplantation survival and transplantation-specific morbidity after heart-lung transplantation (HLTx) or lung transplantation (LTx) in a cohort of Nordic patients with ES to aid decision-making for scheduling transplantation. METHODS: We performed a retrospective, descriptive, population-based study of patients with ES who underwent transplantation from 1985 to 2012. RESULTS: Among 714 patients with ES in the Nordic region, 63 (9%) underwent transplantation. The median age at transplantation was 31.9 (IQR 21.1-42.3) years. Within 30 days after transplantation, seven patients (11%) died. The median survival was 12.0 (95% CI 7.6 to 16.4) years and the overall 1-year, 5-year, 10-year and 15-year survival rates were 84.1%, 69.7%, 55.8% and 40.6%, respectively. For patients alive 1 year post-transplantation, the median conditional survival was 14.8 years (95% CI 8.0 to 21.8), with 5-year, 10-year and 15-year survival rates of 83.3%, 67.2% and 50.0%, respectively. There was no difference in median survival after HLTx (n=57) and LTx (n=6) (14.9 vs 10.6 years, p=0.718). Median cardiac allograft vasculopathy, bronchiolitis obliterans syndrome and dialysis/kidney transplantation-free survival rates were 11.2 (95% CI 7.8 to 14.6), 6.9 (95% CI 2.6 to 11.1) and 11.2 (95% CI 8.8 to 13.7) years, respectively. The leading causes of death after the perioperative period were infection (36.7%), bronchiolitis obliterans syndrome (23.3%) and heart failure (13.3%). CONCLUSIONS: This study shows that satisfactory post-transplantation survival, comparable with contemporary HTx and LTx data, without severe comorbidities such as cardiac allograft vasculopathy, bronchiolitis obliterans syndrome and dialysis, is achievable in patients with ES, with a conditional survival of nearly 15 years.
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Complejo de Eisenmenger/cirugía , Trasplante de Corazón-Pulmón , Trasplante de Pulmón , Adolescente , Adulto , Niño , Toma de Decisiones Clínicas , Técnicas de Apoyo para la Decisión , Complejo de Eisenmenger/diagnóstico por imagen , Complejo de Eisenmenger/mortalidad , Complejo de Eisenmenger/fisiopatología , Femenino , Trasplante de Corazón-Pulmón/efectos adversos , Trasplante de Corazón-Pulmón/mortalidad , Humanos , Trasplante de Pulmón/efectos adversos , Trasplante de Pulmón/mortalidad , Masculino , Selección de Paciente , Complicaciones Posoperatorias/mortalidad , Valor Predictivo de las Pruebas , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Países Escandinavos y Nórdicos , Factores de Tiempo , Tiempo de Tratamiento , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND: Patients with cyanotic congenital heart disease (CCHD) may have a low burden of atherosclerosis. Endothelial dysfunction is an early stage of atherosclerosis and endothelial function is previously studied in smaller CCHD groups with different techniques and variable results. We aimed to examine endothelial function and carotid atherosclerosis in a larger group of CCHD patients. METHODS: This multicentre study assessed endothelial function in adults with CCHD and controls by measuring the dilatory response of the brachial artery to post-ischemic hyperaemia (endothelium-dependent flow-mediated-vasodilatation (FMD)), and to nitroglycerin (endothelium-independent nitroglycerin-induced dilatation (NID)). Flow was measured at baseline and after ischaemia (reactive hyperaemia). Carotid-intima-media-thickness (CIMT), prevalence of carotid plaque and plaque thickness (cPT-max) were evaluated ultrasonographically. Lipoproteins, inflammatory and vascular markers, including sphingosine-1-phosphate (S1P) were measured. RESULTS: Forty-five patients with CCHD (median age 50 years) and 45 matched controls (median age 52 years) were included. The patients presented with lower reactive hyperaemia (409 ± 114% vs. 611 ± 248%, p < 0.0001), however preserved FMD response compared to controls (106.5 ± 8.3% vs. 106.4 ± 6.1%, p = 0.95). In contrast, NID was lower in the patients (110.5 ± 6.1% vs. 115.1 ± 7.4%, p = 0.053). There was no difference in CIMT, carotid plaque or cPT-max. The patients presented with lower high-density-lipoprotein cholesterol, and higher level of inflammatory markers and S1P. CONCLUSION: Adults with CCHD had preserved FMD in the brachial artery, but impaired NID response and lower reactive hyperaemia than controls. The preserved FMD and the comparable prevalence of carotid atherosclerosis indicate that CCHD patients have the same risk of atherosclerosis as controls.
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INTRODUCTION: Survival in patients with cyanotic congenital heart disease (CCHD) has improved dramatically. The result is an ageing population with risk of acquired heart disease. Previous small uncontrolled studies suggested that these patients are protected against the development of atherosclerosis. To test this hypothesis, we sought to determine the prevalence of subclinical atherosclerosis in a larger population of patients with CCHD. METHOD: We compared the prevalence of subclinical atherosclerosis in adult CCHD patients from Denmark, Sweden, Norway and Australia, with that in age-, sex-, smoking status-, and body mass index matched controls. Coronary artery atherosclerosis was assessed on computed tomography with coronary artery calcification (CAC) score. Subclinical atherosclerosis was defined by CAC-scoreâ¯>â¯0. Carotid artery atherosclerosis was evaluated using ultrasound by measuring carotid plaque thickness (cPT-max) and carotid intima media thickness (CIMT). Lipid status was evaluated as an important atherosclerotic risk factor. RESULTS: Seventy-four patients with CCHD (57% women, median age 49.5â¯years) and 74 matched controls (57% women, median age 50.0â¯years) were included. There were no differences between the groups in: CAC-scoreâ¯>â¯0 (21% vs. 19%, respectively; pâ¯=â¯0.8), carotid plaques (19% vs. 9%, respectively; pâ¯=â¯0.1), cPT-max (2.3â¯mm vs. 2.8â¯mm, respectively; pâ¯=â¯0.1) or CIMT (0.61â¯mm vs. 0.61â¯mm, respectively; pâ¯=â¯0.98). And further no significant differences in lipoprotein concentrations measured by ultracentrifugation. CONCLUSION: Young adults with CCHD have similar cardiovascular risk factor profiles and measures of subclinical atherosclerosis, compared with controls. Given their increasing life expectancies, athero-preventive strategies should be an important part of their clinical management.
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Enfermedades de las Arterias Carótidas/diagnóstico por imagen , Enfermedad de la Arteria Coronaria/diagnóstico por imagen , Cianosis/diagnóstico por imagen , Cardiopatías Congénitas/diagnóstico por imagen , Adulto , Anciano , Enfermedades de las Arterias Carótidas/epidemiología , Enfermedad de la Arteria Coronaria/epidemiología , Cianosis/epidemiología , Femenino , Cardiopatías Congénitas/epidemiología , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Adulto JovenRESUMEN
Improved treatment options in paediatric cardiology and congenital heart surgery have resulted in an ageing population of patients with cyanotic congenital heart disease (CCHD). The risk of acquired heart disease such as atherosclerosis increases with age.Previous studies have speculated whether patients with CCHD are protected against atherosclerosis. Results have shown that the coronary arteries of patients with CCHD are free from plaques and stenosis. Decreased carotid intima-media thickness and low total plasma cholesterol may indicate a reduced risk of later development of atherosclerosis. However, the evidence is still sparse and questionable, and a reasonable explanation for the decreased risk of developing atherosclerosis in patients with CCHD is still missing.This review provides an overview of what is known about the prevalence and potential causes of the reduced risk of atherosclerosis in patients with CCHD.
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Aterosclerosis/epidemiología , Cardiopatías Congénitas/complicaciones , Aterosclerosis/etiología , Salud Global , Cardiopatías Congénitas/epidemiología , Humanos , PrevalenciaRESUMEN
OBJECTIVE: Improved diagnostic tools, timely closure of the shunt and a better understanding of the complexity of Eisenmenger syndrome (ES) have led to improved care and treatment in tertiary centres. These may have decreased the incidence of ES and improved survival of patients with ES, although evidence is still lacking. The aim of this study was to investigate temporal changes in incidence, prevalence and mortality in patients with ES for 35 years in the Nordic region. METHODS: This was a retrospective population-based study including 714 patients with ES. Survival analysis was performed based on all-cause mortality and accounting for immortal time bias. RESULTS: The incidence of ES decreased from 2.5/million inhabitants/year in 1977 to 0.2/million inhabitants/year in 2012. Correspondingly, prevalence decreased from 24.6 to 11.9/million inhabitants. The median survival was 38.4 years, with 20-year, 40-year and 60-year survival of 72.5%, 48.4%, and 21.3%, respectively. Complex lesions and Down syndrome were independently associated with worse survival (HR 2.2, p<0.001 and HR 1.8, p<0.001, respectively). Age at death increased from 27.7 years in the period from 1977 to 1992, to 46.3 years from July 2006 to 2012 (p<0.001). CONCLUSIONS: The incidence and prevalence of ES in the Nordic region have decreased markedly during the last decades. Furthermore, the median age at death increased throughout the study period, indicating prolonged life expectancy in the ES population. However, increasing age represents decreased incidence, rather than improved survival. Nonetheless, longevity with ES is still shorter than in the background population.
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Complejo de Eisenmenger/epidemiología , Predicción , Vigilancia de la Población/métodos , Sistema de Registros , Medición de Riesgo/métodos , Adulto , Causas de Muerte/tendencias , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Prevalencia , Estudios Retrospectivos , Países Escandinavos y Nórdicos/epidemiología , Tasa de Supervivencia/tendenciasAsunto(s)
Complejo de Eisenmenger/diagnóstico por imagen , Hemoptisis/diagnóstico por imagen , Embolia Pulmonar/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Adulto , Complejo de Eisenmenger/complicaciones , Resultado Fatal , Hemoptisis/etiología , Humanos , Masculino , Embolia Pulmonar/etiologíaRESUMEN
PURPOSE: To assess spectrophotometric oximetry across a broad range of arterial saturation levels and to study the effect of chronic systemic hypoxemia on retinal oxygen extraction. METHODS: The study included 16 patients with Eisenmenger syndrome, a cyanotic cardiac defect, and 17 healthy volunteers. Oxygen saturation in selected major retinal arteries and veins was assessed using noninvasive spectrophotometric oximetry. Arterial blood gases were determined within 1 day of the ophthalmic examination in blood samples from the femoral artery. RESULTS: The retinal arterial oxygen saturation of 81% ± 9% (mean ± SD) in patients with Eisenmenger syndrome was subnormal and demonstrated more interindividual variation than the 93% ± 3% observed in healthy subjects (P < 0.001). A comparable difference was found for the respective retinal venous oxygen saturations of 44% ± 12% and 59% ± 5% (P < 0.001). Fractional arteriovenous oxygen extraction was comparable between the two groups (37% ± 6% and 34% ± 5%, respectively; P = 0.29). Retinal and femoral artery oxygen saturation were correlated (ρ = 0.82; P < 0.001), the former approximating the latter at least as well as fingertip oximetry. CONCLUSIONS: When compared to arterial blood gas analysis of blood samples drawn by arterial puncture, the gold standard in the field, fundus oximetry was found to be in good overall agreement with the arterial blood samples. Blood flow measurements will be needed to determine whether the systemic hypoxia is completely compensated, as suggested by oxygen extraction being comparable between the two groups.
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Complejo de Eisenmenger/sangre , Hipoxia/sangre , Oxígeno/sangre , Arteria Retiniana/metabolismo , Vena Retiniana/metabolismo , Adulto , Anciano , Análisis de los Gases de la Sangre , Monitoreo de Gas Sanguíneo Transcutáneo , Femenino , Humanos , Masculino , Persona de Mediana Edad , Consumo de Oxígeno , Adulto JovenRESUMEN
Congenital heart disease with left-to-right shunt can induce proliferation, vasoconstriction and thrombosis in the pulmonary vascular bed. Eventually, the patient may develop Eisenmenger syndrome defined as pulmonary arterial hypertension caused by high pulmonary vascular resistance with right-to-left shunt and cyanosis. Patients with Eisenmenger syndrome suffer a high risk of complications in connection with acute medical conditions, extra-cardiac surgery and pregnancy. This article describes the precautions that should be taken to reduce morbidity and mortality in these patients.
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Complejo de Eisenmenger , Complejo de Eisenmenger/complicaciones , Complejo de Eisenmenger/diagnóstico , Complejo de Eisenmenger/mortalidad , Complejo de Eisenmenger/terapia , Femenino , Humanos , Masculino , Embarazo , Complicaciones Cardiovasculares del Embarazo/mortalidad , Pronóstico , Factores de RiesgoRESUMEN
BACKGROUND: Lipid levels generally fall after an acute myocardial infarction. This study was conducted to see what trends lipid levels had in patients who underwent operation for critical limb ischemia. METHODS: The study was prospective and included 30 patients who underwent operation for critical limb ischemia. Serum lipid profiles and C-reactive protein were analyzed before surgery and 3 months after peripheral bypass surgery. In addition, comparison of lipid levels after surgery was made with 287 healthy and 283 ischemic heart disease controls from The Copenhagen City Heart Study. RESULTS: Total, low density lipoprotein, and high density lipoprotein cholesterol levels were found to be significantly lower before surgery than 3 months after surgery (P <.001). In contrast, C-reactive protein was higher before surgery and decreased after 3 months (P <.0001). An inverse linear correlation was found between total cholesterol/low density lipoprotein cholesterol and C-reactive protein (P <.001 and P <.04, respectively). Comparison with controls showed that cholesterol levels 3 months after surgery were significantly lower (P <.009). CONCLUSION: Serum cholesterol levels are not reliable in the preoperative period and should therefore be measured at least 3 months after surgery when they seem to approach a more representative level.