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Patients with Fontan circulation (FC) have a high incidence of clinical complications. However, no biomarker is able to accurately stratify risk. The aim of this study was to analyze the relationship between biomarkers and clinical complications, including carbohydrate antigen 125 (CA125) for the first time, and to propose a risk estimation based on a combination of biomarkers.Cross-sectional study of patients with FC. The clinical endpoint was the combination of heart failure, atrial arrhythmias, veno-venous fistulae, protein-losing enteropathy, or plastic bronchitis. Demographic, clinical, and laboratory variables were analyzed, including CA125, NT-proBNP, renal and liver function, and red cell distribution width (RDW). We performed univariate and multivariate analyses of the relationship between these variables and the composite endpoint. Cutoff values were calculated by ROC curves.We included 56 patients (27.4±7.8 years). A total of 34% showed the composite endpoint, with significantly higher CA125 levels (30.1 IU/mL vs 12.6 IU/mL; P=.001). In the multivariate model, the biomarkers related to the endpoint were LnCA125 (OR, 5.1; 95%CI, 1.2-22), RDW (OR, 1.8; 95%CI, 1.1-3.1), and FIB4 (OR, 38, 95%CI, 1.7-855). The cutoff points were CA125 ≥ 20 U/mL, FIB4 ≥ 0.75, and RDW ≥ 14.5%, and the probability of the occurrence of the endpoint was 81% if ≥ 2 biomarkers were elevated.CA125 elevation is associated with a higher prevalence of complications in patients with Fontan-type circulation. CA125 levels ≥ 20U/mL, FIB4 ≥ 0.75 and RDW ≥ 14.5% identify with a high probability the clinical failure of FC.

[ES] Introducción y objetivos: Hay pocos datos sobre la evolución en adultos de la dilatación de la raíz neoaórtica (RAO) y la insuficiencia valvular neoaórtica (IA) tras la cirugía de switch arterial (SA) en la transposición de grandes arterias. Métodos: Análisis retrospectivo de 152 pacientes con transposición de grandes arterias, mayores de 15 años, intervenidos mediante SA y seguidos durante 4,9 ± 3,3 años en 2 centros de referencia. Se analizaron los cambios de diámetro de la RAO ajustados a superficie corporal y la progresión a grado moderado/grave de la IA con ecocardiografías seriadas. Se realizó un modelo de regresión de Cox para identificar factores predictores de progresión de la IA. Resultados: Inicialmente, 4 pacientes (2,6%) presentaban IA grave (3 habían precisado cirugía valvular) y 9 (5,9%) moderada. La RAO basal media era 20,05 ± 2,4 mm/m2, y al final del seguimiento, 20,73 ± 2,8 mm/m2 (p < 0,001), con un crecimiento medio de 0,14 (IC95%, 0,07-0,2) mm/m2/año. La IA progresó en 20 (13,5%) y 6 (4%) fueron intervenidos. La progresión de IA se asoció con válvula bicúspide, IA inicial, dilatación de la RAO inicial y crecimiento de la RAO. La válvula bicúspide (HR = 3,3; IC95%, 1,1-15,2; p = 0,037), la IA inicial (HR = 5,9; IC95%, 1,6-59,2; p = 0,006) y el crecimiento de la RAO (HR = 4,1; IC95%, 2-13,5; p = 0,023) resultaron predictores independientes. Conclusiones: La dilatación de la RAO y la IA progresan en el adulto joven intervenido mediante SA. La válvula bicúspide, la IA basal y el crecimiento de la RAO son predictores de progresión de IA., [EN] Introduction and objectives: There are limited data on the long-term development of neoaortic root dilatation (NRD) and neoaortic valve regurgitation (AR) after arterial switch operation (ASO) for transposition of the great arteries during adult life. Methods: We performed a retrospective longitudinal analysis of 152 patients older than 15 years who underwent ASO for transposition of the great arteries and who were followed-up for 4.9 ± 3.3 years in 2 referral centers. Sequential changes in body surface-adjusted aortic root dimensions and progression to moderate/severe AR were determined in patients with 2 or more echocardiographic examinations. Risk factors for dilatation were tested by Cox regression to identify predictors of AR progression. Results: At baseline, moderate AR was present in 9 patients (5.9%) and severe AR in 4 (2.6%), of whom 3 had required aortic valve surgery. Initially, the median neoaortic root dimension was 20.05 ± 2.4 mm/m2, which increased significantly to 20.73 ± 2.8 mm/m2 (P < .001) at the end of follow-up. The mean change over time was 0.14 mm/m2/y (95%CI, 0.07-0.2). Progressive AR was observed in 20 patients (13.5%) and 6 patients (4%) required aortic valve surgery. Progressive AR was associated with bicuspid valve, AR at baseline, NRD at baseline, and neoaortic root enlargement. Independent predictors were bicuspid valve (HR, 3.3; 95%CI, 1.1-15.2; P = .037), AR at baseline (HR, 5.9; 95%CI, 1.6-59.2; P = .006) and increase in NRD (HR, 4.1 95%CI, 2-13.5; P = .023). Conclusions: In adult life, NRD and AR progress over time after ASO. Predictors of progressive AR are bicuspid valve, AR at baseline, and increase in NRD.

Resumen Introduccion y objetivos La insuficiencia pulmonar (IP) es una complicacion frecuente tras la intervencion de cardiopatias congenitas. La expresion en leucocitos mononucleares circulantes (LMC) de los adrenoceptores (β1 y β2) y de las cinasas (GRK2, GRK3 y GRK5) refleja los cambios neurohumorales que se producen en la insuficiencia cardiaca (IC). El objetivo principal es describir la expresion genica de dichas moleculas en LMC de pacientes con IP grave. Metodos Estudio prospectivo que analizo la expresion de las moleculas descritas en LMC de pacientes con IP grave en comparacion con controles sanos y pacientes con IC avanzada. Resultados Se estudio a 35 pacientes con IP grave, 22 controles y 13 pacientes con IC. El analisis de comparaciones multiples mostro que en los controles la cantidad de ARN mensajero de adrenoceptor β2 era mayor que el que presentaban los pacientes con IP y con IC, con similar expresion en estos 2 grupos: 748,49 (intervalo, 1.703,87) frente a 402,80 (1.210,81) frente a 287,46 (685,69) (p = 0,001). Estos mismos hallazgos se obtuvieron en la expresion genica de GRK2: 760,89 (1.169,46) frente a 445,17 (1.190,69) frente a 284,09 (585,27) (p Conclusiones El patron de expresion genica de GRK2 y del adrenoceptor β2 de los pacientes con IP grave, como marcadores moleculares de disfuncion cardiaca, se encuentra alterado respecto a los controles y es similar al de los pacientes con IC avanzada.

INTRODUCTION AND OBJECTIVES There are limited data on the long-term development of neoaortic root dilatation (NRD) and neoaortic valve regurgitation (AR) after arterial switch operation (ASO) for transposition of the great arteries during adult life. METHODS We performed a retrospective longitudinal analysis of 152 patients older than 15 years who underwent ASO for transposition of the great arteries and who were followed-up for 4.9±3.3 years in 2 referral centers. Sequential changes in body surface-adjusted aortic root dimensions and progression to moderate/severe AR were determined in patients with 2 or more echocardiographic examinations. Risk factors for dilatation were tested by Cox regression to identify predictors of AR progression. RESULTS At baseline, moderate AR was present in 9 patients (5.9%) and severe AR in 4 (2.6%), of whom 3 had required aortic valve surgery. Initially, the median neoaortic root dimension was 20.05±2.4mm/m2, which increased significantly to 20.73±2.8mm/m2 (P

Background: Patients who have undergone the Fontan procedure are at risk of developing hepatic dysfunction. However, broad recommendations regarding liver monitoring are limited. The purpose of this study was to characterize the frequency of liver disease in adult Fontan patients using multimodality imaging (hepatic magnetic resonance imaging [MRI], acoustic radiation force impulse [ARFI] elastography, or hepatic ultrasound). Methods: In a prospective cross-sectional analysis of adult patients palliated with a Fontan procedure, hepatic MRI, ARFI, and hepatic ultrasound were used to assess for liver disease. The protocol compared (1) varying prevalence of liver disease based on each imaging technique, (2) agreement between different techniques, and (3) association between noninvasive imaging diagnosis of liver disease and clinical variables, including specific liver disease biomarkers. Results: Thirty-seven patients were enrolled. The ARFI results showed high wave propagation velocity in 35 patients (94.6%). All patients had some abnormality in the hepatic MRI. Specifically, 8 patients (21.6%) showed signs of chronic liver disease, 10 patients (27%) had significant liver fibrosis, and 27 patients (73%) had congestion. No correlation was found between liver stiffness measured as propagation velocity and hepatic MRI findings. Only 7 patients had an abnormal hepatic ultrasound study. Conclusions: There is an inherent liver injury in adult Fontan patients. Signs of liver disease were observed in most patients by both hepatic MRI and ARFI elastography but not by ultrasound imaging. Increased liver stiffness did not identify specific disease patterns from MRI, supporting the need for multimodality imaging to characterize liver disease in Fontan patients.

INTRODUCTION AND OBJECTIVES Pulmonary regurgitation (PR) is a frequent complication after repair of congenital heart disease. Lymphocyte expression of adrenoceptors (β1 and β2) and kinases (GRK2, GRK3, and GRK5) reflects the neurohumoral changes that occur in heart failure (HF). The main objective of this study was to describe the gene expression of these molecules in circulating lymphocytes in patients with severe PR. METHODS A prospective study was conducted to analyze lymphocyte expression of these molecules in patients with severe PR and compare it with expression in healthy controls and patients with advanced HF. RESULTS We studied 35 patients with severe PR, 22 healthy controls, and 13 patients with HF. Multiple comparisons analysis showed that β2-adrenoceptor gene expression levels were higher in the control group than in patients in the PR and HF groups and that expression in the latter 2 groups was similar (748.49 [rank 1703.87] vs 402.80 [rank 1210.81] vs 287.46 [rank 685.69] P = .001). Similar findings were obtained in gene expression of GRK2 (760.89 [rank 1169.46] vs 445.17 [rank 1190.69] vs 284.09 [rank 585.27] P < .001). There were no differences in expression levels of these molecules according to clinical variables in patients with PR. CONCLUSIONS The gene expression pattern of GRK2 and β2-adrenoceptor as molecular markers of cardiac dysfunction was altered in patients with severe PR compared with controls and was similar to expression in patients with advanced HF.

La enteropatia pierdeproteinas es una infrecuente pero muy grave complicacion que aparece en determinados pacientes que desarrollan linfangiectasia intestinal secundaria a un incremento de la presion venosa sistemica. Se han descrito diferentes tratamientos, con resultados dispares y en la mayoria de las ocasiones sin respuesta favorable. El objetivo del estudio es mostrar el trasplante cardiaco como una opcion terapeutica adecuada para pacientes sin respuesta positiva al tratamiento medico. En nuestro centro se ha trasplantado a 3 pacientes con estas caracteristicas, con un seguimiento medio de 11 ± 2 meses. Ningun paciente fallecio y en todos ellos se documento una reversibilidad de la enteropatia.

Protein-losing enteropathy is a rare but life-threatening complication that occurs in some patients who develop intestinal lymphangiectasis secondary to increased systemic venous pressure. Although different forms of treatment have been tried, with varying results, the majority were reported to be unsuccessful. The aim of this study was to demonstrate that heart transplantation may be an appropriate therapeutic option for patients who do not respond to medical treatment. At our center, we performed heart transplantations in three patients with this condition. The mean follow-up period was 11+/-2 months. No patient died and the enteropathy regressed in all three.

Background In renal transplantation, the degree of HLA matching has been reported to be negatively correlated with graft loss, with the number of rejections with complications. This association is less clear in heart transplantation, where there are contradictory studies, although most are consistent with those in renal transplantation. The objective of this study was to analyze differences in the probability of survival and the incidence of complications during follow-up according to the degree of HLA-A, -B, and -DR matching. Materials and methods Two hundred forty-three consecutive patients transplanted over a 13-year period were analyzed for age, gender, pretransplant factors associated with mortality, number of rejections and infections, incidence of acute graft failure, arterial hypertension, diabetes, and survival time with cause of death with reference to the number of HLA matches (zero to six). Exclusion criteria included retransplants, heart–lung transplants, pediatric transplants, and perioperative mortality. Groups were compared using the χ2 and ANOVA (Bonferroni posthoc test) tests. Kaplan-Meier survival curves were compared using the log rank test. The significance level was set at P Results The overall probability of survival of our series at 1, 5, and 10 years was 85%, 77%, and 60%, respectively. HLA-A, -B, and -DR compatibility: No significant differences were found when the curves were compared (log-rank: .005). The best survival rates were obtained with lower degrees of matching. No significant differences were found in the number of rejections or infections, although survival rates (P = .007) were higher among those with the lower degrees of matching. Conclusions A higher degree of HLA-A, -B, and -DR matching did not have a positive effect on heart transplant patient survival, rejection episodes or infections.

Introduction and objective. In recent years, the relation between biological markers of inflammation and prognosis in patients suffering from acute coronary syndromes has been investigated. The aim of this study was to evaluate the association between baseline fibrinogen concentrations and the development of clinical events in patients admitted with suspicion of unstable angina and non-Q-wave myocardial infarction. Material and method. Levels of fibrinogen at enrollment were analyzed in 325 consecutive patients with acute coronary syndromes. Fibrinogen values were divided into tertiles and the incidence of clinical events was evaluated at each level. The combination of death and/or myocardial infarction was the main endpoint. Results. Fibrinogen levels were significantly higher in patients who subsequently had myocardial infarction, cardiac death, or both during follow up. The probabilities of death and/or myocardial infarction were 6%, 13%, and 29% (p < 0.0001), respectively, in patients grouped by fibrinogen tertiles (304, 305-374 and 375 mg/dl). Multivariate predictors of combined events were age, previous angina, ST-segment depression in the admission ECG, and fibrinogen into tertiles. The adjusted hazard ratio (95% CI) for patients in the upper tertile was 4.8 (1.614; p = 0.004). Conclusions. High fibrinogen levels were related to a less favorable long-term or short-term outcome in patients admitted for suspicion of unstable angina and non-Qwave myocardial infarction. This association persists after adjustment for other classical risk factors such as age, prior angina, and ST-segment depression in the ECG.

Objective: The development of liver fibrosis and cirrhosis due to long-standing liver congestion is known to occur in adult patients with Fontan circulation. Hepatic elastography has shown to be a useful tool for the noninvasive assessment and staging of liver fibrosis in chronic liver diseases, although the utility of this technique in Fontan patients remains to be adequately studied. Methods: Twenty-one patients with Fontan circulation underwent an abdominal ultrasound and an acoustic radiation force impulse (ARFI) elastography. In order to compare the results from this group, a cohort of 14 healthy controls and another group containing 17 patients with cirrhosis were included. The association between the velocity values measured with elastography and clinical and analytical parameters were also studied. Results: Mean shear waves propagation velocity in liver tissue in the Fontan group was 1.86 ± 0.5 m/s, with 76% of patients over the cirrhosis threshold (1.55 m/s). The control group had a mean velocity of 1.09 ± 0.06 m/s, while the cirrhotic group obtained 2.71 ± 0.51 m/s. Seven patients with Fontan circulation had increased liver enzymes. Liver ultrasound showed evidence of chronic liver disease in six patients. Velocity values obtained in the presence or absence of analytical or liver ultrasound abnormalities showed significant differences in the univariate analysis ( P = .04 and P = .03 respectively). Conclusion: In conclusion, ARFI elastography showed increased wave propagation velocity values in the Fontan population suggesting increased liver stiffness which could be related to advanced fibrosis. A statistically significant association between ARFI values and the presence of analytical and ultrasound abnormalities has been demonstrated.

In recent years, the relation between biological markers of inflammation and prognosis in patients suffering from acute coronary syndromes has been investigated. The aim of this study was to evaluate the association between baseline fibrinogen concentrations and the development of clinical events in patients admitted with suspicion of unstable angina and non-Q-wave myocardial infarction.Levels of fibrinogen at enrollment were analyzed in 325 consecutive patients with acute coronary syndromes. Fibrinogen values were divided into tertiles and the incidence of clinical events was evaluated at each level. The combination of death and/or myocardial infarction was the main endpoint.Fibrinogen levels were significantly higher in patients who subsequently had myocardial infarction, cardiac death, or both during follow up. The probabilities of death and/or myocardial infarction were 6%, 13%, and 29% (p0.0001), respectively, in patients grouped by fibrinogen tertiles (304, 305-374 and 375 mg/dl). Multivariate predictors of combined events were age, previous angina, ST-segment depression in the admission ECG, and fibrinogen into tertiles. The adjusted hazard ratio (95% CI) for patients in the upper tertile was 4.8 (1.6-14; p = 0.004).High fibrinogen levels were related to a less favorable long-term or short-term outcome in patients admitted for suspicion of unstable angina and non-Q-wave myocardial infarction. This association persists after adjustment for other classical risk factors such as age, prior angina, and ST-segment depression in the ECG.

HEART TRANSPLANTATION (HT) is presently the treatment of choice for severe heart disease in patients presenting poor clinical conditions who are not amenable to alternative therapy. The increase in posttransplant survival, particularly following the introduction of cyclosporine, has led to an increasing number of patients who survive for long periods of time. However, all such patients require chronic maintainance immunosuppressive therapy to reduce the incidence and severity of graft rejection. Most centers use a triple therapy protocol consisting of cyclosporine, azathioprine, and steroids. Since immune activity and the tendency toward rejection is greater in the first months after transplantation, most regimens administer higher doses in the early stages, followed by a gradual reduction to maintenance levels. However, despite the use of increasingly lower doses, the morbidity associated with immunosuppressive drugs remains high. The present study investigated the frequency and progression of side effects produced by immunosuppressive medication continuously administered to patients following HT.

Background and purpose: Right ventricular dysfunction (RVD) has been described as an independent factor for morbidity and/ or mortality in various nosological entities. However, there are no data on its true

Right ventricular infarction usually occurs in association with inferior infarction, with no remarkable electrocardiographic signs in conventional leads. This report describes a patient with a previous inferior acute myocardial infarction who developed right ventricular infarction with significant anterior lead ST segment elevation (V1–V4) caused by the loss of two large right ventricular branches during a coronary angioplasty of the right coronary artery. The case is discussed and the literature is reviewed.

La endocarditis infecciosa precoz es una complicacion grave que se observa en el 3% de los pacientes portadores de protesis valvulares cardiacas y que sigue teniendo una mortalidad muy elevada. Aparece antes de los 12 meses del recambio valvular, es mas agresiva que la tardia, y esta producida fundamentalmente por estafilococos. Las manifestaciones clinicas estan relacionadas con la presencia de bacteriemias asociadas a signos de dehiscencia protesica. Presentamos un caso de endocarditis protesica precoz en posicion aortica complicada con insuficiencia aortica severa y fistula entre raiz aortica y auricula derecha, junto con alteracion electrocardiografica consistente en trastorno de la conduccion auriculoventricular e intraventricular.