Corazón univentricular sin cirugía de Fontan: el tipo de paliación importa / Univentricular hearts not undergoing Fontan: the type of palliation matters

Introducción y objetivos: La información sobre pacientes con fisiología univentricular (FU) y flujo pulmonar restrictivo no sometidos a cirugía de Fontan es escasa. El objetivo de este estudio es comparar la supervivencia y los eventos cardiovasculares en estos pacientes según el tipo de paliación.MétodosLos datos de pacientes con FU se obtuvieron de las bases de datos de 7 centros con unidades de cardiopatías congénitas del adulto. Se excluyó a los pacientes que completaron la circulación de Fontan o desarrollaron un síndrome de Eisenmenger. Se crearon 3 grupos según la fuente de flujo pulmonar: G1, flujo anterógrado pulmonar restrictivo; G2, shunt cavopulmonar, y G3, shunt aortopulmonar±shunt cavopulmonar. El objetivo principal fue la muerte.ResultadosSe identificó a 120 pacientes. La media de edad en la primera visita fue 32,2 años. El seguimiento medio fue de 7,1 años. Se asignó a 55 pacientes (45,8%) al G1, 30 (25%) al G2 y 35 (29,2%) al G3. Los pacientes del G3 tenían peores función renal, clase funcional y fracción de eyección en la primera visita y mostraron una disminución más marcada de la fracción de eyección durante el seguimiento, especialmente en comparación con el G1. Veinticuatro pacientes (20%) fallecieron, 38 (31,7%) ingresaron por insuficiencia cardiaca y 21 (17,5%) presentaron aleteo/fibrilación auricular durante el seguimiento. Estos eventos fueron más frecuentes en el G3 y al compararlos con los del G1 se encontraron diferencias significativas en muerte (HR=2,9; IC95%, 1,14-7,37; p=0,026) y aleteo/fibrilación auricular (HR=2,9; IC95%, 1,11-7,68; p=0,037).ConclusionesEl tipo de paliación de los pacientes con FU y flujo pulmonar restrictivo no sometidos a cirugía de Fontan identifica distintos perfiles. Los pacientes paliados con derivaciones aortopulmonares presentan un peor pronóstico, con más morbilidad y mortalidad. (AU)

Introduction and objectives: There is scarce information on patients with single ventricle physiology (SVP) and restricted pulmonary flow not undergoing Fontan circulation. This study aimed to compare survival and cardiovascular events in these patients according to the type of palliation.MethodsSVP patient data were obtained from the databases of the adult congenital heart disease units of 7 centers. Patients completing Fontan circulation or developing Eisenmenger syndrome were excluded. Three groups were created according to the source of pulmonary flow: G1 (restrictive pulmonary forward flow), G2 (cavopulmonary shunt), and G3 (aortopulmonary shunts±cavopulmonary shunt). The primary endpoint was death.ResultsWe identified 120 patients. Mean age at the first visit was 32.2 years. Mean follow-up was 7.1 years. Fifty-five patients (45.8%) were assigned to G1, 30 (25%) to G2, and 35 (29.2%) to G3. Patients in G3 had worse renal function, functional class, and ejection fraction at the first visit and a more marked ejection fraction decline during follow-up, especially when compared with G1. Twenty-four patients (20%) died, 38 (31.7%) were admitted for heart failure, and 21 (17.5%) had atrial flutter/fibrillation during follow-up. These events were more frequent in G3 and significant differences were found compared with G1 in terms of death (HR, 2.9; 95%CI, 1.14-7.37; P=.026) and atrial flutter/fibrillation (HR, 2.9; 95%CI, 1.11-7.68; P=.037).ConclusionsThe type of palliation in patients with SVP and restricted pulmonary flow not undergoing Fontan palliation identifies distinct profiles. Patients palliated with aortopulmonary shunts have an overall worse prognosis with higher morbidity and mortality. (AU)

Univentricular hearts not undergoing Fontan: the type of palliation matters.

INTRODUCTION AND OBJECTIVES: There is scarce information on patients with single ventricle physiology (SVP) and restricted pulmonary flow not undergoing Fontan circulation. This study aimed to compare survival and cardiovascular events in these patients according to the type of palliation. METHODS: SVP patient data were obtained from the databases of the adult congenital heart disease units of 7 centers. Patients completing Fontan circulation or developing Eisenmenger syndrome were excluded. Three groups were created according to the source of pulmonary flow: G1 (restrictive pulmonary forward flow), G2 (cavopulmonary shunt), and G3 (aortopulmonary shunts±cavopulmonary shunt). The primary endpoint was death. RESULTS: We identified 120 patients. Mean age at the first visit was 32.2 years. Mean follow-up was 7.1 years. Fifty-five patients (45.8%) were assigned to G1, 30 (25%) to G2, and 35 (29.2%) to G3. Patients in G3 had worse renal function, functional class, and ejection fraction at the first visit and a more marked ejection fraction decline during follow-up, especially when compared with G1. Twenty-four patients (20%) died, 38 (31.7%) were admitted for heart failure, and 21 (17.5%) had atrial flutter/fibrillation during follow-up. These events were more frequent in G3 and significant differences were found compared with G1 in terms of death (HR, 2.9; 95%CI, 1.14-7.37; P=.026) and atrial flutter/fibrillation (HR, 2.9; 95%CI, 1.11-7.68; P=.037). CONCLUSIONS: The type of palliation in patients with SVP and restricted pulmonary flow not undergoing Fontan palliation identifies distinct profiles. Patients palliated with aortopulmonary shunts have an overall worse prognosis with higher morbidity and mortality.

CA125 como nuevo biomarcador en pacientes con circulación de Fontan / CA125: a new biomarker in patients with Fontan circulation

Introducción y objetivos Los pacientes con circulación de Fontan (CF) presentan una gran incidencia de complicaciones y ningún biomarcador estratifica el riesgo. El objetivo es analizar la asociación de biomarcadores con un perfil clínico de disfunción de la CF, incluyendo por primera vez el antígeno carbohidrato 125 (CA125), y proponer una estimación del riesgo basada en la combinación de biomarcadores. Métodos Estudio transversal de adultos con CF. Se consideró perfil clínico desfavorable el combinado de insuficiencia cardiaca, arritmias auriculares, fístulas venovenosas, enteropatía pierdeproteínas o bronquitis plástica. Se analizaron variables clínicas y analíticas, incluidos CA125, NT-proBNP, función renal y hepática y amplitud de distribución eritrocitaria (ADE). Se realizó un estudio univariado y multivariado de la relación de dichas complicaciones clínicas y curvas ROC para obtener puntos de corte. Resultados Se incluyó a 56 pacientes (media de edad, 27,4±7,8 años). El 34% tenía un perfil clínico desfavorable, con valores de CA125 significativamente mayores (30,1 frente a 12,6 UI/ml; p=0,001). LnCA125 (OR=5,1; IC95%, 1,2-22), ADE (OR=1,8; IC95%, 1,1-3.1) y FIB4 (OR=38; IC95%, 1,7-855) se asociaron con un perfil de disfunción clínica. Los puntos de corte fueron CA125 ≥ 20 U/ml, FIB4 ≥ 0,75 y ADE ≥ 14,5%, y la probabilidad de un perfil clínico desfavorable fue del 81% con 2 o más biomarcadores elevados. Conclusiones El aumento de CA125 se asocia con mayor prevalencia de complicaciones en pacientes con CF. Los valores de CA125 ≥ 20 U/ml, FIB4 ≥ 0,75 y ADE ≥ 14,5% identifican con alta probabilidad fracaso clínico de la CF. (AU)

Introduction and objectives 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. Methods 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. Results 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. Conclusions 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. (AU)

CA125: a new biomarker in patients with Fontan circulation.

INTRODUCTION AND OBJECTIVES: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Spatiotemporal Complexity of Vena Contracta and Mitral Regurgitation Grading Using Three-Dimensional Echocardiographic Analysis.

BACKGROUND: Spatiotemporal complexity of the color Doppler vena contracta challenging the assumption of a circular and constant orifice may lead to mitral regurgitation (MR) grading inconsistencies. Using 3D transesophageal echocardiography, we characterized spatiotemporal vena contracta complexity and its impact on MR severity grading. METHODS: In 192 patients with suspected moderate or severe MR (100 primary MR [PMR]; 92 secondary MR [SMR]), we performed three-dimensional vena contracta area (VCA) quantification using single-frame (midsystolic or VCAmid, maximum or VCAmax) and multiframe (VCAmean) methods, as well as measures of orifice shape (shape index) and systolic variation of VCA. Vena contracta complexity and intermethod discrepancies were analyzed and correlated with functional class and pulmonary vein flow (PVF) patterns and with cardiac magnetic resonance (CMR) in a subset of cases (n = 20). RESULTS: The vena contracta was noncircular (shape index > 1.5) in 90% of patients. Severe noncircularity (shape index > 3) was more prevalent in SMR than in PMR (32.4% vs 14.6%). Variations of the VCA were more prominent in SMR than in PMR. VCAmid showed a low grading agreement with VCAmax (62%) and high grading agreement with VCAmean (83.3%). Pulmonary vein flow systolic reversal was associated with MR severity by VCA in SMR but not in PMR. VCAmid and VCAmean showed a stronger association with systolic flow reversal than VCAmax (area under the curve, 0.88, 0.86, and 0.79, respectively). In the subset of patients with CMR quantification, severe MR by VCAmax was graded as nonsevere by CMR more frequently compared with VCAmid and VCAmean. CONCLUSIONS: Highly prevalent spatiotemporal vena contracta complexity features in MR challenge the assumption of a circular and constant orifice. VCAmid seems the best single-frame approximation to multiframe quantification, and VCAmax may lead to severity overestimation.

Evolutionary Description of Heart Transplantation and Heart-Lung Transplantation in Congenital Heart Disease.

BACKGROUND: Currently, a high percentage of patients with congenital heart disease (CHD) reach adulthood. The consequence is that more and more patients will require a heart transplant (HTx) or heart-lung transplant (HLTx). The objective of the study was to analyze the evolution and temporary trend of the number of HTxs and HLTxs in patients with and without CHD. METHODS: We performed a retrospective analysis of all HTxs and HTLxs from a Spanish transplant hospital. Retransplant and other combined transplants were excluded. HTx and HLTx were divided into 2 groups (CHD or non-CHD). The number of procedures of each modality was grouped in 5 years. RESULTS: A total of 930 HTxs were analyzed between 1987 and 2020; 36 were CHD (18 HTxs and 18 HLTxs). HTx and HLTx in CHD showed a growing progressive trend, probably because of the greater number of these patients who reach adulthood and finally develop advanced heart failure. HTx in patients without CHD showed a very high rise in the first decade, reaching the maximum peak around the year 2000, with a poststabilization trend or even progressive reduction in the number of procedures. HLTx in patients without CHD showed a marked ascent during the first decade with a peak around 2005 and subsequent significant decline in recent years practically in disuse, probably because of the possibility of circulatory assistance in the case of right ventricular failure. CONCLUSIONS: The number of HTxs and HLTxs in CHD has a progressive rise. The number of HTx in patients without CHD remains relatively stable. HLTx in patients without CHD shows a marked decrease.

The economic burden of pulmonary arterial hypertension in Spain.

BACKGROUND: Pulmonary Arterial Hypertension (PAH) is a rare, debilitating, and potentially fatal disease. This study aims to quantify the economic burden of PAH in Spain. METHODS: The study was conducted from a societal perspective, including direct and indirect costs associated with incident and prevalent patients. Average annual costs per patient were estimated by multiplying the number of resources consumed by their unit cost, differentiating the functional class (FC) of the patient. Total annual costs per FC were also calculated, taking the 2020 prevalence and incidence ranges into account. An expert committee validated the information on resource consumption and provided primary information on pharmacological consumption. Unit costs were estimated using official tariffs and salaries in Spain. A deterministic sensitivity analysis was conducted to test the uncertainty of the model. RESULTS: The average annual total cost was estimated at €98,839 per prevalent patient (FC I-II: €65,233; FC III: €103,736; FC IV: €208,821), being €42,110 for incident patients (FC I-II: €25,666; FC III: €44,667; FC IV: €95,188). The total annual cost of PAH in Spain, taking into account a prevalence between 16.0 and 25.9 cases per million adult inhabitants (FC I-II 31.8%; FC III 61.3%; FC IV 6.9%) and an incidence of 3.7, was estimated at €67,891,405 to €106,131,626, depending on the prevalence considered. Direct healthcare costs accounted for 64% of the total cost, followed by indirect costs (24%), and direct non-healthcare costs (12%). The total costs associated with patients in FC I-II ranged between €14,161,651 and €22,193,954, while for patients in FC III costs ranged between €43,763,019 and €68,391,651, and for patients in FC IV between €9,966,735 and €15,546,021. In global terms, patients with the worst functional status (FC IV) account for only 6.9% of the adults suffering from PAH in Spain, but are responsible for 14.7% of the total costs. CONCLUSIONS: PAH places a considerable economic burden on patients and their families, the healthcare system, and society as a whole. Efforts must be made to improve the health and management of these patients since the early stages of the disease.

Long-term Outcomes of Adults With Single Ventricle Physiology Not Undergoing Fontan Repair: A Multicentre Experience.

BACKGROUND: To describe long-term survival and cardiovascular events in adult patients with single ventricle physiology (SVP) without Fontan palliation, focusing on predictors of mortality and comparing groups according to their cardiovascular physiology. METHODS: Multicentre observational and retrospective study including adult patients with SVP without Fontan palliation since their first adult clinic visit. The cohort was subdivided into 3 groups: Eisenmenger, restricted pulmonary flow, and aortopulmonary shunt. Death was considered as the main end point. Other clinical outcomes occurring during follow-up were considered as secondary end points. RESULTS: A total of 146 patients, mean age 32.5 ± 11.1 years, were analysed. Over a mean follow-up of 7.3 ± 4.1 years, 33 patients (22.6%) died. Survival was 86% and 74% at 5 and 10 years, respectively. Right ventricular morphology was not associated with higher mortality. Four variables at baseline were related to a higher mortality: at least moderate atrioventricular valve regurgitation, platelet count < 150 × 103/mm3, GFR < 60 mL/min/1.73 m2, and QRS > 120 ms). A total of 34.2% of patients were admitted to the hospital due to heart failure, and 7.5% received a heart transplant. Other cardiovascular outcomes were also frequent: atrial arrhythmias in 19.2%, stroke in 15.1%, and pacemaker/implantable cardioverter-defibrillator in 6.2%/2.7%. CONCLUSIONS: Adult patients with SVP who had not undergone Fontan exhibit a high mortality rate and frequent major cardiovascular events. At least moderate atrioventricular valve regurgitation, thrombocytopenia, renal dysfunction, and QRS duration > 120 ms at baseline visit allow identification of a cohort of patients at higher risk of mortality.

Progresión y predictores de insuficiencia valvular neoaórtica y dilatación de la raíz neoaórtica en adultos jóvenes tras cirugía de switch arterial / Time course and predictors for neoaortic root dilatation and neoaortic valve regurgitation during adult life after arterial switch operation

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±2,4mm/m2, y al final del seguimiento, 20,73±2,8mm/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. (AU)

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 <.001) at the end of follow-up. The mean change over time was 0.14mm/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. (AU)

Time course and predictors for neoaortic root dilatation and neoaortic valve regurgitation during adult life after arterial switch operation.

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 <.001) at the end of follow-up. The mean change over time was 0.14mm/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.

Cardiopatías congénitas del adulto en España: estructura, actividad y características clínicas / Adult congenital heart disease in Spain: health care structure and activity, and clinical characteristics

INTRODUCCIÓN Y OBJETIVOS: Analizar la estructura asistencial y las características clínicas de las cardiopatías congénitas del adulto en España. MÉTODOS: En 2014 se realizó una encuesta entre 32 centros que se clasificaron como nivel 1 o 2 en función de su estructura asistencial. En 2017 se realizó un registro clínico de todos los pacientes asistidos consecutivamente en cada centro durante un periodo de 2 meses. RESULTADOS: Un total de 31 centros (97%) respondieron la encuesta. Se excluyó a 7 por no disponer de consulta especializada. Hasta el año 2005 solo había 5 centros con dedicación específica, pero en 2014 había 10 centros de nivel 1 y 14 de nivel 2 con un total de 19.373 pacientes en seguimiento. La estructura institucional era completa en la mayoría de los centros, pero solo el 33% disponía de enfermería propia y el 29%, de unidad de transición estructurada. La actividad terapéutica específica supuso el 99 y el 91% de la publicada en los registros nacionales de cirugía y cateterismo terapéutico. Del total, el 44% de los pacientes tenían cardiopatía de complejidad moderada y el 24%, de gran complejidad. Aunque el 46% de los pacientes atendidos en centros de nivel 2 tenían cardiopatías simples, el 17% eran cardiopatías de gran complejidad. CONCLUSIONES: La estructura y la actividad de los centros españoles cumplen las recomendaciones internacionales y son comparables a las de otros países desarrollados. El espectro de cardiopatías en seguimiento muestra una concentración de lesiones de complejidad moderada y gran complejidad incluso en centros de nivel 2. Sería aconsejable reordenar el seguimiento de los pacientes en función de las recomendaciones internacionales

INTRODUCTION AND OBJECTIVES: To assess the structure of health care delivery and the clinical characteristics of adults with congenital heart disease (ACHD) attending specialized centers in Spain. METHODS: A survey was conducted among 32 Spanish centers in 2014. The centers were classified into 2 levels based on their resources. In 2017, a clinical dataset was collected of all consecutive patients attended for a 2-month period at these centers. RESULTS: A total of 31 centers (97%) completed the survey. Seven centers without specialized ACHD clinics were excluded from the analysis. In 2005, only 5 centers met the requirements for specific care. In 2014, there were 10 level 1 and 14 level 2 centers, with a total of 19 373 patients under follow-up. Health care structure was complete in most centers but only 33% had ACHD nurse specialists on staff and 29% had structured transition programs. Therapeutic procedures accounted for 99% and 91% of those reported by National Registries of Cardiac Surgery and Cardiac Catheterization, respectively. Among attended patients, 48% had moderately complex lesions and 24% had highly complex lesions. Although 46% of patients attending level 2 centers had simple lesions, 17% had complex lesions. CONCLUSIONS: The structure for ACHD health care delivery in Spain complies with international recommendations and is similar to that of other developed countries. Congenital heart diseases under specialized care consist mostly of moderately and highly complex lesions, even in level 2 centers. It would be desirable to reorganize patient follow-up according to international recommendations in clinical practice

Adult congenital heart disease in Spain: health care structure and activity, and clinical characteristics.

INTRODUCTION AND OBJECTIVES: To assess the structure of health care delivery and the clinical characteristics of adults with congenital heart disease (ACHD) attending specialized centers in Spain. METHODS: A survey was conducted among 32 Spanish centers in 2014. The centers were classified into 2 levels based on their resources. In 2017, a clinical dataset was collected of all consecutive patients attended for a 2-month period at these centers. RESULTS: A total of 31 centers (97%) completed the survey. Seven centers without specialized ACHD clinics were excluded from the analysis. In 2005, only 5 centers met the requirements for specific care. In 2014, there were 10 level 1 and 14 level 2 centers, with a total of 19 373 patients under follow-up. Health care structure was complete in most centers but only 33% had ACHD nurse specialists on staff and 29% had structured transition programs. Therapeutic procedures accounted for 99% and 91% of those reported by National Registries of Cardiac Surgery and Cardiac Catheterization, respectively. Among attended patients, 48% had moderately complex lesions and 24% had highly complex lesions. Although 46% of patients attending level 2 centers had simple lesions, 17% had complex lesions. CONCLUSIONS: The structure for ACHD health care delivery in Spain complies with international recommendations and is similar to that of other developed countries. Congenital heart diseases under specialized care consist mostly of moderately and highly complex lesions, even in level 2 centers. It would be desirable to reorganize patient follow-up according to international recommendations in clinical practice.

Cambios en la expresión de adrenoceptores y GRK en pacientes con insuficiencia pulmonar valvular crónica / Changes in adrenoceptor and GRK expression in patients with chronic pulmonary regurgitation

Introducción y objetivos: La insuficiencia pulmonar (IP) es una complicación frecuente tras la intervención de cardiopatías congénitas. La expresión en leucocitos mononucleares circulantes (LMC) de los adrenoceptores (ß1 y ß2) 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 expresión génica de dichas moléculas en LMC de pacientes con IP grave. Métodos: Estudio prospectivo que analizó la expresión de las moléculas descritas en LMC de pacientes con IP grave en comparación con controles sanos y pacientes con IC avanzada. Resultados: Se estudió a 35 pacientes con IP grave, 22 controles y 13 pacientes con IC. El análisis de comparaciones múltiples mostró que en los controles la cantidad de ARN mensajero de adrenoceptor ß2 era mayor que el que presentaban los pacientes con IP y con IC, con similar expresión 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 expresión génica de GRK2: 760,89 (1.169,46) frente a 445,17 (1.190,69) frente a 284,09 (585,27) (p < 0,001). No hubo diferencias en la expresión de estas moléculas según las variables clínicas de los pacientes con IP. Conclusiones: El patrón de expresión génica de GRK2 y del adrenoceptor ß2 de los pacientes con IP grave, como marcadores moleculares de disfunción cardiaca, se encuentra alterado respecto a los controles y es similar al de los pacientes con IC avanzada

Introduction and objectives: Pulmonary regurgitation (PR) is a frequent complication after repair of congenital heart disease. Lymphocyte expression of adrenoceptors (ß1 and ß2) 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 ß2-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 ß2-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

Changes in Adrenoceptor and GRK Expression in Patients With Chronic Pulmonary Regurgitation.

INTRODUCTION AND OBJECTIVES: Pulmonary regurgitation (PR) is a frequent complication after repair of congenital heart disease. Lymphocyte expression of adrenoceptors (ß1 and ß2) 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 ß2-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 ß2-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.

Fontan Circulation in Adult Patients: Acoustic Radiation Force Impulse Elastography as a Useful Tool for Liver Assessment.

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.

Phenotypic patterns of right ventricular dysfunction: analysis by cardiac magnetic imaging.

The aim of this study was to use magnetic resonance imaging (MRI) to classify the morphological changes and remodeling of the right ventricle (RV) that occur in different clinical situations and that have an impact on RV function. Most literature has traditionally focused on the left ventricle (LV) and as a result, few studies analyze RV behavior and remodeling. The study evaluated all cardiac MRI performed at our center from 2008 to 2010. We retrospectively identified 159 patients who had some sign of right ventricular dysfunction (RVD) based on MRI findings. We classified patients according to a combination of criteria for RVD and the presence of left ventricle dysfunction (LVD). We considered RVD as any of the following abnormalities: i) depressed RV function; ii) RV dilatation; iii) RV hypertrophy. LVD was considered when there was atrial dilatation, LV hypertrophy, LV dilatation and/or depressed LV function. We obtained 6 pathophysiological patterns: RV pressure overload (1.9%), RV volume overload (15.7%), RV volume overload + LVD (32.7%), depressed RV function + LVD (42.1%), mixed RV overload + LVD (6.9%) and other (0.6%). The most frequent etiology was congenital heart disease (33.3%), followed by idiopathic dilated cardiomyopathy (18.2%), left valvular disease (17.6%), ischemic heart disease (15%), pulmonary disease (9.8%), and other (6.1%). This study helps to classify the different patterns that RV can adopt in different clinical situations and can, therefore, help us to understand the RV pathophysiology.

Reversal of protein-losing enteropathy after heart transplantation in young patients.

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.

Resolución de enteropatía pierdeproteínas tras el trasplante cardiaco en pacientes jóvenes / Reversal of protein-losing enteropathy after heart transplantation in young patients

La enteropatía pierdeproteínas es una infrecuente pero muy grave complicación que aparece en determinados pacientes que desarrollan linfangiectasia intestinal secundaria a un incremento de la presión venosa sistémica. Se han descrito diferentes tratamientos, con resultados dispares y en la mayoría de las ocasiones sin respuesta favorable. El objetivo del estudio es mostrar el trasplante cardiaco como una opción terapéutica adecuada para pacientes sin respuesta positiva al tratamiento médico. En nuestro centro se ha trasplantado a 3 pacientes con estas características, con un seguimiento medio de 11 ± 2 meses. Ningún paciente falleció y en todos ellos se documentó una reversibilidad de la enteropatía (AU)

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 3 patients with this condition. The mean follow-up period was 11(2) months. No patient died and the enteropathy regressed in all 3 (AU)

[Prognostic value of fibrinogen in patients admitted with suspected unstable angina and non-q-wave myocardial infarction]. / Valor pronóstico del fibrinógeno en pacientes ingresados con sospecha de angina inestable o infarto de miocardio sin onda Q.

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 (1.6-14; 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-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.

Valor pronóstico del fibrinógeno en pacientes ingresados con sospecha de angina inestable o infarto de miocardio sin onda Q / Prognostic value of fibrinogen in patients admitted with suspected unstable angina and non-Q-wave myocardial infarction

Introducción y objetivo. Durante los últimos años se ha investigado la relación entre los marcadores biológicos de inflamación y el pronóstico en pacientes con síndromes coronarios agudos. Nuestro objetivo ha sido analizar la asociación entre las concentraciones plasmáticas de fibrinógeno y la aparición de episodios clínicos en pacientes ingresados con sospecha de angina inestable o IAM no Q. Material y método. Analizamos el fibrinógeno al ingreso en 325 pacientes consecutivos ingresados con sospecha de síndrome coronario agudo. Los valores de fibrinógeno se dividieron en terciles y se evaluó la incidencia de episodios en cada estrato. Se realizó un seguimiento medio de 15 meses considerando como episodio principal el combinado de muerte y/o IAM. Resultados. Los valores de fibrinógeno fueron significativamente mayores en los pacientes que presentaron IAM, muerte de causa cardíaca o el episodio combinado durante el seguimiento. El porcentaje de episodio combinado fue del 6, el 13 y el 29 por ciento (p < 0,0001) en pacientes estratificados por terciles de fibrinógeno (304, 305-374 y 375 mg/dl). Los predictores multivariados de episodio combinado fueron la edad, la angina previa, el descenso del segmento ST y el fibrinógeno en terciles. Un valor de fibrinógeno en el tercil superior se asoció al episodio con una hazard ratio de 4,8 (IC del 95 por ciento, 1,6-14; p = 0,004). Conclusiones. Los valores elevados de fibrinógeno se asocian con un peor pronóstico a corto y largo plazo en pacientes ingresados con sospecha de angina inestable o IAM no Q. La asociación se mantiene tras ajustar por factores de riesgo clásicos como la edad, la angina previa y el descenso del segmento ST al ingreso. (AU)