Short- and long-term survival of children treated with ventricular assist devices in Spain, based on 15 years' experience.

OBJECTIVES: To describe the use of ventricular assist devices (VAD) in children in Spain and to identify variables related to survival. METHODS: This is an observational cohort study of all children younger than 18 years of age who underwent an initial implantation of a VAD at any of the 6 paediatric heart transplant centres from May 2006 to December 2020. Subjects were identified retrospectively from each hospital's database. RESULTS: Paracorporeal VADs were implanted in 118 children [pulsatile (63%), continuous (30.5%) or both types (5.9%)]. Small children (<0.7 m2 of body surface area) comprised the majority of this cohort (63.3%). Overall, 67% survived to VAD explantation, and 64.9% survived to hospital discharge. Non-central nervous system haemorrhage (39%) and stroke (38.1%) were the most common complications. Body weight <5 kg, congenital heart disease, pre-implantation bilirubin >34 µmol/l and bridge to decision strategy were associated with a higher mortality at hospital discharge and in the long-term. Interagency registry for mechanically assisted circulatory support (INTERMACS) status 1 and cardiac arrest prior to VAD implantation were related to long-term mortality, whereas pre-implantation renal replacement therapy and extracorporeal membrane oxygenation were not related to mortality. CONCLUSIONS: In Spain, 67% of the VAD-supported children have been bridged to heart transplantation or to recovery. Body weight lower than 5 kg, congenital heart disease diagnosis, cholestatic liver dysfunction, bridge to decision as VAD strategy, INTERMACS-1 status and cardiac arrest were pre-implantation variables related to mortality, whereas pre-implantation renal replacement therapy and extracorporeal membrane oxygenation were not.

Chylothorax in newborns after cardiac surgery: a rare complication?

The aim of this study was to analyze patients diagnosed with chylothorax after congenital heart disease surgery among a cohort of neonatal patients, comparing the evolution, complications, and prognosis after surgery of patients who were and were not diagnosed with chylothorax, and to analyze possible risk factors that may predict the appearance of chylothorax in this population. Retrospective and observational study included all neonates (less than 30 days since birth) who underwent congenital heart disease surgery in a level III neonatal intensive care department. We included infants born between January 2014 and December 2019. We excluded those infants who were born before 34 weeks of gestational age or whose birth weight was less than 1800 g. We also excluded catheter lab procedures and patent ductus arteriosus closure surgeries. Included patients were divided into two groups depending on whether they were diagnosed with chylothorax or not after surgery, and both groups were compared in terms of perinatal-obstetrical information, surgical data, and NICU course after surgery. We included 149 neonates with congenital heart disease surgery. Thirty-one patients (20.8%) developed chylothorax, and in ten patients (32.3%), it was considered large volume chylothorax. Regarding the evolution of these patients, 22 infants responded to general dietetic measures, a catheter procedure was performed in 9, and 5 of them finally required pleurodesis. Cardiopulmonary bypass, median sternotomy, and delayed sternal closure were the surgical variables associated with higher risks of chylothorax. Patients with chylothorax had a longer duration of inotropic support and mechanical ventilation and took longer to reach full enteral feeds. As complications, they had higher rates of cholestasis, catheter-related sepsis, and venous thrombosis. Although there were no differences in neonatal mortality, patients with chylothorax had a higher rate of mortality after the neonatal period. In a multiple linear regression model, thrombosis and cardiopulmonary bypass multiplied by 10.0 and 5.1, respectively, the risk of chylothorax and have an umbilical vein catheter decreases risk. CONCLUSION: We have found a high incidence of chylothorax after neonatal cardiac surgery, which prolongs the average stay and causes significant morbidity and mortality. We suggested that chylothorax could be an underestimated complication of congenital heart disease surgery during the neonatal period. WHAT IS KNOWN: • Acquired chylothorax in the neonatal period usually appears as a complication of congenital heart disease surgery, being the incidence quite variable among the different patient series (2.5-16.8%). The appearance of chylothorax as a complication of a cardiac surgery increases both mortality and morbidity in these patients, which makes it a quality improvement target in the postsurgical management of this population. WHAT IS NEW: •Most of the published studies include pediatric patients of all ages, from newborns to teenagers, and there is a lack of studies focusing on neonatal populations. The main strength of our study is that it reports, to the best of our knowledge, one of the largest series of neonatal patients receiving surgery for congenital heart disease in the first 30 days after birth. We have found a high incidence of chylothorax after cardiac surgery during the neonatal period compared to other studies. We suggested that chylothorax could be an underestimated complication of congenital heart disease surgery during this period of life.

Experiencia con el armazón vascular bioabsorbible Absorb en varios escenarios de cardiopatías congénitas / Experience with the Absorb bioresorbable vascular scaffold in various scenarios of congenital heart disease

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Palivizumab in the prevention of severe respiratory syncytial virus infection in children with congenital heart disease; a novel cost-utility modeling study reflecting evidence-based clinical pathways in Spain.

BACKGROUND: Respiratory syncytial virus (RSV) infection remains one of the major reasons of re-hospitalization among children with congenital heart disease (CHD). This study estimated the cost-effectiveness of palivizumab prophylaxis versus placebo, in Spain, from the societal perspective, using a novel cost-effectiveness model reflecting evidence-based clinical pathways. METHODS: A decision-analytic model, combining a decision tree structure in the first year and a Markov structure in later years, was constructed to evaluate the benefits and costs associated with palivizumab versus no prophylaxis among children with CHD. In the first year of the model, children were at risk of mild (i.e. medically attended, MA-RSV) and severe (hospitalized, RSV-H) RSV infection. The impact of delayed corrective CHD surgery due to RSV infection and the consequence of performed surgery despite severe infection were considered. In later years, patients were at risk of developing asthma and allergic sensitization as sequelae of RSV infection. Input data for the model were derived from the pivotal clinical trial and systematic literature reviews. Indirect costs included parental absence from work and nosocomial infections. In agreement with Spanish guidelines, costs and effects were discounted at 3%. RESULTS: Over a lifetime horizon, palivizumab prophylaxis yielded 0.11 and 0.07 additional quality-adjusted life years (QALYs) and life years (LYs), respectively, at additional costs of € 1,693, resulting in an ICER of € 15,748 per QALY gained and € 24,936 per LY gained. Probabilistic sensitivity analyses demonstrated that the probability of palivizumab prophylaxis being cost-effective at a € 30,000 per QALY threshold was 92.7%. The ICER remained below this threshold for most extreme scenario analyses. CONCLUSIONS: The model demonstrated that palivizumab prophylaxis results in more QALYs than no prophylaxis in children with CHD. Palivizumab prophylaxis was shown to be a cost-effective health care intervention according to the commonly accepted standards of cost-effectiveness in Spain (ICER below the threshold of € 30,000 per QALY).

CHD and respiratory syncytial virus: global expert exchange recommendations.

BACKGROUND: Palivizumab is the standard immunoprophylaxis against serious disease due to respiratory syncytial virus infection. Current evidence-based prophylaxis guidelines may not address certain children with CHD within specific high-risk groups or clinical/management settings. METHODS: An international steering committee of clinicians with expertise in paediatric heart disease identified key questions concerning palivizumab administration; in collaboration with an additional international expert faculty, evidence-based recommendations were formulated using a quasi-Delphi consensus methodology. RESULTS: Palivizumab prophylaxis was recommended for children with the following conditions: <2 years with unoperated haemodynamically significant CHD, who are cyanotic, who have pulmonary hypertension, or symptomatic airway abnormalities; <1 year with cardiomyopathies requiring treatment; in the 1st year of life with surgically operated CHD with haemodynamically significant residual problems or aged 1-2 years up to 6 months postoperatively; and on heart transplant waiting lists or in their 1st year after heart transplant. Unanimous consensus was not reached for use of immunoprophylaxis in children with asymptomatic CHD and other co-morbid factors such as arrhythmias, Down syndrome, or immunodeficiency, or during a nosocomial outbreak. Challenges to effective immunoprophylaxis included the following: multidisciplinary variations in identifying candidates with CHD and prophylaxis compliance; limited awareness of severe disease risks/burden; and limited knowledge of respiratory syncytial virus seasonal patterns in subtropical/tropical regions. CONCLUSION: Evidence-based immunoprophylaxis recommendations were formulated for subgroups of children with CHD, but more data are needed to guide use in tropical/subtropical countries and in children with certain co-morbidities.

Guía clínica de la enfermedad de Pompe infantil / Clinical guidelines for infantile-onset Pompe disease

La enfermedad de Pompe infantil tiene un pronóstico fatal a corto plazo si no se diagnostica precozmente ni se inicia un tratamiento enzimático sustitutivo lo antes posible. Un grupo de especialistas de las diferentes disciplinas involucradas en esta enfermedad ha revisado la evidencia científica actual y ha elaborado por consenso una serie de recomendaciones para el diagnóstico, el tratamiento y el seguimiento de los pacientes. Se recomienda instaurar tratamiento enzimático en todo paciente con enfermedad de Pompe sintomática de comienzo en el primer año de vida, con diagnóstico clínico y enzimático, y una vez conocido el estado CRIM (material inmunológico con reactividad cruzada) (AU)

Infantile-onset Pompe disease has a fatal prognosis in the short term unless it is diagnosed at an early stage and enzyme replacement therapy is not started as soon as possible. A group of specialists from different disciplines involved in this disease have reviewed the current scientific evidence and have drawn up an agreed series of recommendations on the diagnosis, treatment and follow-up of patients. We recommend establishing enzyme treatment in any patient with symptomatic Pompe disease with onset within the first year of life, with a clinical and enzymatic diagnosis, and once the CRIM (cross-reactive immunological material) status is known (AU)

Cardiología fetal, la frontera de la medicina cardiovascular / Fetal cardiology, the frontier of pediatric cardiovascular medicine

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Community-acquired respiratory infections in young children with congenital heart diseases in the palivizumab era: the Spanish 4-season civic epidemiologic study.

INTRODUCTION: To investigate the epidemiology of acute respiratory tract infections (ARIs) in children younger than 24 months old with hemodynamically significant congenital heart diseases. Primary aim: incidence of hospital admission due to ARI. Secondary aims: risk factors, etiologic agents, clinical outcomes, and usefulness of preventive measures. PATIENTS AND METHODS: Prospective, multicenter, epidemiologic study conducted in 57 Spanish hospitals covering four 7-month seasons (2004-2008). RESULTS: A total of 2613 patients were eligible for the study. Three hundred fifty-four patients (13.5%) (95% confidence interval: 12.3-14.9) required a total of 453 hospital admissions. Clinical diagnoses: bronchiolitis (54.1%), upper respiratory tract infection (21%), pneumonia (19.9%), and others (17.4%). Median length of hospital stay: 7.0 days. No etiologic agent was identified in two-thirds of the patients. In the remaining patients either a single agent (26.8%) or polymicrobial infection (5%) was identified. Respiratory syncytial virus (RSV) was the agent that was most commonly found (3.8% specific hospitalization rate). Children receiving adequate RSV prophylaxis (90.5%) had a 58.2% (95% confidence interval: 37.6-78.3) reduction in RSV hospitalization. Risk factors for admission included malnourishment, infant age, male gender, chromosome alterations, wheezing, inadequate RSV prophylaxis fulfillment, and siblings <11 years of age. Pediatric intensive care unit care was required in 21.8% of the admissions and 9 patients (0.34%) died. CONCLUSIONS: Hospital admission rate and severity of ARI remain as important issues in hemodynamically significant congenital heart disease patients. The strict fulfillment of prophylactic recommendations against RSV is the only protective factor that can be modulated to decrease the ARI hospital admission rate.

[Update on pediatric cardiology and congenital heart disease]. / Actualización en cardiología pediátrica y cardiopatías congénitas.

The fields of pediatric cardiology and congenital heart disease have experienced considerable progress in the last few years, with advances in new diagnostic and therapeutic techniques that can be applied at all stages of life from the fetus to the adult. This article reviews scientific publications in a number of areas that appeared between August 2007 and September 2008. In developed countries, congenital heart disease is becoming increasingly prevalent in nonpediatric patients, including pregnant women. Actions aimed at preventing coronary heart disease must be started early in infancy and should involve the promotion of a healthy diet and lifestyle. Recent developments in echocardiography include the introduction of three-dimensional echocardiography and of new techniques such as two-dimensional speckle tracking imaging, which can be used for both anatomical and functional investigations in patients with complex heart disease, including a univentricular heart. Progress has also occurred in fetal cardiology, with new data on prognosis and prognostic factors and developments in intrauterine interventions, though indications for these interventions have still to be established. Heart transplantation has become a routine procedure, supplemented in some cases by circulatory support devices. In catheter interventions, new devices have become available for the closure of atrial or ventricular septal defects and patent ductus arteriosus as well as for percutaneous pulmonary valve implantation. Surgery is also advancing, in some cases with hybrid techniques, particularly for the treatment of hypoplastic left heart syndrome. The article ends with a review of publications on cardiomyopathy, myocarditis and the treatment of bacterial endocarditis.

Absence of cardiovascular effects on young adults exposed in utero to ritodrine: a long-term follow-up.

OBJECTIVE: To determine, using echocardiographs, if young adults exposed in uterus to Ritodrine have long-term cardiovascular effects. STUDY DESIGN: Retrospective follow-up study. Young adults between the ages of 22- and 26-years-old. Young adults (n=19) exposed in uterus to Ritodrine and age-matched controls (n=27) were studied by means of transthoracic echocardiography. All participants were born following full-term pregnancies. Subgroups of those subjects exposed to the highest dose of Ritodrine and for the longest period of time were established. A multivariate descriptive statistical analysis of the values obtained using transthoracic echocardiography was performed. Echocardiography measurements in M-mode included intraventricular septum and posterior wall thickness, systolic and diastolic diameter of the left ventricle, septum/wall quotient, left ventricle mass and short and ejection fractions. Doppler pressed: E and A peaks in the mitral valve and isovolumetric relaxation time were determined. RESULTS: The mass, posterior wall, systolic diameter and the diastolic diameter of the left ventricle were slightly, but not significantly, higher in the Ritodrine group. The E/A peak and the isovolumetric relaxation time were similar in both groups. A negative correlation between the left ventricle mass index and the total Ritodrine dose (r=-0.67; p=0.051) was found; this disappeared in the subgroups exposed to higher doses and for longer times. CONCLUSIONS: The variables studied using echocardiography showed that in uterus exposure to Ritodrine do not have long-term effects on cardiac functionality in young adults.

Actualización en cardiología pediátrica y cardiopatías congénitas / Update on pediatric cardiology and congenital heart disease

En el campo de la cardiología pediátrica y las cardiopatías congénitas, hemos progresado en los últimos años con avances en nuevas técnicas diagnósticas y terapias aplicadas del feto al adulto. Revisamos la literatura relacionada con diversos campos desde agosto de2007 a septiembre de 2008. Las cardiopatías congénitas después de la edad pediátrica cada vez son más prevalentes en los países desarrollados, incluso en gestantes con estos trastornos. La prevención de la enfermedad coronaria debe iniciarse precozmente desde la infancia, y ha de incluir la promoción de una dieta sana y un estilo de vida saludable. Entre los avances en ecocardiografía están la aplicación de la ecocardiografía tridimensional y las nuevas técnicas como la 2D speckle tracking imagen para realizar estudios tanto anatómicos como funcionales en cardiopatías complejas como los corazones un ventriculares. La cardiología fetal ha progresado con nuevos datos y factores pronósticos y en las intervenciones intrauterinas, aunque las indicaciones deben ser consolidadas. El trasplante cardiaco se ha convertido en una terapia habitual, asociada en algunos casos a dispositivos de soporte circulatorio. En intervencionismo se dispone de nuevos dispositivos para cierre de comunicaciones interauriculares, interventriculares y ductus, así como válvulas pulmonares de implantación percutánea. La cirugía está avanzando, en algunos casos combinada con técnicas híbridas, sobre todo en el manejo de la hipoplasia de cavidades izquierdas. Finalmente, revisamos las miocardiopatías, la miocarditis y la profilaxis de la endocarditis bacteriana (AU)

The fields of pediatric cardiology and congenital heart disease have experienced considerable progress in the last few years, with advances in new diagnostic and therapeutic techniques that can be applied at all stages of life from the fetus to the adult. This article reviews scientific publications in a number of areas that appeared between August 2007 and September 2008. In developed countries, congenital heart disease is becoming increasingly prevalent in no pediatric patients, including pregnant women. Actions aimed at preventing coronary heart disease must be started early in infancy and should involve the promotion of a healthy diet and lifestyle. Recent developments in echocardiography include the introduction of three-dimensional echocardiography and of new techniques such as two-dimensional speckle tracking imaging, which can be used for both anatomical and functional investigations in patients with complex heart disease, including a univentricular heart. Progress has also occurred in fetal cardiology, with new data on prognosis and prognostic factors and developments in intrauterine interventions, though indications for these interventions have still to be established. Heart transplantation has become a routine procedure, supplemented in some cases by circulatory support devices. In catheter interventions, new devices have become available for the closure of atrial or ventricular septal defects and patent ductus arteriosus as well as for percutaneous pulmonary valve implantation. Surgery is also advancing, in some cases with hybrid techniques, particularly for the treatment of hypoplastic left heart syndrome. The article ends with are view of publications on cardiomyopathy, myocarditis and the treatment of bacterial endocarditis (AU)

[Treatment with sildenafil, bosentan, or both in children and young people with idiopathic pulmonary arterial hypertension and Eisenmenger's syndrome]. / Tratamiento con sildenafilo y/o bosentán en niños y jóvenes con hipertensión arterial pulmonar idiopática y síndrome de Eisenmenger.

INTRODUCTION AND OBJECTIVES: Pulmonary arterial hypertension carries a poor prognosis in both adult and pediatric patients. Current understanding of the mechanisms underlying pulmonary arterial hypertension has enabled the rapid development of appropriate drugs, such as endothelin receptor antagonists and 5-phosphodieste-rase inhibitors, that can be administered orally and which are generally well tolerated. The aims of the present study were to evaluate functional class and exercise capacity following long-term treatment with sildenafil or bosentan in patients with idiopathic pulmonary arterial hypertension and Eisenmenger's syndrome and to compare results in the two groups. METHODS: Seven patients were included in the pulmonary arterial hypertension study, and diagnoses of idiopathic pulmonary arterial hypertension were confirmed. Five patients were treated with sildenafil, while two received bosentan. The five patients with a non-restrictive ventricular septal defect and pulmonary arterial hypertension were treated with sildenafil. In one patient, bosentan was added to the sildenafil. RESULTS: Both sildenafil and bosentan significantly improved exercise capacity in patients with idiopathic pulmonary arterial hypertension. The treatment effect was less in those with Eisenmenger physiology. Although the improvement in World Health Organization functional class was greater in patients with idiopathic pulmonary arterial hypertension, it was significant in both groups. CONCLUSIONS: Long-term treatment with sildenafil and bosentan improved both exercise capacity and functional class in patients with idiopathic pulmonary arterial hypertension and in those with hypertension due to congenital heart disease. The changes were more marked in patients with idiopathic pulmonary arterial hypertension.

Tratamiento con sildenafilo y/o bosentán en niños y jóvenes con hipertensión arterial pulmonar idiopática y síndrome de Eisenmenger / Treatment with sildenafil, bosentan, or both in children and young people with idiopathic pulmonary arterial hypertension and Eisenmenger's Syndrome

Introducción y objetivos. La hipertensión arterial pulmonar tiene mal pronóstico en pacientes adultos y pediátricos. El conocimiento actual de los mecanismos que condicionan la hipertensión arterial pulmonar ha permitido descubrir medicamentos como los antagonistas de los receptores de la endotelina e inhibidores de la 5-fosfodiesterasa, administrables ambos por vía oral y generalmente bien tolerados. En este estudio se valora la capacidad funcional y la tolerancia al ejercicio en el tratamiento a largo plazo con sildenafilo o bosentán en pacientes con hipertensión arterial pulmonar idiopática y síndrome de Eisenmenger, y se comparan los resultados en ambos grupos de pacientes. Métodos. Siete pacientes fueron incluidos en el protocolo de estudio de la hipertensión arterial pulmonar, y en ellos se confirmó el diagnóstico de hipertensión arterial pulmonar idiopática. Se trató con sildenafilo a 5 pacientes y con bosentán a 2. Cinco pacientes con comunicación interventricular no restrictiva e hipertensión arterial pulmonar fueron tratados con sildenafilo. En uno de ellos se añadió bosentán al tratamiento con sildenafilo. Resultados. El sildenafilo y el bosentán mejoraron significativamente la capacidad de ejercicio en pacientes con hipertensión arterial pulmonar idiopática. El efecto fue menor en los pacientes con fisiología de síndrome de Eisenmenger. La mejoría en la clase funcional de la Organización Mundial de la Salud fue mayor en los pacientes con hipertensión arterial pulmonar idiopática, aunque fue significativa en ambos grupos. Conclusiones. El tratamiento a largo plazo con sildenafilo y bosentán mejora la capacidad de ejercicio y la clase funcional en la hipertensión arterial pulmonar idiopática y debida a cardiopatías congénitas. Los cambios son más llamativos en los pacientes con hipertensión arterial pulmonar idiopática (AU)

Introduction and objectives. Pulmonary arterial hypertension carries a poor prognosis in both adult and pediatric patients. Current understanding of the mechanisms underlying pulmonary arterial hypertension has enabled the rapid development of appropriate drugs, such as endothelin receptor antagonists and 5-phosphodieste-rase inhibitors, that can be administered orally and which are generally well tolerated. The aims of the present study were to evaluate functional class and exercise capacity following long-term treatment with sildenafil or bosentan in patients with idiopathic pulmonary arterial hypertension and Eisenmenger's syndrome and to compare results in the two groups. Methods. Seven patients were included in the pulmonary arterial hypertension study, and diagnoses of idiopathic pulmonary arterial hypertension were confirmed. Five patients were treated with sildenafil, while two received bosentan. The five patients with a non-restrictive ventricular septal defect and pulmonary arterial hypertension were treated with sildenafil. In one patient, bosentan was added to the sildenafil. Results. Both sildenafil and bosentan significantly improved exercise capacity in patients with idiopathic pulmonary arterial hypertension. The treatment effect was less in those with Eisenmenger physiology. Although the improvement in World Health Organization functional class was greater in patients with idiopathic pulmonary arterial hypertension, it was significant in both groups. Conclusions. Long-term treatment with sildenafil and bosentan improved both exercise capacity and functional class in patients with idiopathic pulmonary arterial hypertension and in those with hypertension due to congenital heart disease. The changes were more marked in patients with idiopathic pulmonary arterial hypertension (AU)

[Management by hypothermia of junctional ectopic tachycardia appearing after pediatric heart surgery]. / Tratamiento mediante hipotermia de la taquicardia ectópica de una unión tras cirugía cardíaca infantil.

Junctional ectopic tachycardia (JET) is a complication of the repair of congenital cardiac malformations that responds poorly to conventional treatment. We report our experience with the use of moderate hypothermia in its management. Twelve infants with postoperative JET treated with hypothermia were reviewed. The mean interval between the diagnosis of JET and initiation of hypothermia was 1.5 0.5 hours. In the first 24 hours of hypothermia, central temperature and heart rate decreased significantly. Arterial pressure and diuresis tended to increase and central venous pressure tended to decrease. No direct adverse events occurred. All the patients but one survived and are alive and free of neurological deficits after 15 12 months.

Tratamiento mediante hipotermia de la taquicardia ectópica de la unión tras cirugía cardíaca infantil / Management by hypothermia of junctional ectopic tachycardia appearing after pediatric heart surgery

La taquicardia ectópica de la unión (TEU) es una complicación que aparece tras las reparaciones de cardiopatías congénitas, con una escasa respuesta al tratamiento convencional. Describimos nuestra experiencia en el uso de hipotermia moderada como parte de su tratamiento. Se revisaron las historias de 12 pacientes con TEU postoperatoria tratados con hipotermia. El intervalo entre el diagnóstico de TEU y el inicio de la hipotermia fue de 1,5 ñ 0,5 h. Durante las primeras 24 h hubo un descenso significativo de la temperatura y la frecuencia cardíaca, con tendencia al aumento de la presión arterial y de la diuresis y al descenso de la presión venosa central. No hubo complicaciones relacionadas con la hipotermia. Todos excepto un paciente sobrevivieron y tras un seguimiento de 15 ñ 12 meses permanecen vivos y sin secuelas neurológicas (AU)