The Dutch national paediatric heart transplantation programme: outcomes during a 23-year period.

Background: Since 1998, there has been a national programme for paediatric heart transplantations (HT) in the Netherlands. In this study, we investigated waiting list mortality, survival post-HT, the incidence of common complications, and the patients' functional status during follow-up.

Methods: All children listed for HT from 1998 until October 2020 were included.

Cardiac allograft vasculopathy and donor age affecting permanent pacemaker implantation after heart transplantation.

Aims: The need for permanent pacemakers (PMs) after heart transplantation (HT) is increasing. The aim was to determine the influence of cardiac allograft vasculopathy (CAV), donor age, and other risk factors on PM implantations early and late after HT and its effect on survival.

Methods And Results: A retrospective, single-centre study was performed including HTs from 1984 to July 2018.

Cyanosis due to an isolated atrial septal defect: case report and review of the literature.

Isolated atrial defects usually lead to left-to-right shunt and right ventricular volume load. Descriptions of cyanosis with this congenital heart defect are rare.We describe a rare case of inferior caval vein flow directed through an atrial septal defect in the fossa ovalis leading to severe cyanosis, but without any additional intracardiac anatomical abnormalities.

Expanding the clinical and genetic spectrum of ALPK3 variants: Phenotypes identified in pediatric cardiomyopathy patients and adults with heterozygous variants.

Introduction: Biallelic damaging variants in ALPK3, encoding alpha-protein kinase 3, cause pediatric-onset cardiomyopathy with manifestations that are incompletely defined.

Methods And Results: We analyzed clinical manifestations of damaging biallelic ALPK3 variants in 19 pediatric patients, including nine previously published cases. Among these, 11 loss-of-function (LoF) variants, seven compound LoF and deleterious missense variants, and one homozygous deleterious missense variant were identified.

Biallelic Variants in , Encoding a Cytosolic Targeting Factor of Tail-Anchored Proteins, Cause Rapidly Progressive Pediatric Cardiomyopathy.

Background: Pediatric cardiomyopathies are a clinically and genetically heterogeneous group of heart muscle disorders associated with high morbidity and mortality. Although knowledge of the genetic basis of pediatric cardiomyopathy has improved considerably, the underlying cause remains elusive in a substantial proportion of cases.

Methods: Exome sequencing was used to screen for the causative genetic defect in a pair of siblings with rapidly progressive dilated cardiomyopathy and death in early infancy.

Tracking of structural and functional cardiac measures from infancy into school-age.

Objective Cardiac structure and function are important predictors for cardiovascular disease in adults. Not much is known about tracking of cardiac measures, other than left ventricular mass, from early life onwards. We examined whether and to what extent cardiac measures track from infancy into school-age.

Early Infant Growth Velocity Patterns and Cardiovascular and Metabolic Outcomes in Childhood.

Objective: To evaluate the impact of infant growth on childhood health by examining the associations of detailed longitudinal infant weight velocity patterns with childhood cardiovascular and metabolic outcomes.

Study Design: In a population-based prospective cohort study of 4649 children, we used repeated growth measurements at age 0-3 years to derive peak weight velocity (PWV), age at adiposity peak (AGEAP), and body mass index at adiposity peak (BMIAP). At age 6 years, we measured blood pressure, left ventricular mass, and cholesterol, triglyceride, and insulin concentrations and defined children with clusters of risk factors.

Longitudinal growth during fetal life and infancy and cardiovascular outcomes at school-age.

Objective: Low birth weight is associated with cardiovascular disease. We examined the effects of fetal and infant growth patterns on cardiovascular outcomes in children.

Methods: In a population-based prospective cohort study among 6239 children, we estimated fetal-femur length and weight by 20 and 30 weeks ultrasound, and child length and weight at birth, 0.

Management of children with dilated cardiomyopathy in The Netherlands: Implications of a low early transplantation rate.

Background: The policy for listing and transplant for children with dilated cardiomyopathy (DCM) in The Netherlands has been conservative because of low donor availability. The effects of this policy on outcome are reported.

Methods: This was a multicenter, nationwide study performed in 148 children with DCM.

Third trimester fetal hemodynamics and cardiovascular outcomes in childhood: the Generation R study.

Objective: Low birth weight is associated with cardiovascular disease in adulthood. Hemodynamic adaptations related to fetal growth restriction may underlie these associations, through persistent influences on cardiovascular development. We examined the associations of third trimester fetal hemodynamics with cardiovascular outcomes in childhood.

Parental smoking during pregnancy and cardiovascular structures and function in childhood: the Generation R Study.

Background: Foetal smoke exposure might lead to foetal developmental adaptations that permanently affect the cardiovascular system. We assessed the associations of both maternal and paternal smoking during pregnancy with childhood cardiovascular structures and function.

Method: In a prospective cohort study among 5565 children, we examined whether maternal and paternal smoking during pregnancy are associated with blood pressure, carotid-femoral pulse wave velocity and left cardiac structures and function in 6-year-old children.

Infant feeding patterns are associated with cardiovascular structures and function in childhood.

Nutrition in infancy seems to be associated with cardiovascular disease and its risk factors in adulthood. These associations may be explained by cardiovascular developmental adaptations in childhood in response to specific infant feeding patterns. The aim of this study was to assess whether duration and exclusivity of breastfeeding and timing of introduction of solid foods affect cardiovascular development in childhood.

Maternal educational level and blood pressure, aortic stiffness, cardiovascular structure and functioning in childhood: the generation R study.

Background: In adults, low level of education was shown to be associated with higher blood pressure levels and alterations in cardiac structures and function. It is currently unknown whether socioeconomic inequalities in arterial and cardiac alterations originate in childhood. Therefore, we investigated the association of maternal education with blood pressure levels, arterial stiffness, and cardiac structures and function at the age of 6 years and potential underlying factors.

Parental psychological distress during pregnancy and childhood cardiovascular development. The Generation R Study.

Background: Maternal psychological distress during pregnancy might lead to higher fetal cortisol exposure, which subsequently leads to fetal cardiovascular developmental adaptations and cardiovascular dysfunction in later life.

Aims: We examined whether maternal and paternal psychological distress was associated with the cardiovascular outcome measurements in school age children.

Study Design And Subjects: In a population-based prospective cohort study among 4831 children, we assessed maternal and paternal psychological distress during pregnancy by questionnaire, using the Brief Symptom Inventory (see Fig.

Enlarged right ventricular size at 11 years' follow-up after closure of secundum-type atrial septal defect in children.

Background: The fate of right ventricular dimensions after surgical closure of secundum-type atrial septal defects remains unclear. The objectives of this study were to assess ventricular dimensions, exercise capability, and arrhythmias of patients operated for secundum-type atrial septal defect and compare the results with those in healthy references.

Methods: A total of 78 consecutive patients underwent surgical closure for a secundum-type atrial septal defect between 1990 and 1995.

Growth, obesity, and cardiac structures in early childhood: the Generation R Study.

Cardiac structural adaptations in response to physical growth and obesity in older children have been identified and might have long-term consequences. We examined the associations of growth and obesity with cardiac structures during the first 2 years of life. In a population-based prospective cohort study among 974 children, left atrial diameter, left ventricular diastolic diameter, left ventricular mass, aortic root diameter, and fractional shortening were repeatedly measured by ultrasound at the ages of 1.

Glucocorticoid receptor-9beta polymorphism is associated with systolic blood pressure and heart growth during early childhood. The Generation R Study.

Background: glucocorticoid receptor-9β polymorphism (rs6198) is associated with the susceptibility for cardiovascular disease.

Aim: to examine whether the GR-9 β variant is also associated with blood pressure and heart growth in early childhood.

Study Design: this study was embedded in a population-based prospective cohort study from fetal life onwards.

Breastfeeding is not associated with left cardiac structures and blood pressure during the first two years of life. The Generation R Study.

Background: Shorter duration of breastfeeding in infancy has been suggested to be associated with an increased risk of cardiovascular disease in adulthood. Early cardiovascular adaptations due to breastfeeding may explain these associations.

Aim: To investigate whether breastfeeding affects left cardiac structures and blood pressure development in early childhood.

Reliability of echocardiographic measurements of left cardiac structures in healthy children.

Background: Echocardiographic measurements are widely used as outcomes of different studies. The aim of this study was to assess intraobserver and interobserver reliability of echocardiographic measurements in healthy children.

Materials And Methods: We studied 28 children, with a median age of 7.

Clinical outcome 5 to 18 years after the Fontan operation performed on children younger than 5 years.

Objective: This study assessed clinical condition at midterm follow-up after total cavopulmonary connection for a functionally univentricular heart performed on children younger than 5 years.

Methods: Thirty-four Fontan patients (median age 10.4 years, range 6.

Cardiac structures track during the first 2 years of life and are associated with fetal growth and hemodynamics: the Generation R Study.

Background: The aim of this study is to examine whether cardiac size and function track in early childhood and are associated with fetal and early postnatal growth and blood flow characteristics.

Methods: This study was embedded in a population-based prospective cohort study from fetal life onward. Fetal growth and fetal and placental blood flow parameters in second and third trimester of pregnancy were measured by ultrasound and Doppler.

Outcome after reoperation for atrioventricular septal defect repair.

Results of surgical repair of atrioventricular septal defect (AVSD), both partial (PAVSD) and complete (CAVSD), have improved. However, reoperation is not uncommon. This report describes our experience in 59 patients who underwent reoperation after AVSD repair, between 1977 and 2008.

Pulmonary artery size and function after Fontan operation at a young age.

Purpose: To assess pulmonary artery (PA) size, flow variables, and wall shear stress (WSS) in patients after Fontan operation at a young age.

Materials And Methods: Flow in the branch PA was obtained with phase contrast velocity-encoded cardiovascular magnetic resonance imaging in 14 patients before and after low-dose dobutamine stress (7.5 microg/kg/min) and in 17 healthy controls at rest.

A variant of the IGF-I gene is associated with blood pressure but not with left heart dimensions at the age of 2 years: the Generation R Study.

Background And Objective: A common variant of the IGF-I gene has been shown to be associated with cardiovascular disease in adulthood. The objective of this study was to examine whether this variant of the IGF-I gene is associated with blood pressure and left heart dimensions in early childhood.

Research Design And Methods: This study was embedded in the Generation R Study, a population-based prospective cohort study from foetal life onwards.

Cardiac status after childhood growth hormone treatment of Turner syndrome.

Context: In Turner syndrome (TS), GH treatment is well established. Data on cardiac status after discontinuation of treatment are scarce. This study aimed to assess biventricular size and function in TS at least 6 months after discontinuation of GH treatment.