Impaired placentation is an important contributing factor to intra-uterine growth restriction and pre-eclampsia in fetuses with congenital heart defects (CHD). These pregnancy complications occur... Show moreImpaired placentation is an important contributing factor to intra-uterine growth restriction and pre-eclampsia in fetuses with congenital heart defects (CHD). These pregnancy complications occur more frequently in pregnancies with fetal CHD. One of the most important factors influencing the life of children with CHD is neurodevelopmental delay, which seems to start already in utero. Delayed neurodevelopment in utero may be correlated or even (partly) explained by impaired placentation in CHD cases. This systematic review provides an overview of published literature on placental development in pregnancies with fetal CHD. A systematic search was performed and the Newcastle-Ottawa scale was used to access data quality. Primary outcomes were placenta size and weight, vascular and villous architecture, immunohistochemistry, angiogenic biomarkers and/or placental gene expression. A total of 1161 articles were reviewed and 21 studies were included. Studies including CHD with a genetic disorder or syndrome and/or multiple pregnancies were excluded. Lower placental weight and elevated rates of abnormal umbilical cord insertions were found in CHD. Cases with CHD more frequently showed microscopic placental abnormalities (i.e. abnormal villous maturation and increased maternal vascular malperfusion lesions), reduced levels of angiogenic biomarkers and increased levels of anti-angiogenic biomarkers in maternal serum and umbilical cord blood. Altered gene expression involved in placental development and fetal growth were found in maternal serum and CHD placentas. In conclusion, abnormal placentation is found in CHD. More extensive studies are needed to elucidate the contribution of impaired placentation to delayed neurodevelopment in CHD cases. Show less
With the studies described in this thesis, we were able to investigate cardiovascular compromise in complicated monochorionic twin pregnancy in great detail.All clinicians caring for monochorionic... Show moreWith the studies described in this thesis, we were able to investigate cardiovascular compromise in complicated monochorionic twin pregnancy in great detail.All clinicians caring for monochorionic twins should perform an echocardiogram at mid‑gestation and should carefully examine both neonates at birth. In case of abnormal perioperative fetal Dopplers in twin-twin transfusion syndrome (TTTS), we should be aware of the increased risk of fetal demise or neurodevelopmental impairment. In all surviving TTTS twins, but also in twin pregnancies with selective fetal growth restriction, cardiac abnormalities should be ruled out by follow-up fetal and neonatal echocardiography. Routine long‑term follow-up should be available to all TTTS twins, since TTTS may also have an impact beyond the perinatal phase.Furthermore, both color‑coded Tissue Doppler Imaging (cTDI) and myocardial performance index (MPI) are potentially valuable techniques which can be used in the risk stratification in monochorionic twins. Show less
Purpose After surgery for congenital heart disease in children and adolescents, an active lifestyle is important to achieve and maintain good physical fitness. This study aimed to describe physical... Show morePurpose After surgery for congenital heart disease in children and adolescents, an active lifestyle is important to achieve and maintain good physical fitness. This study aimed to describe physical activity, as well as the use of patients' and parents' preferences for rehabilitative care after surgery for congenital heart disease. Methods This cross-sectional study included patients aged 4-16 years old, who had undergone surgery for congenital heart disease 2 years prior to the study onset. Patients and/or parents were invited to complete questionnaires on physical activity, and on their preferences for, and -use of, rehabilitative care after surgery. Results Forty-five of the 92 eligible patients and/or parents completed the questionnaires. The median age of the children was 12.2 years (IQR 7.8-16.1). Two children (4%) met the recommendations for taking part in moderate physical activity and 13 (29%) having done so for vigorous physical activity. Postoperatively 15 children (33%) had had physical therapy in primary care. More than 50% of the patients/parents would have preferred to have had more information on physical activity and to have taken part in an individual exercise program delivered during their primary care. Conclusion The majority of eligible patients do not meet public health recommendations for engaging in physical activity and do not use physical therapy after surgery for congenital heart disease. The majority of patients and parents preferred more information as well as individual physical therapy treatments. Show less
Gijtenbeek, M.; Haak, M.C.; Harkel, A.D.J. ten; Bokenkamp, R.; Eyskens, B.; Ortibus, E.; ... ; Lewi, L. 2020
Introduction:Monochorionic twins are at increased risk of congenital heart defects (CHDs). Up to 26% have a birth weight <1,500 g, a CHD requiring neonatal surgery, therefore, poses particular... Show moreIntroduction:Monochorionic twins are at increased risk of congenital heart defects (CHDs). Up to 26% have a birth weight <1,500 g, a CHD requiring neonatal surgery, therefore, poses particular challenges.Objective:The aim of the study was to describe pregnancy characteristics, perinatal management, and outcome of monochorionic twins diagnosed with critical coarctation of the aorta (CoA).Methods:We included monochorionic twins diagnosed with critical CoA (2010-2019) at 2 tertiary referral centers, and we systematically reviewed the literature regarding CoA in monochorionic twins.Results:Seven neonates were included. All were the smaller twin of pregnancies complicated by selective fetal growth restriction. The median gestational age at birth was 32 weeks (28-34). Birth weight of affected twins ranged as 670-1,800 g. One neonate underwent coarctectomy at the age of 1 month (2,330 g). Six underwent stent implantation, performed between day 8 and 40, followed by definitive coarctectomy between 4 and 9 months in 4. All 7 developed normally, except for 1 child with neurodevelopmental delay. Three co-twins had pulmonary stenosis, of whom 1 required balloon valvuloplasty. The literature review revealed 10 cases of CoA, all in the smaller twin. Six cases detected in the first weeks after birth were treated with prostaglandins alone, by repeated transcatheter angioplasty or by surgical repair, with good outcome in 2 out of 6.Conclusions:CoA specifically affects the smaller twin of growth discordant monochorionic twin pairs. Stent implantation is a feasible bridging therapy to surgery in these low birth weight neonates. Show less
Introduction:Invasive fetal cardiac intervention (FCI) for pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (PS) has been performed with small single... Show moreIntroduction:Invasive fetal cardiac intervention (FCI) for pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (PS) has been performed with small single-institution series reporting technical and physiological success. We present the first multicenter experience.Objectives:Describe fetal and maternal characteristics of those being evaluated for FCI, including pregnancy/neonatal outcome data using the International Fetal Cardiac Intervention Registry (IFCIR).Methods:We queried the IFCIR for PAIVS/PS cases evaluated from January 2001 to April 2018 and reviewed maternal/fetal characteristics, procedural details, pregnancy and neonatal outcomes. Data were analyzed using standard descriptive statistics.Results:Of the 84 maternal/fetal dyads in the registry, 58 underwent pulmonary valvuloplasty at a median gestational age of 26.1 (21.9-31.0) weeks. Characteristics of fetuses undergoing FCI varied in terms of tricuspid valve (TV) size, TV regurgitation, and pulmonary valve patency. There were fetal complications in 55% of cases, including 7 deaths and 2 delayed fetal losses. Among those who underwent successful FCI, the absolute measurement of the TV increased by 0.32 (+/- 0.17) mm/week from intervention to birth. Among 60 liveborn with known outcome, there was a higher percentage having a biventricular circulation following successful FCI (87 vs. 43%).Conclusions:Our data suggest a possible benefit to fetal therapy for PAIVS/PS, though rates of technically unsuccessful procedures and procedure-related complications, including fetal loss were substantial. FCI criteria are extremely variable, making direct comparison to nonintervention patients challenging and potentially biased. More uniform FCI criteria for fetuses with PAIVS/PS are needed to avoid unnecessary procedures, expose only fetuses most likely to sustain a benefit, and to enable comparisons to be made with nonintervention patients. Show less
This thesis provides insight in the impact of congenital and acquired heart diseases on electrophysiology and hemodynamics in the heart, that could help understand the often complex... Show moreThis thesis provides insight in the impact of congenital and acquired heart diseases on electrophysiology and hemodynamics in the heart, that could help understand the often complex pathophysiological mechanisms involved in cardiovascular diseases and might aid in the early detection of patients prone to cardiovascular deterioration. The first part of this thesis shows the value of the electrocardiogram and vectorcardiogram in the assessment of patients with right-sided acquired and congenital heart defects. The 3D vectorcardiographic VG and SA could be valuable in the assessment of patients with right ventricular volume or pressure overload. The second part of this thesis shows significant steps towards clinical utility of 4D flow MRI. Valvular flow quantification with 4D flow MRI, especially in combination with automated valve tracking, has great potential as a future clinical standard. The third part of this thesis provides important insights in intraventricular hemodynamics in Fontan patients. In time to come, knowledge on intraventricular hemodynamics could aid in determining follow-up frequency and the ideal timing of initiation of heart failure management, perhaps even on an individual patient’s level. Moreover, these findings can create a platform for further research in the field of intraventricular hemodynamics in healthy subjects and different patient groups. Show less
This thesis provides insights in characteristics of newly introduced echocardiographic parameters in healthy children and their use in follow-up of patients with a congenital heart defect (CHD)... Show moreThis thesis provides insights in characteristics of newly introduced echocardiographic parameters in healthy children and their use in follow-up of patients with a congenital heart defect (CHD) after surgery. In healthy children, reference values and characteristics of two echocardiographic techniques, tissue Doppler imaging (TDI) and speckle-tracking strain imaging, are described. TDI-derived velocity parameters are reproducible and feasible in children. Yet use of age or growth-dependent reference values is recommended, especially in neonates. Speckle-tracking strain-derived global peak strain parameters have a good reproducibility, are age-independent and remain unchanged during periods of significant growth, such as the neonatal period. We encourage use of these parameters. In contrast, the limited reproducibility of parameters describing intraventricular time-differences is worrying. Our follow-up studies in CHD patients undergoing corrective surgery with cardiopulmonary bypass, describe a significant decrease in biventricular performance immediately postoperatively. Subsequently, left ventricular performance recovered to control values. In contrast, right ventricular performance remained impaired in most subgroups of CHD patients up to one year postoperatively. Considering the prognostic value of right ventricular performance parameters, these studies underline the need for careful evaluation of both left and right ventricular performance postoperatively. Furthermore, a longer-lasting negative influence of cardiopulmonary bypass on ventricular performance was suggested. Show less
