Publications by authors named "Roel L F Van der Palen"

Introduction: Transposition of the great arteries (TGA), especially with intact ventricular septum (TGA-IVS), presents unique challenges during fetal-to-neonatal transition, which can contribute to developing persistent pulmonary hypertension of the newborn (PPHN).

Case Presentation: A male newborn with TGA-IVS, delivered via caesarean section, presented with hypoxemia and tachycardia immediately after birth (preductal SpO: 50-60%, post-ductal SpO: 70-75%). Echocardiography revealed a floppy interatrial septum and two interatrial connections with bidirectional shunting.

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Background: Pulmonary artery (PA) stenosis is common after arterial switch operation (ASO) for transposition of the great arteries (TGA). Differences between balloon angioplasty (BA) and stents on right ventricular (RV) and PA pressures are not well studied.

Objectives: The purpose of this study was to analyze percutaneous PA interventions' frequency after ASO, complications, and the effects of BA and stents on RV and PA pressures.

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Background: Frequent premature ventricular contractions (PVCs) in children are usually considered benign. Symptoms and left ventricular dysfunction are indications for treatment with antiarrhythmic drugs.

Objective: This study aimed to evaluate the efficacy of flecainide vs metoprolol in reducing PVCs in children.

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Background: Left ventricular apical pacing (LVAP) is considered to preserve left ventricular (LV) systolic function in both patients with and without congenital heart disease. However, sporadic LVAP-associated cardiac dysfunction in children with complex structural heart disease was recently reported. We present the case of a 2.

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Background: Neoaortic root dilatation (NeoARD) and neoaortic regurgitation (NeoAR) are common sequelae following the arterial switch operation (ASO) for transposition of the great arteries.

Objectives: The authors aimed to estimate the cumulative incidence of NeoAR, assess whether larger neoaortic root dimensions were associated with NeoAR, and evaluate factors associated with the development of NeoAR during long-term follow-up.

Methods: Electronic databases were systematically searched for articles that assessed NeoAR and NeoARD after ASO, published before November 2022.

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Article Synopsis
  • The study assessed the long-term clinical outcomes of patients who underwent an arterial switch operation (ASO) for transposition of the great arteries (TGA), highlighting a growing patient population.
  • Findings indicated that at age 35, the survival rate was high at 93%, but a significant percentage (36%) required re-interventions, particularly affecting the right ventricular outflow tract.
  • The research concluded that while survival rates are favorable, continuous monitoring and potential interventions are necessary throughout the patients' lives, with specific subtypes of TGA presenting higher risks for complications.
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  • High concentrations of oxygen increase the risk of hyperoxemia in infants with persistent pulmonary hypertension (PPHN), leading to the study of its occurrence during treatment with inhaled nitric oxide (iNO).
  • A review of medical records from 181 infants revealed that a significant percentage experienced hyperoxemia, with 82% having at least one arterial oxygen tension (PaO) greater than 13 kPa.
  • The findings indicate that while treating PPHN aims to manage hypoxemia, hyperoxemia is a common, inadvertent side effect during iNO therapy.
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Background: Balloon atrial septostomy (BAS) is an emergent and essential cardiac intervention to enhance intercirculatory mixing at atrial level in deoxygenated patients diagnosed with transposition of the great arteries (TGA) and restrictive foramen ovale. The recent recall of several BAS catheters and the changes in the European legal framework for medical devices (MDR 2017/745), has led to an overall scarcity of BAS catheters and raised questions about the use, safety, and experience of the remaining NuMED Z-5 BAS catheter.

Aims: To evaluate and describe the practice and safety of the Z-5 BAS catheter, and to compare it to the performance of other BAS catheters.

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Background: After the arterial switch operation (ASO) for transposition of the great arteries (TGA), neo-aortic dilatation and coronary arterial anomalies, especially an interarterial course and acute coronary artery take-off angle, are commonly found. Long-term follow-up data after ASO is scarce. Aim of this study was to determine the prevalence of neo-aortic dilatation and coronary abnormalities, with special emphasis on acute coronary take-off angle, in adult TGA-ASO patients.

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  • The study examined the presence of fibrosis and nerve innervation in hearts of individuals with transposition of the great arteries (TGA), utilizing 22 postmortem specimens across different treatment stages.
  • Newborn TGA hearts showed significantly higher levels of interstitial fibrosis compared to healthy hearts, indicating early structural changes due to altered oxygen saturation.
  • Following surgical interventions like Mustard/Senning and arterial switch operations, further increases in fibrosis were observed, particularly in the left ventricle, along with a decrease in nerve innervation, suggesting potential impacts on heart function post-surgery.
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  • The modified Yacoub aortocoronary flap technique offers a solution for coronary transfer during the arterial switch operation, especially in cases of 'non-separable' single sinus coronary arteries with an intramural course.
  • In a study of 516 patients who underwent the arterial switch operation, 14 utilized this technique; hospital mortality was noted in 3 cases due to coronary complications.
  • Long-term follow-up showed that survivors had no significant cardiac issues and stable artery relationships, indicating the technique's effectiveness as a salvage option.
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Background: Coronary anomalies are present in one-third of all patients with transposition of the great arteries (TGA) and have been associated with increased risk of adverse outcomes after the arterial switch operation. Therefore, knowledge about coronary anatomy remains key.

Case Summary: A 5-day-old girl with prenatal diagnosis of Taussig-Bing anomaly (double outlet right ventricle with TGA and large subpulmonary ventricular septal defect) along with aortic arch hypoplasia and coarctation of the aorta underwent the arterial switch operation with closure of the ventricular septal defect and aortic arch repair.

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In patients with critical left ventricular outflow tract obstruction but adequately sized ventricles, the treatment of choice is biventricular repair. Several options have been proposed, including neonatal Yasui or Ross-Konno operation. However, each of these procedures carries a high mortality risk, especially in syndromic neonates.

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Background: A chronic imbalance of the autonomic nervous system(ANS) may contribute to long term complications in different congenital heart diseases. The purpose of this study was to determine whether the ANS plays a role in the long-term outcome of patients with Transposition of great arteries(TGA) after arterial switch operation(ASO) as its contribution is as yet not clear.

Methods: The ANS activity was evaluated non-invasively in 26 TGA patients and 52 age-appropriate healthy subjects combining impedance cardiography and electrocardiography.

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Rationale: Dextro-transposition of the great arteries (D-TGA) is a severe congenital heart defect which affects approximately 1 in 4,000 live births. While there are several reports of D-TGA patients with rare variants in individual genes, the majority of D-TGA cases remain genetically elusive. Familial recurrence patterns and the observation that most cases with D-TGA are sporadic suggest a polygenic inheritance for the disorder, yet this remains unexplored.

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Background: Most patients with congenital heart disease survive into adulthood; however, residual abnormalities remain and management of the patients is life-long and personalized. Patients with surgical repair of transposition of the great arteries, for example, face the risk to develop neoaortic valve regurgitation. Cardiologists update the prognosis of the patient intuitively with updated information of the cardiovascular status of the patient, for instance from echocardiographic imaging.

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We present a case of a prematurely born 2-month-old girl with a double outlet right ventricle with pulmonary atresia and a left pulmonary artery coarctation arising after ductal closure. The case highlights the importance of knowledge and identification of such an anomaly in patients with congenital heart malformations with severe pulmonary stenosis to atresia.

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Multisystem inflammatory syndrome in children is a rare, potentially life-threatening postinfectious complication in children after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. It is currently unknown if multisystem inflammatory syndrome in children (MIS-C) can recur upon reinfection with SARS-CoV-2. Here, we report on a former MIS-C patient who was reinfected with SARS-CoV-2 without recurrence of MIS-C.

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Background: The aim of this retrospective cohort study was to determine the incidence, potential risk factors, characteristics, and outcomes of acute kidney injury in children following the arterial switch operation for transposition of the great arteries.

Methods: Retrospective review of children who underwent ASO between 2000 and 2020 in our tertiary children's hospital in the Netherlands. Pre-and post-ASO serum creatinine levels were collected.

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Objectives: The objective of this study was to assess our 43-year experience with arterial switch operation (ASO) for transposition of the great arteries (TGA) by analysing cardiac outcome measures (hospital and late mortality, reoperations and catheter interventions, significant coronary artery obstruction) and to identify risk factors for reoperation and catheter interventions.

Methods: A total of 490 patients who underwent ASO for TGA from 1977 to 2020 were included in this retrospective, single-centre study. Data on reoperation and catheter intervention of hospital survivors were estimated by the Kaplan-Meier method and compared using a long-rank test.

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Aortic aneurysm is associated with aberrant blood flow and wall shear stress (WSS). This can be studied by coupling magnetic resonance imaging (MRI) with computational fluid dynamics (CFD). For patient-specific simulations, extra attention should be given to the variation in segmentation of the MRI data-set and its effect on WSS.

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After the arterial switch operation (ASO) for transposition of the great arteries (TGA), many patients have an impaired exercise tolerance. Exercise tolerance is determined with cardiopulmonary exercise testing by peak oxygen uptake (VO). Unlike VO, the oxygen uptake efficiency slope (OUES) does not require a maximal effort for interpretation.

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Objectives: Progressive root dilatation is an important complication in patients with transposition of the great arteries (TGA) after arterial switch operation (ASO) that may be caused by altered flow dynamics. Aortic wall shear stress (WSS) distribution at rest and under dobutamine stress (DS) conditions using 4D flow magnetic resonance imaging were investigated in relation to thoracic aorta geometry.

Methods: 4D flow magnetic resonance imaging was performed in 16 adolescent TGA patients after ASO (rest and DS condition) and in 10 healthy controls (rest).

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Background: Hemodynamic aorta parameters can be derived from 4D flow MRI, but this requires lumen segmentation. In both commercially available and research 4D flow MRI software tools, lumen segmentation is mostly (semi-)automatically performed and subsequently manually improved by an observer. Since the segmentation variability, together with 4D flow MRI data and image processing algorithms, will contribute to the reproducibility of patient-specific flow properties, the observer's lumen segmentation reproducibility and repeatability needs to be assessed.

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