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

29 Publications

  • Page 1 of 1

Pulmonary ductal coarctation: An entity associated with congenital heart defects involving the right ventricle outflow tract.

J Card Surg 2021 Oct 12. Epub 2021 Oct 12.

Division of Pediatric Cardiology, Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands.

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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http://dx.doi.org/10.1111/jocs.16067DOI Listing
October 2021

Reinfection With Severe Acute Respiratory Syndrome Coronavirus 2 Without Recurrence of Multisystem Inflammatory Syndrome in Children.

Pediatr Infect Dis J 2021 Aug 10. Epub 2021 Aug 10.

From the Department of Pediatrics, Division of Pediatric Immunology and Infectiology, Willem-Alexander Children's Hospital Department of Medical Microbiology Cardio Vascular Imaging Group (CVIG), Department of Radiology Department of Pediatrics, Division of Pediatric Cardiology, Willem-Alexander Children's Hospital Department of Pediatrics, Division of Pediatric Rheumatology, Willem-Alexander Children's Hospital, Leiden University Medical Center, Leiden, the Netherlands.

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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http://dx.doi.org/10.1097/INF.0000000000003280DOI Listing
August 2021

Acute kidney injury after the arterial switch operation: incidence, risk factors, and outcomes.

Cardiol Young 2021 Aug 5:1-6. Epub 2021 Aug 5.

Department of Pediatric intensive care, Leiden University Medical Centre LUMC, Leiden, the Netherlands.

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. Severe AKI was defined as 100% serum creatinine rise or estimated creatinine clearance <35 ml/min/1.73 m2 according to pRIFLE criteria. Logistic regression was used to adjust for confounders.

Results: A total of 242 children were included. Fifty-seven (24%) children developed severe AKI after ASO. Four patients with severe AKI were treated with renal replacement therapy. Children with severe AKI had a longer duration of mechanical ventilation 4.5 (1.0-29) versus 3 (1.0-12) days (p = 0.001), longer PICU stay 7 (2-76) versus 5 (1-70) days, (p = 0.001), higher rate of myocardial infarction 5% versus 0.5% (p = 0.001), sepsis 24% versus 9% (p = 0.002), post-operative pulmonary hypertension 19% versus 6% (p = 0.002), post-operative bleeding 9% versus 3% (p = 0.044), longer time to sternal closure 3 (1-19) versus 2 (1-6) days, (p = 0.009), and a higher mortality rate 9.0% versus 0.5% (p = 0.001) compared to children without severe AKI. Sepsis was a risk factor for developing severe AKI.

Conclusions: In this single-centre cohort, 24% of our patients developed severe AKI after ASO, which is associated with increased morbidity, longer PICU stay, and higher mortality.
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http://dx.doi.org/10.1017/S1047951121003176DOI Listing
August 2021

Long-term outcome after the arterial switch operation: 43 years of experience.

Eur J Cardiothorac Surg 2021 05;59(5):968-977

Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, Netherlands.

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. Risk factors for reoperation and/or catheter intervention were assessed by multivariate Cox regression analysis.

Results: Hospital mortality occurred in 43 patients (8.8%), late death in 12 patients (2.9%) and 43 patients were lost to follow-up. Median follow-up time of 413 hospital survivors was 15.6 (interquartile range 7.0-22.4) years. Reoperations were performed in 83 patients (117 reoperations). Neoaortic valve regurgitation with root dilatation was the second most common indication for reoperation (15/83 patients, 18.1%) after right ventricular outflow tract obstruction (50/83 patients, 60.2%). Risk factors for any reoperation on multivariable analysis were: TGA morphological subtype [TGA with ventricular septal defect: hazard ratio (HR) = 1.99, 95% confidence interval (CI) 1.18-3.36; P = 0.010 and Taussig-Bing: HR = 2.17, 95% CI 1.02-4.64; P = 0.045], aortic arch repair associated with ASO (HR = 3.03, 95% CI 1.62-5.69; P = 0.001) and a non-usual coronary artery anatomy (HR = 2.41, 95% CI 1.45-4.00; P = 0.001). One hundred and one catheter interventions were performed in 54 patients, usually for relief of supravalvular pulmonary stenosis (44/54 patients, 81.5%) or arch obstruction (10/54 patients, 18.5%). Main risk factor for catheter intervention on multivariable analysis was aortic arch repair associated with ASO (HR = 2.95, 95% CI 1.37-6.36; P = 0.006). Significant coronary artery stenosis was relatively uncommon (9/413 patients, 2.2%) but may be underrepresented.

Conclusions: Patients after ASO typically have good long-term clinical outcomes but reoperations and interventions remain necessary in some patients. Neoaortic valve regurgitation with root dilatation is the second most common indication for reoperation after right ventricular outflow tract obstruction and an increasing need for neoaortic valve and root redo surgery in future is to be expected.
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http://dx.doi.org/10.1093/ejcts/ezab006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8106945PMC
May 2021

Geometrically induced wall shear stress variability in CFD-MRI coupled simulations of blood flow in the thoracic aortas.

Comput Biol Med 2021 06 20;133:104385. Epub 2021 Apr 20.

Department of Chemical Engineering, Faculty of Applied Sciences, Delft University of Technology and J.M. Burgerscentrum Research School for Fluid Mechanics, Delft, the Netherlands. Electronic address:

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. We performed CFD simulations of blood flow in the aorta for ten different volunteers and provided corresponding WSS distributions. The aorta of each volunteer was segmented four times. The same inlet and outlet boundary conditions were applied for all segmentation variations of each volunteer. Steady-state CFD simulations were performed with inlet flow based on phase-contrast MRI during peak systole. We show that the commonly used comparison of mean and maximal values of WSS, based on CFD in the different segments of the thoracic aorta, yields good to excellent correlation (0.78-0.95) for rescan and moderate to excellent correlation (0.64-1.00) for intra- and interobserver reproducibility. However, the effect of geometrical variations is higher for the voxel-to-voxel comparison of WSS. With this analysis method, the correlation for different segments of the whole aorta is poor to moderate (0.43-0.66) for rescan and poor to good (0.48-0.73) for intra- and interobserver reproducibility. Therefore, we advise being critical about the CFD results based on the MRI segmentations to avoid possible misinterpretation. While the global values of WSS are similar for different modalities, the variation of results is high when considering the local distributions.
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http://dx.doi.org/10.1016/j.compbiomed.2021.104385DOI Listing
June 2021

Oxygen Uptake Efficiency Slope is Strongly Correlated to VO Long-Term After Arterial Switch Operation.

Pediatr Cardiol 2021 Apr 1;42(4):866-874. Epub 2021 Feb 1.

Division of Paediatric Cardiology, Department of Paediatrics, Leiden University Medical Center, Leiden, The Netherlands.

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. The value of OUES has not been assessed in a large group of patients after ASO. The purpose of this study was to determine OUES and VO, evaluate its interrelationship and assess whether exercise tolerance is related to ventricular function after ASO. A cardiopulmonary exercise testing, assessment of physical activity score and transthoracic echocardiography (fractional shortening and left/right ventricular global longitudinal peak strain) were performed to 48 patients after ASO. Median age at follow-up after ASO was 16.0 (IQR 13.0-18.0) years. Shortening fraction was normal (36 ± 6%). Left and right global longitudinal peak strain were reduced: 15.1 ± 2.4% and 19.5 ± 4.5%. This group of patients showed lower values for all cardiopulmonary exercise testing parameters compared to the reference values: mean VO% 75% (95% CI 72-77) and mean OUES% 82(95% CI 77-87); without significant differences between subtypes of TGA. A strong-to-excellent correlation between the VO and OUES was found (absolute values: R = 0.90, p < 0.001; normalized values: R = 0.79, p < 0.001). No correlation was found between cardiopulmonary exercise testing results and left ventricle function parameters. In conclusion, OUES and VO were lower in patients after ASO compared to reference values but are strongly correlated, making OUES a valuable tool to use in this patient group when maximal effort is not achievable.
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http://dx.doi.org/10.1007/s00246-021-02554-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8110507PMC
April 2021

Wall shear stress in the thoracic aorta at rest and with dobutamine stress after arterial switch operation.

Eur J Cardiothorac Surg 2021 04;59(4):814-822

Department of Radiology, Leiden University Medical Center, Leiden, Netherlands.

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). The primary outcome measure was the WSS distribution along the aortic segments and the WSS change with DS in TGA patients. Based on the results, we secondary zoomed in on factors [aortic geometry and left ventricular (LV) function parameters] that might relate to these WSS distribution differences. Aortic diameters, arch angle, LV function parameters (stroke volume, LV ejection fraction, cardiac output) and peak systolic aortic WSS were obtained.

Results: TGA patients had significantly larger neoaortic root and smaller mid-ascending aorta (AAo) dimensions and aortic arch angle. At rest, patients had significantly higher WSS in the entire thoracic aorta, except for the dilated root. High WSS levels beyond the proximal AAo were associated with the diameter decrease from the root to the mid-AAo (correlation coefficient r = 0.54-0.59, P = 0.022-0.031), not associated with the aortic arch angle. During DS, WSS increased in all aortic segments (P < 0.001), most pronounced in the AAo segments. The increase in LV ejection fraction, stroke volume and cardiac output as a result of DS showed a moderate linear relationship with the WSS increase in the distal AAo (correlation coefficient r = 0.54-0.57, P = 0.002-0.038).

Conclusions: Increased aortic WSS was observed in TGA patients after ASO, related to the ASO-specific geometry, which increased with DS. Stress-enhanced elevated WSS may play a role in neoaortic root dilatation and anterior aortic wall thinning of the distal AAo.
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http://dx.doi.org/10.1093/ejcts/ezaa392DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8083947PMC
April 2021

Reproducibility of Aorta Segmentation on 4D Flow MRI in Healthy Volunteers.

J Magn Reson Imaging 2021 04 11;53(4):1268-1279. Epub 2020 Nov 11.

Department of Radiology, Leiden University Medical Center, Leiden, The Netherlands.

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.

Purpose: To determine the interexamination, interobserver reproducibility, and intraobserver repeatability of aortic lumen segmentation on 4D flow MRI.

Study Type: Prospective and retrospective.

Population: A healthy volunteer cohort of 10 subjects who underwent 4D flow MRI twice. Also, a clinical cohort of six subjects who underwent 4D flow MRI once.

Field Strength/sequence: 3T; time-resolved three-directional and 3D velocity-encoded sequence (4D flow MRI).

Assessment: The thoracic aorta was segmented on the 4D flow MRI in five systolic phases. By positioning six planes perpendicular to a segmentation's centerline, the aorta was divided into five segments. The volume, surface area, centerline length, maximal diameter, and curvature radius were determined for each segment.

Statistical Tests: To assess the reproducibility, the coefficient of variation (COV), Pearson correlation coefficient (r), and intraclass correlation coefficient (ICC) were calculated.

Results: The interexamination and interobserver reproducibility and intraobserver repeatability were comparable for each parameter. For both cohorts there was very good reproducibility and repeatability for volume, surface area, and centerline length (COV = 10-32%, r = 0.54-0.95 and ICC = 0.65-0.99), excellent reproducibility and repeatability for maximal diameter (COV = 3-11%, r = 0.94-0.99, ICC = 0.94-0.99), and good reproducibility and repeatability for curvature radius (COV = 25-62%, r = 0.73-0.95, ICC = 0.84-0.97).

Data Conclusion: This study demonstrated no major reproducibility and repeatability limitations for 4D flow MRI aortic lumen segmentation.

Level Of Evidence: 3 TECHNICAL EFFICACY STAGE: 2.
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http://dx.doi.org/10.1002/jmri.27431DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7984392PMC
April 2021

Imaging large arteries after arterial switch operation.

Heart 2020 06;106(12):891-950

Division of Pediatric Cardiology, Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands

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http://dx.doi.org/10.1136/heartjnl-2019-316504DOI Listing
June 2020

Altered Ascending Aorta Hemodynamics in Patients After Arterial Switch Operation for Transposition of the Great Arteries.

J Magn Reson Imaging 2020 04 7;51(4):1105-1116. Epub 2019 Oct 7.

Division of Pediatric Cardiology, Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands.

Background: Patients with transposition of the great arteries (TGA) have an altered aortic geometry after an arterial switch operation (ASO), with neo-aortic root dilatation as an important complication. Geometry-related aortic hemodynamics have been assumed to contribute to pathology of the ascending aorta (AAo).

Purpose: To evaluate aortic flow displacement (FD) and regional wall shear stress (WSS) in relation to ascending neo-aortic geometry in children after ASO.

Study Type: Prospective.

Population: Twenty-eight TGA patients after ASO and 10 healthy volunteers.

Field Strength/sequence: 3.0T/4D flow (segmented fast-spoiled echo pulse), noncontrast-enhanced MR angiography (Dixon), and anatomic images (SSFP).

Assessment: Aortic diameters and body surface area-indexed aortic dimensions (Z-scores), normalized FD and planar ascending aortic WSS.

Statistical Tests: Mann-Whitney and chi-square tests for differences in FD magnitude, WSS, and FD directionality between groups, respectively. Spearman rank correlation to assess the degree of association between aortic geometry, FD and WSS parameters. Shapiro-Wilk test to evaluate distribution normality on the absolute differences in octant location between FD and WSS.

Results: TGA patients showed a significantly dilated proximal AAo and relatively small mid-AAo dimensions at the level of the pulmonary arteries (Z-scores neo-aortic root: 4.38 ± 1.96 vs. 1.52 ± 0.70, P < 0.001; sinotubular junction: 3.48 ± 2.67 vs. 1.38 ± 1.30, P = 0.010; mid-AAo: 0.32 ± 3.06 vs. 1.69 ± 1.24, P = 0.001). FD magnitude was higher in TGA patients (neo-aortic root: 0.048 ± 0.027 vs. 0.021 ± 0.006, P < 0.001; sinotubular junction: 0.054 ± 0.037 vs. 0.029 ± 0.013, P < 0.05) and was related to the neo-aortic Z-score. Clear areas of higher WSS at the right and anterior aortic wall regions along the distal AAo were detected in TGA patients, most pronounced in those with relatively smaller mid-AAo diameters.

Data Conclusion: TGA-specific geometry related to the ASO, evidenced by neo-aortic root dilatation and a sudden change in vessel diameter at mid-AAo level, leads to more aortic flow asymmetry in the proximal AAo and WSS distribution with higher WSS at the right and anterior aortic wall regions along the distal AAo.

Level Of Evidence: 1 Technical Efficacy Stage: 3 J. Magn. Reson. Imaging 2020;51:1105-1116.
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http://dx.doi.org/10.1002/jmri.26934DOI Listing
April 2020

Progression of aortic root dilatation and aortic valve regurgitation after the arterial switch operation.

Heart 2019 11 10;105(22):1732-1740. Epub 2019 Jul 10.

Division of Pediatric Cardiology, Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands.

Objective: To study neo-aortic growth and the evolution of neo-aortic valve regurgitation (AR) in patients with transposition of the great arteries (TGA) after arterial switch operation (ASO) from newborn to adulthood and to identify patients at risk.

Methods: Neo-aortic dimensions (annulus/root/sinotubular junction) and neo-aortic valve regurgitation were assessed serially in 345 patients with TGA who underwent ASO between 1977 and 2015. Linear mixed-effect models were used to assess increase of neo-aortic dimensions over time and to identify risk factors for dilatation. Risk factor analysis for AR by using time-dependent Cox regression models.

Results: After a rapid increase in the first year after ASO and proportional growth in childhood, neo-aortic dimensions continue to increase in adulthood without stabilisation. Annual diameter increase in adulthood was 0.39±0.06, 0.63±0.09 and 0.54±0.11 mm for, respectively, neo-aortic annulus, root and sinotubular junction, all significantly exceeding normal growth. AR continues to develop over time: freedom from AR ≥moderate during the first 25 years post-ASO was 69%. Risk factors for root dilatation were complex TGA anatomy (TGA-ventricular septal defect (VSD), double outlet right ventricle with subpulmonary VSD) and male gender. Risk factors for AR ≥moderate were: complex TGA anatomy and neo-aortic growth. Per millimetre increase in aortic root dimension, there was a 9% increase in the hazard of AR ≥moderate. Bicuspid pulmonary valve did not relate to the presence of root dilatation or AR.

Conclusion: After ASO, neo-aortic dilatation proceeds beyond childhood and is associated with an increase in AR incidence over time. Careful follow-up of the neo-aortic valve and root function is mandatory, especially in males and in patients with complex TGA anatomy.
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http://dx.doi.org/10.1136/heartjnl-2019-315157DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6855793PMC
November 2019

Umbilical Vein Catheter Protruding Through a Pulmonary Vein in a Patient with an Infracardiac Type Total Abnormal Pulmonary Venous Drainage.

Pediatr Cardiol 2019 04 30;40(4):878-879. Epub 2019 Mar 30.

Department of Paediatric Cardiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.

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http://dx.doi.org/10.1007/s00246-019-02094-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6451718PMC
April 2019

Scan-rescan reproducibility of segmental aortic wall shear stress as assessed by phase-specific segmentation with 4D flow MRI in healthy volunteers.

MAGMA 2018 Oct 26;31(5):653-663. Epub 2018 May 26.

Department of Radiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.

Objective: The aim was to investigate scan-rescan reproducibility and observer variability of segmental aortic 3D systolic wall shear stress (WSS) by phase-specific segmentation with 4D flow MRI in healthy volunteers.

Materials And Methods: Ten healthy volunteers (age 26.5 ± 2.6 years) underwent aortic 4D flow MRI twice. Maximum 3D systolic WSS (WSSmax) and mean 3D systolic WSS (WSSmean) for five thoracic aortic segments over five systolic cardiac phases by phase-specific segmentations were calculated. Scan-rescan analysis and observer reproducibility analysis were performed.

Results: Scan-rescan data showed overall good reproducibility for WSSmean (coefficient of variation, COV 10-15%) with moderate-to-strong intraclass correlation coefficient (ICC 0.63-0.89). The variability in WSSmax was high (COV 16-31%) with moderate-to-good ICC (0.55-0.79) for different aortic segments. Intra- and interobserver reproducibility was good-to-excellent for regional aortic WSSmax (ICC ≥ 0.78; COV ≤ 17%) and strong-to-excellent for WSSmean (ICC ≥ 0.86; COV ≤ 11%). In general, ascending aortic segments showed more WSSmax/WSSmean variability compared to aortic arch or descending aortic segments for scan-rescan, intraobserver and interobserver comparison.

Conclusions: Scan-rescan reproducibility was good for WSSmean and moderate for WSSmax for all thoracic aortic segments over multiple systolic phases in healthy volunteers. Intra/interobserver reproducibility for segmental WSS assessment was good-to-excellent. Variability of WSSmax is higher and should be taken into account in case of individual follow-up or in comparative rest-stress studies to avoid misinterpretation.
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http://dx.doi.org/10.1007/s10334-018-0688-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6132557PMC
October 2018

Scan-rescan reproducibility of diastolic left ventricular kinetic energy, viscous energy loss and vorticity assessment using 4D flow MRI: analysis in healthy subjects.

Int J Cardiovasc Imaging 2018 Jun 5;34(6):905-920. Epub 2018 Jan 5.

Department of Radiology, Leiden University Medical Center, Leiden, The Netherlands.

The aim of the current study was to assess the scan-rescan reproducibility of left ventricular (LV) kinetic energy (KE), viscous energy loss (EL) and vorticity during diastole from four-dimensional flow magnetic resonance imaging (4D flow MRI) in healthy subjects. Twelve volunteers (age 27 ± 3 years) underwent whole-heart 4D flow MRI twice in one session. In-scan consistency was evaluated by correlation between KE and EL. EL was computed to measure the amount of EL relative to KE over diastole. Scan-rescan analysis was performed to test reproducibility of volumetric measurements of KE, EL, EL and vorticity in the LV over early (E) and late (A) diastolic filling. In-scan consistency between KE and EL was strong-excellent (E-filling scan1: r = 0.92, P < 0.001; scan2: ρ = 0.96, P < 0.001 and A-filling scan1: ρ = 0.87, P < 0.001; scan2: r = 0.99, P < 0.001). For the majority of subjects (10 out of 12), KE and EL measures showed good to strong reproducibility. However, with a wide range of agreement [intraclass correlation (ICC): 0.64-0.95] and coefficients of variation (CV) ≤ 25%. EL showed strong reproducibility for all 12 subjects with a strong ICC (0.94, P < 0.001) and a CV of 9%. Scan-rescan reproducibility of volumetric vorticity showed good-excellent ICCs (0.83-0.95) with CVs ≤ 11%. In conclusion, the current study shows strong-excellent in-scan consistency and overall good agreement between scans for 4D flow MRI assessment of left ventricular kinetic energy, energy loss and vorticity over diastole. However, substantial differences between the scans were also found in some parameters in two out of twelve subjects. Strong reproducibility was found in the dimensionless EL, which measures the amount of viscous energy loss relative to the average kinetic energy over diastole.
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http://dx.doi.org/10.1007/s10554-017-1291-zDOI Listing
June 2018

In-scan and scan-rescan assessment of LV in- and outflow volumes by 4D flow MRI versus 2D planimetry.

J Magn Reson Imaging 2018 02 22;47(2):511-522. Epub 2017 Jun 22.

Department of Radiology, Leiden University Medical Center, Leiden, the Netherlands.

Purpose: To evaluate the in-scan and scan-rescan consistency of left ventricular (LV) in- and outflow assessment from 1) 2D planimetry; 2) 4D flow magnetic resonance imaging (MRI) with retrospective valve tracking, and 3) 4D flow MRI with particle tracing.

Materials And Methods: Ten healthy volunteers (age 27 ± 3 years) underwent multislice cine short-axis planimetry and whole-heart 4D flow MRI on a 3T MRI scanner twice with repositioning between the scans. LV in- and outflow was compared from 1) 2D planimetry; 2) 4D flow MRI with retrospective valve tracking over the mitral valve (MV) and aortic valve (AV), and 3) 4D flow MRI with particle tracing through forward and backward integration of velocity data.

Results: In-scan consistency between MV and AV flow volumes is excellent for both 4D flow MRI methods with r ≥ 0.95 (P ≤ 0.001). In-scan AV and MV flow by retrospective valve tracking shows good to excellent correlations versus AV and MV flow by particle tracing (r ≥ 0.81, P ≤ 0.004). Scan-rescan SV assessment by 2D planimetry shows excellent reproducibility (intraclass correlation [ICC] = 0.98, P < 0.001, coefficient of variation [CV] = 7%). Scan-rescan MV and AV flow volume assessment by retrospective valve tracking shows strong reproducibility (ICCs ≥ 0.89, P ≤ 0.05, CVs = 12%), as well as by forward and backward particle tracing (ICCs ≥ 0.90, P ≤ 0.001, CVs ≤ 11%). Multicomponent particle tracing shows good scan-rescan reproducibility (ICCs ≥ 0.81, P ≤ 0.007, CVs ≤ 16%).

Conclusion: LV in- and outflow assessment by 2D planimetry and 4D flow MRI with retrospective valve tracking and particle tracing show good in-scan consistency and strong scan-rescan reproducibility, which indicates that both 4D flow MRI methods are reliable and can be used clinically.

Level Of Evidence: 2 Technical Efficacy Stage: 2 J. Magn. Reson. Imaging 2018;47:511-522.
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http://dx.doi.org/10.1002/jmri.25792DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5811894PMC
February 2018

Distribution of blood flow velocity in the normal aorta: Effect of age and gender.

J Magn Reson Imaging 2018 02 26;47(2):487-498. Epub 2017 May 26.

Department of Radiology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois, USA.

Purpose: To apply flow distribution analysis in the entire aorta across a wide age range from pediatric to adult subjects.

Material And Methods: In all, 98 healthy subjects (age 9-78 years, 41 women) underwent 4D flow MRI at 1.5T and 3T for the assessment of 3D blood flow in the thoracic aorta. Subjects were categorized into age groups: group 1 (n = 9, 5 women): 9-15 years; group 2 (n = 13, 8 women): 16-20 years; group 3 (n = 27, 14 women): 21-39 years; group 4 (n = 40, 11 women): 40-59 years; group 5 (n = 9, 3 women): >60 years. Data analysis included the 3D segmentation of the aorta, aortic valve peak velocity, mid-ascending aortic diameter, and calculation of flow velocity distribution descriptors (mean, median, standard deviation, incidence of velocities >1 m/s, skewness, and kurtosis of aortic velocity magnitude). Ascending aortic diameter was normalized by body surface area.

Results: Age was significantly associated with normalized aortic diameter (R = 0.73, P < 0.001), skewness (R = 0.76, P < 0.001), and kurtosis (R = 0.74, P < 0.001), all adjusted by heart rate. Aortic peak velocity and velocity distribution descriptors, adjusted by heart rate, were significantly different between age groups (P < 0.001, analysis of covariance). Skewness and kurtosis significantly increased (P < 0.001) during adulthood (>40 years) as compared with childhood (<21 years). Men and women revealed significant differences (P ≤ 0.05) for peak velocity, incidence, mean, median, standard deviation, and skewness, all adjusted by heart rate.

Conclusion: Aortic hemodynamics significantly change with age and gender, indicating the importance of age- and gender-matched control cohorts for the assessment of the impact of cardiovascular disease on aortic blood flow.

Level Of Evidence: 3 Technical Efficacy: Stage 5 J. Magn. Reson. Imaging 2018;47:487-498.
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http://dx.doi.org/10.1002/jmri.25773DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5702593PMC
February 2018

Altered aortic 3D hemodynamics and geometry in pediatric Marfan syndrome patients.

J Cardiovasc Magn Reson 2017 Mar 17;19(1):30. Epub 2017 Mar 17.

Department of Radiology, Feinberg School of Medicine, Northwestern University , Chicago, IL, USA.

Background: Blood flow dynamics make it possible to better understand the development of aortopathy and cardiovascular events in patients with Marfan syndrome (MFS). Aortic 3D blood flow characteristics were investigated in relation to aortic geometry in children and adolescents with MFS.

Methods: Twenty-five MFS patients (age 15.6 ± 4.0 years; 11 females) and 21 healthy controls (age 16.0 ± 2.6 years; 12 females) underwent magnetic resonance angiography and 4D flow CMR for assessment of thoracic aortic size and 3D blood flow velocities. Data analysis included calculation of aortic diameter and BSA-indexed aortic dimensions (Z-score) along the thoracic aorta, 3D mean systolic wall shear stress (WSS) in ten aortic segments and assessment of aortic blood flow patterns.

Results: Aortic root (root), ascending (AAo) and descending (DAo) aortic size was significantly larger in MFS patients than healthy controls (Root Z-score: 3.56 ± 1.45 vs 0.49 ± 0.78, p < 0.001; AAo Z-score 0.21 ± 0.95 vs -0.54 ± 0.64, p = 0.004; proximal DAo Z-score 2.02 ± 1.60 vs 0.56 ± 0.66, p < 0.001). A regional variation in prevalence and severity of flow patterns (vortex and helix flow patterns) was observed, with the aortic root and the proximal DAo (pDAo) being more frequently affected in MFS. MFS patients had significantly reduced WSS in the proximal AAo (pAAo) outer segment (0.65 ± 0.12 vs. 0.73 ± 0.14 Pa, p = 0.029) and pDAo inner segment (0.74 ± 0.17 vs. 0.87 ± 0.21 Pa, p = 0.021), as well as higher WSS in the inner segment of the distal AAo (0.94 ± 0.14 vs. 0.84 ± 0.15 Pa, p = 0.036) compared to healthy subjects. An inverse relationship existed between pDAo WSS and both pDAo diameter (R = -0.53, p < 0.001) and % diameter change along the pDAo segment (R = -0.64, p < 0.001).

Conclusions: MFS children and young adults have altered aortic flow patterns and differences in aortic WSS that were most pronounced in the pAAo and pDAo, segments where aortic dissection or rupture often originate. The presence of vortex flow patterns and abnormal WSS correlated with regional size of the pDAo and are potentially valuable additional markers of disease severity.
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http://dx.doi.org/10.1186/s12968-017-0345-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5356404PMC
March 2017

Unravelling cardiovascular disease using four dimensional flow cardiovascular magnetic resonance.

Int J Cardiovasc Imaging 2017 Jul 25;33(7):1069-1081. Epub 2016 Nov 25.

Division of Pediatric Cardiology, Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands.

Knowledge of normal and abnormal flow patterns in the human cardiovascular system increases our understanding of normal physiology and may help unravel the complex pathophysiological mechanisms leading to cardiovascular disease. Four-dimensional (4D) flow cardiovascular magnetic resonance (CMR) has emerged as a suitable technique that enables visualization of in vivo blood flow patterns and quantification of parameters that could potentially be of prognostic value in the disease process. In this review, current image processing tools that are used for comprehensive visualization and quantification of blood flow and energy distribution in the heart and great vessels will be discussed. Also, imaging biomarkers extracted from 4D flow CMR will be reviewed that have been shown to distinguish between normal and abnormal flow patterns. Furthermore, current applications of 4D flow CMR in the heart and great vessels will be discussed, showing its potential as an additional diagnostic modality which could aid in disease management and timing of surgical intervention.
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http://dx.doi.org/10.1007/s10554-016-1031-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5489572PMC
July 2017

Reoperation for right ventricular outflow tract obstruction after arterial switch operation for transposition of the great arteries and aortic arch obstruction.

Eur J Cardiothorac Surg 2016 May;49(5):e91-6

Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, Netherlands

Objectives: Right ventricular outflow tract obstruction (RVOTO) is one of the reasons for late reinterventions after repair of transposition of the great arteries (TGA) with aortic arch obstruction (AAO). The aim of the present study was to identify predictors of reoperation for RVOTO in patients who underwent arterial switch operation (ASO) and arch repair for TGA or Taussig-Bing anomaly with AAO.

Methods: Between 1977 and 2015, 45 patients [TGA/intact ventricular septum (IVS) 5, TGA/ventricular septal defect (VSD) 13, Taussig-Bing 27] with coarctation (21), arch hypoplasia (5), coarctation and hypoplasia (12) and aortic arch interruption (7) underwent ASO and arch repair. The median age at the ASO was 19 days (range, 1 day to 12.7 years). AAO was repaired concomitantly with ASO in 36 patients. Operation reports and 2D-echocardiographic data were retrospectively reviewed to determine the following parameters: position of the great arteries, coronary artery anatomy, and diameters of RVOT, aortic annulus, aortic sinotubular-junction, pulmonary annulus and transverse aortic arch previous to ASO. The median follow-up time was 6 years (range, 0-30 years). Four patients were lost to follow-up; reliable echo data were available in 24 subjects. Cox proportional hazard models were performed to examine predictors of reoperation for RVOTO.

Results: Thirty-day mortality rate after ASO was 13% (n = 6), and late mortality rate 9% (n = 4). Ten patients (TGA/VSD 2, Taussig-Bing 8) had 14 reoperations for RVOTO. One patient died after reoperation. Taussig-Bing anomaly was a significant predictor of reoperation for RVOTO [hazard ratio (HR) = 5.5, 95% confidence interval (CI) = 1.15-26.38, P = 0.033]. Higher preoperative aortic annulus Z-score significantly decreased the reoperation risk (HR = 0.6, 95% CI = 0.42-0.93, P = 0.020). In reoperated patients, the mean gradient across the RVOT reduced from 84 ± 12.2 mmHg prior to reoperation to 15.29 ± 13.70 mmHg at latest follow-up.

Conclusions: Taussig-Bing anomaly and smaller preoperative aortic annulus diameter (Z-score) were significant predictors of reoperation for RVOTO in patients after ASO for TGA or Taussig-Bing anomaly with AAO. In Taussig-Bing hearts, the more complex anatomy often necessitates modifications of the operation technique, sometimes precluding RVOT relief at primary ASO. During follow-up, the possibility of recurrent RVOTO should always be considered in this specific patient population. Yet, in case of a reoperation for RVOTO, the surgical relief is in general effective.
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http://dx.doi.org/10.1093/ejcts/ezw026DOI Listing
May 2016

Uhl's anomaly: Clinical spectrum and pathophysiology.

Int J Cardiol 2016 Apr 4;209:118-21. Epub 2016 Feb 4.

Department of Pediatric Cardiology, Emma Children's Hospital, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.

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http://dx.doi.org/10.1016/j.ijcard.2016.02.048DOI Listing
April 2016

Four-dimensional flow cardiovascular magnetic resonance for the evaluation of the atrial baffle after Mustard repair.

Eur Heart J Cardiovasc Imaging 2016 Mar 20;17(3):353. Epub 2015 Dec 20.

Division of Pediatric Cardiology, Department of Pediatrics, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, The Netherlands.

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http://dx.doi.org/10.1093/ehjci/jev331DOI Listing
March 2016

Persistent neo-aortic growth during adulthood in patients after an arterial switch operation.

Heart 2014 Sep 16;100(17):1360-5. Epub 2014 May 16.

Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands The Netherlands Heart Institute, Utrecht, The Netherlands.

Objective: After the arterial switch operation (ASO), disproportional neo-aortic growth during childhood has been reported. Even though it has been suggested neo-aortic dilation will stabilise in adulthood, data are lacking. The aim of this study was to assess the change in neo-aortic dimensions, prevalence of neo-aortic dilation >40 mm and long-term outcome in adults who underwent ASO in childhood.

Methods: All 116 ASO patients operated in a tertiary referral centre and born before 1995 were included. Of these, 83 (72%) survived to adulthood (>17 years) and six were lost to follow-up. Neo-aortic measurements performed in adulthood were collected from available echocardiographic, cardiovascular magnetic resonance and CT images. The time trend was analysed using a mixed model, adjusted for imaging modality.

Results: Clinical data with at least one measurement of the neo-aortic diameter were available in 77 (93%) adult patients and serial measurements in 65 (78%). At baseline (median age 18.1 years), mean neo-aortic diameter was 36±5 mm. Mean neo-aortic growth was 0.31 mm/year (p<0.001 compared with normal value 0.08 mm/year) and was linear over time. Freedom from neo-aortic dilation beyond a diameter of 40 mm was 23% at 28 years of age. During a mean clinical follow-up in adulthood of 7.2 years (IQR 4.0 to 10.1), 3 (4%) patients underwent neo-aortic replacement. No other neo-aortic complications occurred.

Conclusions: In early adulthood, neo-aortic growth was on average linear and did not stabilise over time.
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http://dx.doi.org/10.1136/heartjnl-2014-305702DOI Listing
September 2014

Clinical recognition of mid-aortic syndrome in children.

Eur J Pediatr 2013 Mar 31;172(3):413-6. Epub 2012 Jul 31.

Department of Pediatric Nephrology, Radboud University Nijmegen Medical Centre, P.O. Box 9101, Route 804, 6500 HB, Nijmegen, The Netherlands.

Mid-aortic syndrome is characterized by narrowing of the abdominal aorta, usually with the involvement of renal arteries and other visceral branches. The combination of the presence of an abdominal bruit, diminished or absent pulsations of the lower extremities, and a blood pressure discrepancy between upper and lower extremities is the classic triad associated with mid-aortic syndrome. However, it has a wide variety of clinical symptoms, and awareness of the variable presentation can lead to early diagnosis of the vascular anomaly. We report three cases presenting at three different stages of this disease, such as hydrops fetalis, refractory hypertension, and intracerebral bleeding. In conclusion, these cases highlight the importance of blood pressure measurements in all patients and accurate physical examination for early recognition of a mid-aortic syndrome.
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http://dx.doi.org/10.1007/s00431-012-1800-yDOI Listing
March 2013

[A boy with a red swelling of the lower leg].

Ned Tijdschr Geneeskd 2010 ;154:A549

Máxima Medisch Centrum, afd. Kindergeneeskunde, Veldhoven, The Netherlands.

A 13-year-old boy presented with erythema nodosum on the anterior and lateral side of his lower legs due to enteritis caused by Salmonella enteritidis.
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June 2010

Long-term follow-up of GH-treated girls with Turner syndrome: metabolic consequences.

Horm Res 2009 9;71(6):343-9. Epub 2009 Jun 9.

Department of Pediatrics, Division of Endocrinology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.

Aims: To investigate the metabolic consequences of long-term GH treatment in young women with Turner syndrome (TS), several years after GH discontinuation.

Methods: Follow-up study of a randomized GH dose-response trial, with 3 GH dosages (1.3, 2.0, and 2.7 mg/m(2)/day). Thirty-nine TS patients (20.0 +/- 2.1 years) participated 4.8 +/- 1.9 years after GH discontinuation. Mean GH treatment duration was 8.7 +/- 2.0 years. Fasting glucose, insulin, and serum lipids were measured.

Results: Several years after GH discontinuation, insulin sensitivity remained lower, while beta-cell function and fasting insulin levels remained higher than before treatment. Only BMI influenced beta-cell function. Serum total cholesterol (TC), low-density lipoprotein and high-density lipoprotein (HDL) had further increased compared to 6 months after GH, resulting in higher TC, but also higher HDL levels compared to controls. The atherogenic index remained constant, but lower than controls.

Conclusions: Besides height, GH therapy in girls with TS has additional beneficial effects on serum lipids. Nearly 5 years after discontinuation of GH therapy the favorable effect of GH was still noticeable. The GH-induced decrease in insulin sensitivity, however, remained unchanged, possibly due to having TS.
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http://dx.doi.org/10.1159/000223419DOI Listing
September 2009

Long-term follow-up of GH-treated girls with Turner syndrome: BMI, blood pressure, body proportions.

Horm Res 2009 8;71(6):336-42. Epub 2009 Jun 8.

Department of Pediatrics, Division of Endocrinology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.

Aims: To investigate whether long-term growth hormone (GH) treatment influenced blood pressure (BP), body proportions and BMI in young Turner syndrome (TS) women several years after GH discontinuation.

Methods: A follow-up study of a randomized GH dose-response trial with 3 GH dosages (1.3, 2.0, and 2.7 mg/m(2)/day). 39 TS patients (20.0 +/- 2.1 years) participated 4.8 (1.9) years after GH discontinuation. Mean GH duration was 8.7 (2.0) years.

Measurements: BP, BMI and body proportions.

Results: During GH treatment, DBP had decreased. At the long-term follow-up study, DBP had increased and was similar to pretreatment levels. DBP was negatively influenced by GH dose. SBP was not influenced by GH dose or duration. The BMI increased gradually during and after GH therapy. During GH therapy, shape values of sitting height had decreased to normal values, of foot had increased, and both remained constant after GH discontinuation.

Conclusions: GH therapy in girls with TS has, besides height, additional beneficial effects on BP and body proportions, except foot length. Nearly 5 years after ending GH, the favorable effect of GH on BP was still noticeable. The BMI increased gradually over the years, not influenced by GH.
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http://dx.doi.org/10.1159/000223418DOI Listing
September 2009
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