Background In response to an increased need for patient information in congenital heart disease, we previously developed an online, evidence-based information portal for patients with congenital... Show moreBackground In response to an increased need for patient information in congenital heart disease, we previously developed an online, evidence-based information portal for patients with congenital aortic and pulmonary valve disease. To assess its effectiveness, a stepped-wedge cluster randomised trial was conducted. Methods Adult patients and caregivers of paediatric patients with congenital aortic and/or pulmonary valve disease and/or tetralogy of Fallot who visited the outpatient clinic at any of the four participating centres in the Netherlands between 1 March 2016-1 July 2017 were prospectively included. The intervention (information portal) was introduced in the outpatient clinic according to a stepped-wedge randomised design. One month after outpatient clinic visit, each participant completed a questionnaire on disease-specific knowledge, anxiety, depression, mental quality of life, involvement and opinion/attitude concerning patient information and involvement. Results 343 participants were included (221 control, 122 intervention). Cardiac diagnosis (p=0.873), educational level (p=0.153) and sex (p=0.603) were comparable between the two groups. All outcomes were comparable between groups in the intention-to-treat analyses. However, only 51.6% of subjects in the intervention group (n=63) reported actually visiting the portal. Among these subjects (as-treated), disease-specific knowledge (p=0.041) and mental health (p=0.039) were significantly better than in control subjects, while other baseline and outcome variables were comparable. Conclusion Even after being invited by their cardiologists, only half of the participants actually visited the information portal. Only in those participants that actually visited the portal, knowledge of disease and mental health were significantly better. This underlines the importance of effective implementation of online evidence-based patient information portals in clinical practice. Show less
Hoven, A.T. van den; Yilmazer, S.; Bons, L.R.; Grootel, R.W.J. van; Geenen, L.W.; Berendoncks, A.M. van; ... ; Bosch, A.E. van den 2021
Aim: This study aimed to describe the systolic left ventricular global longitudinal strain (LvGLS) and left ventricular peak early diastolic strain rate (Sre) in adult women with Turner syndrome ... Show moreAim: This study aimed to describe the systolic left ventricular global longitudinal strain (LvGLS) and left ventricular peak early diastolic strain rate (Sre) in adult women with Turner syndrome (TS) and to determine its relationship with exercise capacity and clinical parameters. Methods: In this cross-sectional cohort study, consecutively included adult TS women underwent an electrocardiogram, transthoracic echocardiogram (TTE) and cardiopulmonary exercise test (CPET) on the same day. LvGLS and Sre were measured using 2D speckle tracking analysis (STE) and compared with age-matched healthy female controls. Results: Ninety-four adult women ( age 36 +/- 13 years) with TS and 32 healthy age-matched female controls were included. Women with TS had a significantly impaired/reduced systolic LvGLS (-17.82 +/- 2.98% vs. -21.80 +/- 1.85%, p < 0.001) and Sre (0.98 +/- 0.32 s-1 vs. 1.27 +/- 0.19 s-1, p > 0.001), compared to healthy female controls. Furthermore, TS women had reduced diastolic function as measured by conventional echocardiographic parameters: A higher A-wave ( p < 0.001), lower E/A-ratio (p = 0.001), longer deceleration time (p = 0.006), and a higher E/E'-ratio (p < 0.001). Women with TS also had a significantly reduced maximal workload (p = 0.033), reduced oxygen uptake (p < 0.001) and a reduced maximal heart rate (p < 0.001) during exercise. Multivariable linear regression analysis revealed that Age, karyotype and QT-duration were significantly associated with Sre, but not with LvGLS, in the TS population. Conclusion: Systolic and diastolic strain and exercise capacity were significantly reduced in TS women compared to healthy women. No correlation between strain itself and exercise capacity could be demonstrated, but correlations with conventional TTE parameters and baseline characteristics were found. Show less
Background: Patients with a bicuspid aortic valve (BAV) are at risk of developing valve deterioration and aortic dilatation. We aimed to investigate whether blood biomarkers are associated with... Show moreBackground: Patients with a bicuspid aortic valve (BAV) are at risk of developing valve deterioration and aortic dilatation. We aimed to investigate whether blood biomarkers are associated with disease stage inpatients with BAV.Methods: Serum levels of high sensitivity C-reactive protein (hsCRP), high sensitivity troponin T (hsTnT), N-terminal pro-B-type natriuretic peptide (NT-proBNP), and total transforming growth factor-beta 1 (TGF-beta 1) were measured in adult BAV patients with valve dysfunction or aortic pathology. Age-matched general population controls were included for TGF beta-1 measurements. Correlation analyses and multivariable linear regression were used to determine the association between (2log-transformed) biomarker levels and aortic valve regurgitation, aortic valve stenosis, aortic dilatation, or left ventricular function.Results: hsCRP and hsTnT were measured in the total group of 183 patients (median age 34 years, 25th-75th percentile 23-46), NT-proBNP in 162 patients, and TGF-beta 1 beta in 108 patients. Elevated levels of NT-proBNP were found in 20% of the BAV patients, elevated hsTnT in 6%, and elevated hsCRP in 7%. Higher hsTnT levels were independently associated with aortic regurgitation [odds ratio per doubling (OR2log) 1.34, 95% CI 1.01;1.76] and higher NT-proBNP levels with aortic valve maximal velocity (beta(2log) 0.17, 95%CI 0.07;028) and aortic regurgitation (OR2log 1.41, 95%CI 1.11;1.79). Both BAV patients with (9.9 +/- 2.7 ng/mL) and without aortic dilatation (10.4 +/- 2.9 ng/mL) showed lower TGF-beta 1 levels compared to general population controls (n = 85, 11.8 +/- 3.2 ng/mL).Conclusions: Higher NT-proBNP and hsTNT levels were associated with aortic valve disease in BAV patients. TGF-beta 1 levels were lower in BAV patients than in the general population, and not related to aortic dilatation. Longitudinal data are needed to further investigate the prognostic value of biomarkers in these patients. (C) 2020 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved. Show less
Hoven, A.T. van den; Yilmazer, S.; Chelu, R.G.; Grootel, R.W.J. van; Minderhoud, S.C.S.; Bons, L.R.; ... ; Hirsch, A. 2020
Left ventricular global longitudinal strain (LVGLS) analysis is a sensitive measurement of myocardial deformation most often done using speckle-tracking transthoracic echocardiography (TTE). We... Show moreLeft ventricular global longitudinal strain (LVGLS) analysis is a sensitive measurement of myocardial deformation most often done using speckle-tracking transthoracic echocardiography (TTE). We propose a novel approach to measure LVGLS using feature-tracking software on the magnitude dataset of 4D flow cardiovascular magnetic resonance (CMR) and compare it to dynamic computed tomography (CT) and speckle tracking TTE derived measurements. In this prospective cohort study 59 consecutive adult patients with a bicuspid aortic valve (BAV) were included. The study protocol consisted of TTE, CT, and CMR on the same day. Image analysis was done using dedicated feature-tracking (4D flow CMR and CT) and speckle-tracking (TTE) software, on apical 2-, 3-, and 4-chamber long-axis multiplanar reconstructions (4D flow CMR and CT) or standard apical 2-, 3-, and 4-chamber acquisitions (TTE). CMR and CT GLS analysis was feasible in all patients. Good correlations were observed for GLS measured by CMR (- 21 +/- 3%) and CT (- 20 +/- 3%) versus TTE (- 20 +/- 3%, Pearson's r: 0.67 and 0.65, p < 0.001). CMR also correlated well with CT (Pearson's r 0.62, p < 0.001). The inter-observer analysis showed moderate to good reproducibility of GLS measurement by CMR, CT and TTE (Pearsons's r: 0.51, 0.77, 0.70 respectively; p < 0.05). Additionally, ejection fraction (EF), end-diastolic and end-systolic volume measurements (EDV and ESV) correlated well between all modalities (Pearson's r > 0.61, p < 0.001). Feature-tracking GLS analysis is feasible using the magnitude images acquired with 4D flow CMR. GLS measurement by CMR correlates well with CT and speckle-tracking 2D TTE. GLS analysis on 4D flow CMR allows for an integrative approach, integrating flow and functional data in a single sequence. Not applicable, observational study. Show less
Pol, V. van de; Bons, L.R.; Lodder, K.; Kurakula, K.B.; Sanchez Duffhues, G.; Siebelink, H.M.J.; ... ; Goumans, M.J. 2019
Bicuspid aortic valve (BAV), the most common congenital heart defect, is associated with an increased prevalence of aortic dilation, aortic rupture and aortic valve calcification. Endothelial cells... Show moreBicuspid aortic valve (BAV), the most common congenital heart defect, is associated with an increased prevalence of aortic dilation, aortic rupture and aortic valve calcification. Endothelial cells (ECs) play a major role in vessel wall integrity. Little is known regarding EC function in BAV patients due to lack of patient derived primary ECs. Endothelial colony forming cells (ECFCs) have been reported to be a valid surrogate model for several cardiovascular pathologies, thereby facilitating an in vitro system to assess patient-specific endothelial dysfunction. Therefore, the aim of this study was to investigate cellular functions in ECFCs isolated from BAV patients. Outgrowth and proliferation of ECFCs from patients with BAV (n = 34) and controls with a tricuspid aortic valve (TAV, n = 10) were determined and related to patient characteristics. Interestingly, we were only able to generate ECFCs from TAV and BAV patients without aortic dilation, and failed to isolate ECFC colonies from patients with a dilated aorta. Analyzing EC function showed that while proliferation, cell size and endothelial-to-mesenchymal transition were similar in TAV and BAV ECFCs, migration and the wound healing capacity of BAV ECFCs is significantly higher compared to TAV ECFCs. Furthermore, calcification is blunted in BAV compared to TAV ECFCs. Our results reveal ECs dysfunction in BAV patients and future research is required to unravel the underlying mechanisms and to further validate ECFCs as a patient-specific in vitro model for BAV. Show less
Background: No established reference-standard technique is available for ascending aortic diameter measurements. The aim of this study was to determine agreement between modalities and techniques... Show moreBackground: No established reference-standard technique is available for ascending aortic diameter measurements. The aim of this study was to determine agreement between modalities and techniques.Methods: In patients with aortic pathology transthoracic echocardiography, computed tomography angiography (CTA) and magnetic resonance angiography (MRA) were performed. Aortic diameters were measured at the sinus of Valsalva (SoV), sinotubular junction (STJ) and tubular ascending aorta (TAA) during mid-systole and end-diastole. In echocardiography both the inner edge-to-inner edge (14 edge) and leading edge-to-leading edge (L-L edge) methods were applied, and the length of the aortic annulus to the most cranial visible part of the ascending aorta was measured. In CTA and MRA the I-1 method was used.Results: Fifty patients with bicuspid aortic valve (36 +/- 13 years, 26' female) and 50 Turner patients (35 +/- 13 years) were included. Comparison of all aortic measurements showed a mean difference of 5.4 +/- 2.7 mm for the SoV, 5.1 +/- 2.0 mm for the STJ and 4.8 +/- 2.1 mm for the TAA. The maximum difference was 18 mm. The best agreement was found between echocardiography L-L edge and CTA during mid-systole. CTA and MRA showed good agreement. A mean difference of 1.5 +/- 1.3 mm and 1.8 +/- 1.5 mm was demonstrated at the level of the STJ and TAA comparing mid-systolic with end-diastolic diameters. The visible length of the aorta increased on average 5.3 +/- 5.1 mmW during mid-systole.Conclusions: MRA and CTA showed best agreement with L-L edge method by echocardiography. In individual patients large differences in ascending aortic diameter were demonstrated, warranting measurement standardization. The use of CIA or MRA is advised at least once. (C) 2018 Elsevier B.V. All rights reserved. Show less
Pol, V. van de; Bons, L.R.; Lodder, K.; Kurakula, K.B.; Sanchez Duffhues, G.; Siebelink, H.M.J.; ... ; Goumans, M.J. 2019
Bicuspid aortic valve (BAV), the most common congenital heart defect, is associated with an increased prevalence of aortic dilation, aortic rupture and aortic valve calcification. Endothelial cells... Show moreBicuspid aortic valve (BAV), the most common congenital heart defect, is associated with an increased prevalence of aortic dilation, aortic rupture and aortic valve calcification. Endothelial cells (ECs) play a major role in vessel wall integrity. Little is known regarding EC function in BAV patients, due to lack of patient derived primary ECs. Endothelial colony forming cells (ECFCs) have been reported to be a valid surrogate model for several cardiovascular pathologies, thereby facilitating an in vitro system to assess patient-specific endothelial dysfunction. Therefore, the aim of this study was to investigated cellular functions in ECFCs isolated from BAV patients. Outgrowth and proliferation of ECFCs from patients with BAV (n=34) and controls with a tricuspid aortic valve (TAV, n=10) was determined and related to patient characteristics. Interestingly, we were only able to generate ECFCs from TAV and BAV patients without aortic dilation and failed to isolate ECFC colonies from patients with a dilated aorta. Analyzing EC function showed that while proliferation, cell size and endothelial-to-mesenchymal transition were similar in TAV and BAV ECFCs, migration and the wound healing capacity of BAV ECFCs is significantly higher compared to TAV ECFCs. Furthermore, calcification is blunted in BAV compared to TAV ECFCs. Our results reveal ECs dysfunction in BAV patients and future research is required to unravel the underlying mechanisms and to further validate ECFCs as a patient-specific in vitro model for BAV. Show less
