Objectives: The primary aim was to gain insight into the growth of the aortic root in children and young adults with Marfan syndrome (MFS). Furthermore, we aimed to identify a clinical profile of... Show moreObjectives: The primary aim was to gain insight into the growth of the aortic root in children and young adults with Marfan syndrome (MFS). Furthermore, we aimed to identify a clinical profile of patients with MFS who require an aortic root replacement at a young age with specific interest in age, sex, height and fibrillin-1 (FBN1) genotype. Methods: Aortic root dimensions of 97 patients with MFS between 0 year and 20 years and 30 controls were serially assessed with echocardiography. Trends were analysed using a linear mixed-effect model. Additionally, including only patients with MFS, we allowed trends to differ by sex, aortic root replacement and type of FBN1 mutation. Results: Average aortic root dilatation in patients with MFS became more pronounced after the age of 8 years. In the MFS cohort, male patients had a significantly greater aortic root diameter than female patients, which was in close relationship with patient height. There was no difference in aortic root growth between children with dominant negative (DN) or haploinsufficient FBN1 mutations. However, DN-FBN1 variants resulting in loss of cysteine content were associated with a more severe phenotype. Eleven children needed an aortic root replacement. Compared with patients with MFS without aortic root surgery, these children had a significantly larger aortic root diameter from an early age. Conclusions:This study provides clinically useful longitudinal growth charts on aortic root growth in children and young adults with MFS. Children requiring prophylactic aortic root replacement during childhood can be identified at a young age. Our growth charts can help clinicians in decision making with regard to follow-up and prophylactic therapy. Loss of cysteine content in the FBN1 protein was associated with larger aortic root dimensions. Show less
OBJECTIVES Long-term prognosis after ventricular septal defect (VSD) correction in childhood is excellent. Nevertheless, decreased biventricular systolic performance has been described immediately... Show moreOBJECTIVES Long-term prognosis after ventricular septal defect (VSD) correction in childhood is excellent. Nevertheless, decreased biventricular systolic performance has been described immediately following VSD surgery in children. In an effort to better understand this decrease and its time-course, we characterized biventricular systolic performance following VSD closure in paediatric patients up to 20 months postoperatively. METHODS Thirty-nine children undergoing VSD surgery and 22 age-matched controls were included for echocardiographic follow-up of left (LV) and right ventricular (RV) systolic performance. LV fractional shortening and tricuspid annular plane systolic excursion (TAPSE) were assessed. Additionally, tissue Doppler imaging measurements were obtained at the basal LV lateral wall and RV free wall to assess both LV and RV systolic (S(')) performance. Studies were performed preoperatively, 1 day postoperatively, at discharge and 3-20 months postoperatively at medium-term follow-up. RESULTS After an initial decrease in biventricular systolic performance, a significant recovery was observed within the first year after VSD surgery. After a medium-term follow-up of 8.4 ± 5.3 months, LV systolic performance parameters were normalized, while RV systolic performance parameters remained impaired in patients vs controls (TAPSE: 12.5 ± 1.2 vs 18.5 ± 3.2 mm, RV S('):8.9 ± 1.3 vs 12.5 ± 2.2 cm/s). CONCLUSIONS Within the first year after VSD correction, LV systolic performance had normalized, while RV systolic performance remained significantly impaired up to 20 months after VSD closure. Both detrimental effects of open heart surgery with cardiopulmonary bypass and preoperative alterations may add to the observed postoperative impairment of specifically RV performance. Show less
Klitsie, L.M.; Roest, A.A.W.; Kuipers, I.M.; Hulst, A.E. van der; Hazekamp, M.G.; Blom, N.A.; Harkel, A.D.J. ten 2013
Cardiac surgery with cardiopulmonary bypass is associated with the development of a systemic inflammatory response, which can lead to myocardial damage. However, knowledge concerning the time... Show moreCardiac surgery with cardiopulmonary bypass is associated with the development of a systemic inflammatory response, which can lead to myocardial damage. However, knowledge concerning the time course of ventricular performance deterioration and restoration after correction of a congenital heart defect (CHD) in pediatric patients is sparse. Therefore, the authors perioperatively quantified left ventricular (LV) and right ventricular (RV) performance using echocardiography. Their study included 141 patients (ages 0-18 years) undergoing CHD correction and 40 control subjects. The study assessed LV systolic performance (fractional shortening) and diastolic performance (mitral Doppler flow) in combination with RV systolic performance [tricuspid annular plane systolic excursion (TAPSE)] and diastolic performance (tricuspid Doppler flow). Additionally, systolic (S') and diastolic (E', A', E/E') tissue Doppler imaging (TDI) measurements were obtained at the LV lateral wall, the interventricular septum, and the RV free wall. Echocardiographic studies were performed preoperatively, 1 day postoperatively, and at hospital discharge after 9 ± 5 days. Although all LV echocardiographic measurements showed a deterioration 1 day after surgery, only LV TDI measurements were impaired in patients at discharge versus control subjects (S': 5.7 ± 2.0 vs 7.1 ± 2.7 cm/s; E': 9.8 ± 3.9 vs 13.7 ± 5.1 cm/s; E/E': 12.2 ± 6.4 vs 8.8 ± 4.3; p < 0.05). In the RV, TAPSE and RV TDI velocities also were impaired in patients at discharge versus control subjects (TAPSE: 9 ± 3 vs 17 ± 5 mm; S': 5.2 ± 1.7 vs 11.4 ± 3.4 cm/s; E': 7.3 ± 2.5 vs 16.3 ± 5.2 cm/s; E/E': 12.5 ± 6.8 vs 4.8 ± 1.9; p < 0.05). Furthermore, longer aortic cross-clamp times were associated with more impaired postoperative LV and RV performance (p < 0.05). In conclusion, both systolic and diastolic biventricular performances were impaired shortly after CHD correction. This impairment was detected only by TDI parameters and TAPSE. Furthermore, a longer-lasting negative influence of cardiopulmonary bypass on myocardial performance was suggested. Show less
Hulst, A.E. van der; Roest, A.A.W.; Holman, E.R.; Vliegen, H.W.; Hazekamp, M.G.; Bax, J.J.; ... ; Delgado, V. 2012
The thesis provides new insights into advanced echocardiographic and magnetic resonance imaging techniques for comprehensive mechanical assessment of the right ventricle in healthy children and in... Show moreThe thesis provides new insights into advanced echocardiographic and magnetic resonance imaging techniques for comprehensive mechanical assessment of the right ventricle in healthy children and in pediatric patients with right ventricular dysfunction. It is shown that the right ventricle does not contract synchronously in the longitudinal direction and that this is related to right ventricular function. Various echocardiographic (Tissue Doppler imaging, Speckle tracking, 3D echocardiography) and magnetic resonance imaging techniques (Tissue Velocity imaging, 3D flow imaging) are validated for use in the right ventricle in pediatric patients. Show less
Purpose: To compare velocity-encoded (VE) magnetic resonance (MR) imaging with tissue Doppler imaging to assess right ventricular (RV) peak systolic velocities and timing of velocities in patients... Show morePurpose: To compare velocity-encoded (VE) magnetic resonance (MR) imaging with tissue Doppler imaging to assess right ventricular (RV) peak systolic velocities and timing of velocities in patients with corrected tetralogy of Fallot and healthy subjects. Materials and Methods: Local institutional review board approval was obtained; patients or their parents gave informed consent. Thirty-three patients (20 male, 13 female; median age, 12 years; interquartile range [IQR], 11-15 years; age range, 8-18 years) and 19 control subjects (12 male, seven female; median age, 14 years; IQR, 12-16 years; age range, 8-18 years) underwent VE MR imaging and tissue Doppler imaging. Peak systolic velocity and time to peak systolic velocity (percentage of cardiac cycle) were assessed at the RV free wall (RVFW) and RV outflow tract (RVOT). Data were analyzed by using linear regression, paired and unpaired tests, and Bland-Altman plots. Results: Good correlation and agreement between the two techniques were observed. For peak systolic velocity at RVFW, r = 0.95 (mean difference, -0.4 cm/sec, P < .01), and at RVOT, r = 0.95 (mean difference, -0.4 cm/sec, P = .02). For timing at RVFW, r = 0.94 (mean difference, -0.2%, P = .44), and at RVOT, r = 0.89 (mean difference, -0.5%, P = .01). Peak systolic velocity was reduced in patients with corrected tetralogy of Fallot (at RVFW, median was 8.2 cm/sec [IQR, 6.4-9.7 cm/sec] vs 12.4 cm/sec [IQR, 10.8-13.8 cm/sec], P,.01; at RVOT, 4.7 cm/sec [IQR, 4.1-7.2 cm/sec] vs 10.2 cm/sec [IQR, 8.7-11.2 cm/sec], P,.01). The time delay between RVFW and RVOT was observed, which was significantly shorter in patients with corrected tetralogy of Fallot (median, 5.9% [IQR, 4.9%-7.4%] vs 8.4% [IQR, 6.6%-12.4%], P < .01). Conclusion: VE MR imaging and tissue Doppler imaging enable assessment of RV systolic performance and timing of velocities at the RVFW and RVOT in patients with corrected tetralogy of Fallot. Both techniques can be used interchangeably to clinically assess velocities and timing of velocities of the RV. (C) RSNA, 2011 Show less
Hulst, A.E. van der; Roest, A.A.W.; Delgado, V.; Holman, E.R.; Roos, A. de; Blom, N.A.; Bax, J.J. 2011
OBJECTIVE Right ventricular (RV) dysfunction is common in patients with corrected tetralogy of Fallot (cToF). Abnormalities in the temporal pattern of RV mechanical activation have been observed in... Show moreOBJECTIVE Right ventricular (RV) dysfunction is common in patients with corrected tetralogy of Fallot (cToF). Abnormalities in the temporal pattern of RV mechanical activation have been observed in patients with cToF, but the relationship with RV performance remains unclear. This study characterises RV performance and the temporal sequence of RV deformation in patients with cToF and healthy controls. STUDY DESIGN 37 patients with cToF were compared with 18 controls. Using two-dimensional speckle tracking analysis, global RV strain was assessed. In addition, time to peak strain and the time difference between RV inlet and RV outlet (RV time delay) was assessed. MAIN OUTCOME MEASURE The relation between RV performance and RV time delay was assessed with linear regression analysis. Results RV strain was reduced in patients compared with controls (-20.9 ± 4.3% vs -30.7 ± 3.4%, p<0.001). Time to peak strain at the RV inlet showed no differences between patients with cToF and controls (406 ± 55 ms vs 405 ± 67 ms, p=0.429), whereas time to peak strain at the RV outlet was significantly delayed in patients with cToF (339 ± 75 ms vs 262 ± 85 ms, p=0.003). Consequently, RV time delay was significantly shorter in patients with cToF than in controls (66 ± 48 ms vs 143 ± 53 ms, p<0.001). A close relation between RV time delay and RV strain was observed (r=-0.70, p<0.001). CONCLUSION In patients with cToF, RV outlet deformation is delayed, causing a reduction in RV time delay which is significantly related to impairment in RV performance. Show less
Hulst, A.E. van der; Roest, A.A.W.; Delgado, V.; Holman, E.R.; Roos, A. de; Blom, N.A.; Bax, J.J. 2011
Objective Right ventricular (RV) dysfunction is common in patients with corrected tetralogy of Fallot (cToF). Abnormalities in the temporal pattern of RV mechanical activation have been observed in... Show moreObjective Right ventricular (RV) dysfunction is common in patients with corrected tetralogy of Fallot (cToF). Abnormalities in the temporal pattern of RV mechanical activation have been observed in patients with cToF, but the relationship with RV performance remains unclear. This study characterises RV performance and the temporal sequence of RV deformation in patients with cToF and healthy controls. Study design 37 patients with cToF were compared with 18 controls. Using two-dimensional speckle tracking analysis, global RV strain was assessed. In addition, time to peak strain and the time difference between RV inlet and RV outlet (RV time delay) was assessed. Main outcome measure The relation between RV performance and RV time delay was assessed with linear regression analysis. Results RV strain was reduced in patients compared with controls (-20.9 +/- 4.3% vs -30.7 +/- 3.4%, p < 0.001). Time to peak strain at the RV inlet showed no differences between patients with cToF and controls (406 +/- 55 ms vs 405 +/- 67 ms, p=0.429), whereas time to peak strain at the RV outlet was significantly delayed in patients with cToF (339 +/- 75 ms vs 262 +/- 85 ms, p=0.003). Consequently, RV time delay was significantly shorter in patients with cToF than in controls (66 +/- 48 ms vs 143 +/- 53 ms, p < 0.001). A close relation between RV time delay and RV strain was observed (r=-0.70, p < 0.001). Conclusion In patients with cToF, RV outlet deformation is delayed, causing a reduction in RV time delay which is significantly related to impairment in RV performance. Show less
Hulst, A.E. van der; Westenberg, J.J.M.; Kroft, L.J.M.; Bax, J.J.; Blom, N.A.; Roos, A. de; Roest, A.A.W. 2010
Purpose: To evaluate three-dimensional (3D) velocity-encoded (VE) magnetic resonance (MR) imaging, as compared with two-dimensional (2D) VE MR imaging, for assessment of pulmonary valve (PV) and... Show morePurpose: To evaluate three-dimensional (3D) velocity-encoded (VE) magnetic resonance (MR) imaging, as compared with two-dimensional (2D) VE MR imaging, for assessment of pulmonary valve (PV) and tricuspid valve (TV) flow, with planimetry as the reference standard, and to evaluate diastolic function in patients with a corrected tetralogy of Fallot (TOF). Materials and Methods: Local institutional review board approval was obtained, and patients or their parents gave informed consent. Twenty-five patients with a corrected TOF (12 male, 13 female; mean age, 13.1 years +/- 2.7 [standard deviation]; age range, 8-18 years) and 19 control subjects (12 male, seven female; mean age, 14.1 years +/- 2.4; age range, 8-18 years) underwent planimetric MR imaging, 2D VE MR imaging, and 3D VE MR imaging for TV and PV flow evaluation. For evaluation of diastolic function, PV and TV flow were summated. Data were analyzed by using linear regression analysis, paired and unpaired t testing, and Bland-Altman plots. Results: Strong correlations between the 2D VE MR and 3D VE MR measurements of PV flow (for forward flow: r = 0.87, P < .01; for backward fl ow: r = 0.97, P < .01) were observed. With PV effective fl ow as a reference, 3D TV effective fl ow measurements were more accurate than 2D TV effective fl ow measurements: In patients, the mean 2D TV effective fl ow versus 2D PV effective fl ow difference was 17.6 mL +/- 11 (P < .001), and the mean 3D TV effective fl ow versus 3D PV effective fl ow difference was - 1.2 mL +/- 4.7 (P = .22). Diastolic functional impairment in patients could be detected at 3D VE MR imaging diastolic assessment. Conclusion: Three-dimensional VE MR imaging is accurate for PV fl ow assessment and is more accurate than 2D VE MR imaging for TV fl ow evaluation. Assessment of diastolic function with 3D VE MR imaging can facilitate ongoing research of diastolic dysfunction in patients with a corrected TOF. Show less
Hulst, A.E. van der; Delgado, V.; Holman, E.R.; Kroft, L.J.M.; Roos, A. de; Hazekamp, M.G.; ... ; Roest, A.A.W. 2010
In patients with corrected tetralogy of Fallot (cToF), left ventricular (LV) dysfunction is closely related to right ventricular (RV) dysfunction, indicating adverse ventricular ventricular... Show moreIn patients with corrected tetralogy of Fallot (cToF), left ventricular (LV) dysfunction is closely related to right ventricular (RV) dysfunction, indicating adverse ventricular ventricular interactions. However, the mechanism that links RV dysfunction to LV dysfunction remains unclear. In this prospective study, 32 patients with cToF and 19 controls were enrolled. With cardiac magnetic resonance imaging, biventricular ejection fractions were assessed. Using 2-dimensional speckle tracking, global and regional RV and LV strains and LV twist were assessed. To detect and characterize ventricular-ventricular interaction, the relation between global and regional RV mechanics and global and regional IN mechanics was assessed. Global RV strain, global LV strain, and LV twist were decreased in patients with cToF. Global RV strain correlated with global I,V strain (r = 0.66, p < 0.001) and LV twist (r = -0.72, p < 0.001), indicating the presence of adverse ventricular ventricular interaction. Furthermore, close relations were observed between apical RV strain and apical LV strain (r = 0.62, p < 0.001) and apical LV rotation (r = 0.67, p < 0.001). In conclusion, RV strain was significantly related to LV strain and I,V twist in patients with cToF and controls. Furthermore, apical RV strain correlated with apical IN strain and apical LV rotation, indicating adverse apical ventricular ventricular interactions. (C) 2010 Elsevier Inc. All rights reserved. (Am J Cardiol 2010;106:723-729) Show less
