OBJECTIVES:This study evaluates the long-term outcome of neonatal aortic arch roof enlargement using ductal patency in the context of coarctation associated with aortic arch hypoplasia. METHODS:Retrospective single-centre analysis of children undergoing roof enlargement of the distal arch (left common carotid artery-left subclavian artery) without cardiopulmonary bypass (utilizing ductal patency for lower body perfusion); followed by resection and extended end-to-end anastomosis, through a left posterior thoracotomy. This study evaluates the long-term outcome with emphasis on arch growth and shape. RESULTS:Thirty consecutive patients were included (2006-24). Median age and weight were 6.0 [interquartile range: 4.0-7.8)] days and 3.1 (2.7-3.5) kg, respectively. Simple congenital heart disease with simple intracardiac shunts (n = 17) and complex congenital heart disease (Complete Atrioventricular Septal Defect (AVSD), interrupted aortic arch and univentricular hearts) (n = 13) constituted the cohort. Non-ischaemic clamp time for roof enlargement was 43 (36-50) min. Ischaemic clamp time for coarctation resection was 23 (21-25) min. Pulmonary artery banding was performed in 19 (63.3%) patients. Twenty-seven (90%) successfully underwent staged repair at 6.1 (4.5-8.2) months age. Follow-up was complete at a median duration of 46.9 (21.7-159.9) months. All patients survived the operation and are in good health at follow-up. Median ventilation time, ICU and hospital stay were 1 (1-2), 3 (2-5) and 23.5 (14-40) days, respectively. No patient developed any neurological complication. Three developed left subclavian artery thrombosis, one requiring surgical revision. With one unrelated late accidental death 14 years after neonatal repair, Kaplan-Meier survival was 90.9 [50.8-98.7]% at 15 years. Two patients underwent arch re-enlargement at the inner curvature to accommodate the DKS during stage 2, resulting in freedom from reoperation of 93.3[75.9-98.3]% at 10 years. All survivors enjoy subjective normal exercise tolerance with no relevant gradient. No patient is on anti-hypertensive medication. Median Z value of the proximal, transverse and distal arch was normalized to -0.88 (-2.19 to -0.12), -0.66(-1.33 to 0.08) and 0.34 (-0.10 to 1.33), respectively, at the last follow-up. Twenty-three (76.7%) arches achieved Romanesque shape at follow-up. CONCLUSIONS:Long-term results of this minimally invasive approach show proportional growth without relevant gradient, freedom from hypertension and a Roman arch form, thus making it the preferred approach.

Ventricular volumetry using a short-axis stack of two-dimensional (D) cine balanced steady-state free precession (bSSFP) sequences is crucial in any cardiac magnetic resonance imaging (MRI) examination. This task becomes particularly challenging in children due to multiple breath-holds. To assess the diagnostic performance of accelerated 3-RR cine MRI sequences using deep learning reconstruction compared with standard 2-D cine bSSFP sequences. Twenty-nine consecutive patients (mean age 11 ± 5, median 12, range 1–17 years) undergoing cardiac MRI were scanned with a conventional segmented 2-D cine and a deep learning accelerated cine (three heartbeats) acquisition on a 1.5-tesla scanner. Short-axis volumetrics were performed (semi-)automatically in both datasets retrospectively by two experienced readers who visually assessed image quality employing a 4-point grading scale. Scan times and image quality were compared using the Wilcoxon rank-sum test. Volumetrics were assessed with linear regression and Bland–Altman analyses, and measurement agreement with intraclass correlation coefficient (ICC). Mean acquisition time was significantly reduced with the 3-RR deep learning cine compared to the standard cine sequence (45.5 ± 13.8 s vs. 218.3 ± 44.8 s; P < 0.001). No significant differences in biventricular volumetrics were found. Left ventricular (LV) mass was increased in the deep learning cine compared with the standard cine sequence (71.4 ± 33.1 g vs. 69.9 ± 32.5 g; P < 0.05). All volumetric measurements had an excellent agreement with ICC > 0.9 except for ejection fraction (EF) (LVEF 0.81, RVEF 0.73). The image quality of deep learning cine images was decreased for end-diastolic and end-systolic contours, papillary muscles, and valve depiction (2.9 ± 0.5 vs. 3.5 ± 0.4; P < 0.05). Deep learning cine volumetrics did not differ significantly from standard cine results except for LV mass, which was slightly overestimated with deep learning cine. Deep learning cine sequences result in a significant reduction in scan time with only slightly lower image quality.

Cardiovascular magnetic resonance (CMR) imaging is recommended in patients with congenital heart disease (CHD) in clinical practice guidelines as the imaging standard for a large variety of diseases. As CMR is evolving, novel techniques are becoming available. Some of them are already used clinically, whereas others still need further evaluation. In this statement, the authors give an overview of relevant new CMR techniques for the assessment of CHD. Studies with reference values for these new techniques are listed in the Supplementary data online, supplement.

Cardiovascular magnetic resonance (CMR) imaging is recommended in patients with congenital heart disease (CHD) in clinical practice guidelines as the imaging standard for a large variety of diseases. As CMR is evolving, novel techniques are becoming available. Some of them are already used clinically, whereas others still need further evaluation. In this statement, the authors give an overview of relevant new CMR techniques for the assessment of CHD. Studies with reference values for these new techniques are listed in the Supplementary data online, supplement.

Duchenne muscular dystrophy is life-limiting. Cardiomyopathy, which mostly ensues in the second decade of life, is the main cause of death. Treatment options are still limited. The TAMDMD (NCT03354039) trial assessed motor function, muscle strength and structure, laboratory biomarkers, and safety in 79 ambulant boys with genetically confirmed Duchenne muscular dystrophy, 6.5–12 years of age, receiving either daily tamoxifen 20 mg or placebo for 48 weeks. In this post-hoc analysis, available echocardiographic data of ambulant patients recruited at one study centre were retrieved and compared before and after treatment. Data from 14 patients, median 11 (interquartile range, IQR, 11–12) years of age was available. Baseline demographic characteristics were similar in participants assigned to placebo (n = 7) or tamoxifen (n = 7). Left ventricular end-diastolic diameter in the placebo group (median and IQR) was 39 (38–41) mm at baseline and 43 (38–44) mm at study end, while it was 44 (41–46) mm at baseline and 41 (37–46) mm after treatment in the tamoxifen group. Left ventricular fractional shortening in the placebo group was 35

BackgroundLeft pulmonary artery (LPA) stenting is often required in single ventricle (SV) patients. Due to their close anatomical relationship an LPA stent could potentially compress the left main bronchus (LMB). We assessed the impact of LPA stenting on bronchial size, pulmonary volumes, and lung function in a cohort of SV patients.MethodsForty-nine patients underwent cardiovascular magnetic resonance (CMR) and 36 spirometry 11 (8–15) years after Fontan. All patients were free of respiratory symptoms. LPA stents were inserted in 17 (35%) patients at 8.8 (3.4–12.6) years. Area/shape of the main bronchi (n = 46) and lung volumes (n = 47) were calculated from CMR-ZTE images for each lung and transformed in right-to-left (r/l) ratio and indexed for BSA. The effect of early stent insertion (prior to stage III) was analyzed.ResultsPatients with LPA stent had larger r/l ratio for main bronchus area (p < 0.001) and r/l ratio difference for lung volumes was slightly larger in patients with early stenting. A trend toward a deformation of LMB shape in patients with LPA stent and toward a higher prevalence of abnormal spirometry in patients with early stent implantation was observed.ConclusionsIn this cohort of patients, early insertion of LPA stents seems to relate with smaller LMB sizes and a trend toward smaller left lung volume and higher prevalence of impaired lung function. Whether these findings are caused by the stent or, at least to a certain degree, present prior to the implantation needs to be verified.

In many cardiac diseases, right and left ventricular volumes in systole and diastole are diagnostically and prognostically relevant. Measurements are made by segmentation of the myocardial borders on cardiac magnetic resonance (CMR) images. Automatic detection of myocardial contours is possible by signal thresholding techniques, but must be validated before use in clinical settings. Biventricular volumes were measured in end-diastole (EDVi) and in end-systole (ESVi) both manually and with the MassK application, with signal thresholds at 30%, 50%, and 70%. Stroke volumes (SV) and cardiac indices (CI) were calculated from volumetric measurements and from flow measured in the ascending aorta and the main pulmonary artery, and both methods were compared. Reproducibility of volumetric measurements was tested in 20 patients. Measurements were acquired in 94 patients aged 15 ± 9 years referred for various conditions. EDVi and ESVi of both ventricles were largest with manual segmentation and inversely proportional to the MassK threshold. Manual and k30 SV and CI corresponded best to flow measurements. Interobserver variability was low for all volumes manually and with MassK. In conclusion, manual and 30% threshold-based biventricular volume segmentation agree best with two-dimensional, phantom-corrected phase contrast flow measurements in a young cardiac referral population and are well reproducible.

Four-dimensional time-resolved phase-contrast cardiovascular magnetic resonance imaging (4D flow MRI) enables blood flow quantification in multiple vessels, which is crucial for patients with congenital heart disease (CHD). We investigated net flow volumes in the ascending aorta and pulmonary arteries by four different postprocessing software packages for 4D flow MRI in comparison with 2D cine phase-contrast measurements (2D PC). 4D flow and 2D PC datasets of 47 patients with biventricular CHD (median age 16, range 0.6–52 years) were acquired at 1.5 T. Net flow volumes in the ascending aorta, the main, right, and left pulmonary arteries were measured using four different postprocessing software applications and compared to offset-corrected 2D PC data. Reliability of 4D flow postprocessing software was assessed by Bland–Altman analysis and intraclass correlation coefficient (ICC). Linear regression of internal flow controls was calculated. Interobserver reproducibility was evaluated in 25 patients. Correlation and agreement of flow volumes were very good for all software compared to 2D PC (ICC ≥ 0.94; bias ≤ 5

Purpose To find the best level to measure aortic flow for quantification of aortic regurgitation (AR) in 4D flow CMR. Methods In 27 congenital heart disease patients with AR (67% male, 31 ± 16 years) two blinded observers measured antegrade, retrograde, net aortic flow volumes and regurgitant fractions at 6 levels in 4D flow: (1) below the aortic valve (AV), (2) at the AV, (3) at the aortic sinus, (4) at the sinotubular junction, (5) at the level of the pulmonary arteries (PA) and (6) below the brachiocephalic trunk. 2D phase contrast (2DPC) sequences were acquired at the level of PA. All patients received prior transthoracic echocardiography (TTE) with AR severity grading according to a recommended multiparametric approach. Results After assigning 2DPC measurements into AR grading, agreement between TTE AR grading and 2DPC was good ( κ = 0.88). In 4D flow, antegrade flow was similar between the six levels ( p = 0.87). Net flow was higher at level 1–2 than at levels 3–6 ( p < 0.05). Retrograde flow and regurgitant fraction at level 1–2 were lower compared to levels 3–6 ( p < 0.05). Reproducibility (inter-reader agreement: ICC 0.993, 95% CI 0.986–0.99; intra-reader agreement: ICC 0.982, 95%CI 0.943–0.994) as well as measurement agreement between 4D flow and 2DPC (ICC 0.994; 95%CI 0.989 – 0.998) was best at the level of PA. Conclusion For estimating severity of AR in 4D flow, best reproducibility along with best agreement with 2DPC measurements can be expected at the level of PA. Measurements at AV or below AV might underestimate AR.

Objectives: Pathologic ejection fraction (EF), shortening fraction (FS), and standard heart failure biomarkers (high sensitive troponin T and N-terminal brain natriuretic peptide) during follow-up after childhood cancer have been associated with irreversible cardiac damage. We aimed to evaluate strain imaging values by echocardiography and new biomarkers for heart failure with preserved ejection fraction (HFpEF) as potential more sensitive parameters for cardiac deterioration in childhood cancer survivors (CCS). Materials and Methods: Prospective study with 50 CCS (median 16.2 y) at a median follow-up of 13 years. In addition to standard echo and laboratory parameters for heart failure, strain measurements and new biomarkers, including myocardial inflammation (interleukin 6), extracellular matrix (ECM) remodeling (C-telopeptide for type I collagen, intact N-terminal propeptide of type III procollagen), and other heart failure biomarkers (galectin 3, solutable ST2, growth differentiation factor 15), were obtained and compared with 50 healthy controls. Results: No significant differences in EF, FS, high sensitive troponin T, N-terminal brain natriuretic peptide, interleukin 6, solutable ST2, and galectin 3 were found between study and control groups. In contrast, strain imaging showed significant differences between both groups (global longitudinal strainGLS -16.1% vs. -20.4%, P<0.0001; global circumferential strain -14.3 vs. -21.4%, P<0.0001), detecting 66% (global longitudinal strain) and 76% (global circumferential strain) of patients with pathologic values in contrast to 6% (EF) and 16% (FS) for standard parameters. Markers for disturbances of ECM remodeling (C-telopeptide for type I collagen, intact N-terminal propeptide of type III procollagen, each P<0.0001) and growth differentiation factor 15 (P<0.0001) were significantly different between the groups. Conclusion: Strain imaging and new cardiac biomarkers used in HFpEF focusing on ECM remodeling appear to be more sensitive in detecting early remodeling processes in CCS than standard echo and laboratory parameters.

Background and Aim Fontan patients tend to have reduced physical exercise capacity. This study investigates physical activity (PA) and its relationship to exercise capacity, heart rates, cardiac function, biomarkers, health-related quality of life (HRQoL), and sleep quality. Methods Cardiovascular magnetic resonance (CMR), exercise testing (CPET), 24 h-ECG, and blood samples were prospectively performed in 38 patients, age 13 (11–16) years. PA was assessed by accelerometer during 7 consecutive days. HRQoL was self-assessed with KIDSCREEN-27 and SF-36 according to patients' age; sleep quality with Pediatric Sleep Questionnaire (PSQ) and Pittsburgh Sleep Quality Index (PSQI). Results Daily moderate to vigorous physical activity (MVPA) was in median (IQR) 40 (28–57) mins; 7/38 (18%) patients reached the recommended 60 mins/day of MVPA. MVPA did not correlate with gender, age, single ventricle morphology, time from Fontan, heart rate, ventricular volumes, and ejection fraction at CMR, biomarkers, or CPET. Physical wellbeing ( r = 0.33, p = 0.04), autonomy ( r = 0.39, p = 0.03), and social support ( r = 0.43, p = 0.009) assessed using the KIDSCREEN-27, and both physical ( r = 0.57, p = 0.03) and mental ( r = 0.54, p = 0.04) domains of the SF-36 questionnaire correlated with daily minutes of MVPA. PSQI global sleeping score ( r = −0.7, p = 0.007), and PSQ scales for behavior ( r = −0.36; p = 0.03) correlated with daily minutes of MVPA. Conclusion Only 18% of the Fontan patients meet the recommendation for daily MVPA. Measures of exercise capacity, cardiac function or chronotropic competence are not correlated to daily physical activity. In contrast, HRQoL and sleep quality seem to be associated with regular physical activity.

Introduction: The aim of this study was to evaluate the feasibility of identifying the fetal cardiac and thoracic vascular structures with non-gated dynamic balanced steady-state free precession (SSFP) MRI sequences. Methods: We retrospectively assessed the visibility of cardiovascular anatomy in 60 fetuses without suspicion of congenital heart defect. Non-gated dynamic balanced SSFP sequences were acquired in three anatomic planes of the fetal thorax. The images were analyzed following a segmental approach in consensus reading by an experienced pediatric cardiologist and radiologist. An imaging score was defined by giving one point to each visualized structure, yielding a maximum score of 21 points. Image quality was rated from 0 (poor) to 2 (excellent). The influence of gestational age (GA), field strength, placenta position, and maternal panniculus on image quality and imaging score were tested. Results: 30 scans were performed at 1.5T, 30 at 3T. Heart position, atria, and ventricles could be seen in all 60 fetuses. Basic diagnosis (>12 points) was achieved in 54 cases. The mean imaging score was 16.8+/−3.8. Maternal panniculus (r = −0.3; p = 0.015) and GA (r = 0.6; p < 0.001) correlated with imaging score. Field strength influenced image quality, with 1.5T being better than 3T images (p = 0.012). Imaging score or quality was independent of placenta position. Conclusion: Fetal cardiac MRI with non-gated SSFP sequences enables recognition of basic cardiovascular anatomy.

To use 4D-flow MRI to describe systemic and non-systemic ventricular flow organisation and energy loss in patients with repaired d-transposition of the great arteries (d-TGA) and normal subjects. Pathline tracking of ventricular volumes was performed using 4D-flow MRI data from a 1.5-T GE Discovery MR450 scanner. D-TGA patients following arterial switch (n = 17, mean age 14 ± 5 years) and atrial switch (n = 15, 35 ± 6 years) procedures were examined and compared with subjects with normal cardiac anatomy and ventricular function (n = 12, 12 ± 3 years). Pathlines were classified by their passage through the ventricles as direct flow, retained inflow, delayed ejection flow, and residual volume and visually and quantitatively assessed. Additionally, viscous energy losses (ELv) were calculated. In normal subjects, the ventricular flow paths were well ordered following similar trajectories through the ventricles with very little mixing of flow components. The flow paths in all atrial and some arterial switch patients were more irregular with high mixing. Direct flow and delayed ejection flow were decreased in atrial switch patients’ systemic ventricles with a corresponding increase in residual volume compared with normal subjects (p = 0.003 and p < 0.001 respectively) and arterial switch patients (p < 0.0001 and p < 0.001 respectively). In non-systemic ventricles, arterial switch patients had increased direct flow and decreased delayed ejection fractions compared to normal (p = 0.007 and p < 0.001 respectively) and atrial switch patients (p = 0.01 and p < 0.001 respectively). Regions of high levels of mixing of ventricular flow components showed elevated ELv. 4D-flow MRI pathline tracking reveals disordered ventricular flow patterns and associated ELv in d-TGA patients. • 4D-flow MRI can be used to assess intraventricular flow dynamics in d-TGA patients. • d-TGA arterial switch patients mostly show intraventricular flow dynamics representative of normal subjects, while atrial switch patients show increased flow disorder and different proportions of intraventricular flow volumes. • Flow disruption and disorder increase viscous energy losses.

Patients after surgical repair of Tetralogy of Fallot (rTOF) may suffer a decrease in left ventricular (LV) function. The aim of our study is to evaluate a novel method of assessing LV torsion in patients with rTOF, as an early indicator of systolic LV dysfunction. Motion tracking based on image registration regularized by the equilibrium gap principle, known as equilibrated warping, was employed to assess LV torsion. Seventy-six cases of rTOF and ten normal controls were included. The group of controls was assessed for reproducibility using both equilibrated warping and standard clinical tissue tracking software (CVI42, version 5.10.1, Calgary, Canada). Patients were dichotomized into two groups: normal vs. loss of torsion. Torsion by equilibrated warping was successfully obtained in 68 of 76 (89%) patients and 9 of 10 (90%) controls. For equilibrated warping, the intra- and interobserver coefficients of variation were 0.095 and 0.117, respectively, compared to 0.260 and 0.831 for tissue tracking by standard clinical software. The intra- and inter-observer intraclass correlation coefficients for equilibrated warping were 0.862 and 0.831, respectively, compared to 0.992 and 0.648 for tissue tracking. Loss of torsion was noted in 32 of the 68 (47%) patients with rTOF. There was no difference in LV or RV volumes or ejection fraction between these groups. The assessment of LV torsion by equilibrated warping is feasible and shows good reliability. Loss of torsion is common in patients with rTOF and its robust assessment might contribute into uncovering heart failure in an earlier stage.

Purpose To analyze the dependence of flow volume measurements on 3D cine phase-contrast MRI (4D-flow MRI) background phase correction. Methods In 31 subjects scanned on a 1.5T MRI scanner, flow volume measurements at 4 vessels were made using phantom corrected 2D phase contrast and 4D flow with background phase correction performed by linear, second, third, and fourth-order polynomial fitting to static tissue. Variations in the amount and distribution of static tissue were made to investigate the influence on flow volume measurements. Results Bland Altman comparison of 2D phase-contrast and 4D-flow measurements showed low bias (2.3%-4.8%) and relatively large limits of agreement (13.5%-17.6%). Approximately half of this was attributable to sequence and physiological differences between the 2 scan sequences, demonstrated by smaller limits of agreement (5.3%-10.0%) when comparing 4D-flow measurements with differing background phase corrections. Using only 20% of available static tissue points for polynomial fitting resulted in only 1% difference in flow volume measurements. Using asymmetrically distributed static tissue or including nonstatic tissue for polynomial fitting yielded highly variable differences in flow volume measurements, which became more variable with increased polynomial order. Completely asymmetric static tissue selection resulted in high deviations in flow volume measurements (mean > 7%, max = 345%). Conclusion Comparisons between 2D phase-contrast and 4D-flow volume measurements should consider influences from sequence and physiological differences. A subset of static tissue points may be used with low impact on flow measurements, but should avoid the inclusion of nonstatic tissue and avoid asymmetric distribution. Higher-order polynomial fits are more susceptible to inaccurate static tissue selection.