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Cardiovascular MRI has become an essential imaging modality in children with congenital heart disease (CHD) in the last 15–20 years. With use of appropriate sequences, it provides important information on cardiovascular anatomy, blood flow and function for initial diagnosis and post-surgical or -interventional monitoring in children. Although considered as more sophisticated and challenging than CT, in particular in neonates and infants, MRI is able to provide information on intra- and extracardiac haemodynamics, in contrast to CT. In recent years, four-dimensional (4-D) flow MRI has emerged as an additional MR technique for retrospective assessment and visualisation of blood flow within the heart and any vessel of interest within the acquired three-dimensional (3-D) volume. Its application in young children requires special adaptations for the smaller vessel size and faster heart rate compared to adolescents or adults. In this article, we provide an overview of 4-D flow MRI in various types of complex CHD in neonates and infants to demonstrate its potential indications and beneficial application for optimised individual cardiovascular assessment. We focus on its application in clinical routine cardiovascular workup and, in addition, show some examples with pathologies other than CHD to highlight that 4-D flow MRI yields new insights in disease understanding and therapy planning. We shortly review the essentials of 4-D flow data acquisition, pre- and post-processing techniques in neonates, infants and young children. Finally, we conclude with some details on accuracy, limitations and pitfalls of the technique.

Objective: Pulmonary valve regurgitation (PR) and right ventricular (RV) dilatation are important residual findings after surgical repair of tetralogy of Fallot (TOF). We sought to describe the natural course of RV dilatation over time in patients with severe PR after TOF repair and to determine risk factors for quick progression of RV dilatation and dysfunction. Methods: Data of 85 consecutive TOF patients with PR and RV dilatation, undergoing serial cardiovascular magnetic resonance (CMR) scans between July 2002 and December 2016 in two institutions, were retrospectively reviewed. The dataset was analyzed regarding right and left ventricular (LV) volume and function and potential risk factors of progressive RV dilatation Results: There was no significant increase in RV end-diastolic volumes (RVEDVi) indexed body surface area (BSA) (median 150 [81-249] vs. 150 [82-260] mL/m2) and end-systolic volumes indexed for BSA (RVESVi) (75 [20-186] vs. 76 [39-189] mL/m2) between the first and last CMR in the overall group. Similarly, there were no significant changes in LV volumes indexed for BSA (LVEDVi 78 [56-137] vs. 81 [57-128] mL/m2 and LV end-systolic volume index 34 [23-68] vs. 35 [18-61] mL/m2). Global function remained also unchanged for both ventricles. RVEDVi increased statistically significantly (≥ 20 mL/m2) in twenty patients (24%) from 154 mL/m2 (87-237) to 184 mL/m2 (128-260, P < 0.001). LV dimensions showed a similar trend with LVEDVi increase from 80 ml/m2 (57-98) to 85 ml/m2 (72-105, P = 0.002). Shorter time interval between repair and first CMR was the only risk factor predictive for progressive RV dilatation. Conclusion: In the majority of patients with repaired TOF and severe PR, RV dilatation is unchanged during a follow-up of 3 years. RV dilatation seems to progress early after surgery and subsequently stabilize. RV dilatation significantly progresses in a subgroup of 24% of patients, with a shorter time interval since surgical repair.

Background A velocity offset error in phase contrast cardiovascular magnetic resonance (CMR) imaging is a known problem in clinical assessment of flow volumes in vessels around the heart. Earlier studies have shown that this offset error is clinically relevant over different systems, and cannot be removed by protocol optimization. Correction methods using phantom measurements are time consuming, and assume reproducibility of the offsets which is not the case for all systems. An alternative previously published solution is to correct the in-vivo data in post-processing, interpolating the velocity offset from stationary tissue within the field-of-view. This study aims to validate this interpolation-based offset correction in-vivo in a multi-vendor, multi-center setup. Methods Data from six 1.5 T CMR systems were evaluated, with two systems from each of the three main vendors. At each system aortic and main pulmonary artery 2D flow studies were acquired during routine clinical or research examinations, with an additional phantom measurement using identical acquisition parameters. To verify the phantom acquisition, a region-of-interest (ROI) at stationary tissue in the thorax wall was placed and compared between in-vivo and phantom measurements. Interpolation-based offset correction was performed on the in-vivo data, after manually excluding regions of spatial wraparound. Correction performance of different spatial orders of interpolation planes was evaluated. Results A total of 126 flow measurements in 82 subjects were included. At the thorax wall the agreement between in-vivo and phantom was − 0.2 ± 0.6 cm/s. Twenty-eight studies were excluded because of a difference at the thorax wall exceeding 0.6 cm/s from the phantom scan, leaving 98. Before correction, the offset at the vessel as assessed in the phantom was − 0.4 ± 1.5 cm/s, which resulted in a − 5 ± 16% error in cardiac output. The optimal order of the interpolation correction plane was 1st order, except for one system at which a 2nd order plane was required. Application of the interpolation-based correction revealed a remaining offset velocity of 0.1 ± 0.5 cm/s and 0 ± 5% error in cardiac output. Conclusions This study shows that interpolation-based offset correction reduces the offset with comparable efficacy as phantom measurement phase offset correction, without the time penalty imposed by phantom scans. Trial registration The study was registered in The Netherlands National Trial Register (NTR) under TC 4865 . Registered 19 September 2014. Retrospectively registered.

Aims Having a child with congenital heart disease (CHD) can affect parental health-related quality of life (HR-QoL). We investigated the long-term trajectories of mental HRQoL (m-HRQoL) in mothers of children with CHD and examined risk factors for persistent low m-HRQoL. Methods One hundred twenty-five mothers of children with CHD completed a standardized questionnaire on m-HRQoL (mental subscale SF-12) after the children’s first open-heart surgery and subsequently when the children were 1, 4, 6, 10, and 13 years old. A z-score for m-HRQoL was calculated with national norms. Latent class growth analysis (LCGA) was used to identify subgroups of mothers with regards to their m-HRQoL trajectories over time. Regression analysis investigated predictors for chronically low m-HRQoL. Results Compared to norms, mothers of children with CHD had significantly lower m-HRQoL immediately after open-heart surgery ( β  = −0.30 ( CI-95: −0.44, −0.15)). Subsequently, m-HRQoL increased to a normal level (m-HRQoL compared to the norm from 1 to 13 years: β ranges between 0.05 and 0.27). LCGA revealed two distinct groups of m-HRQoL trajectories: A group with normal m-HRQoL (75% of mothers, means z-scores range between − 0.76 and 0.62) and a group with chronically low m-HRQoL (25% of mothers, mean z-scores range between −1.32 and −0.10). Chronically, low m-HRQoL was associated with poorer social support ( OR  = 3.39 ( CI-95: 1.40, 8.49), p  = 0.008) but not with parental education, migration background, number of open-heart surgeries, diagnosis of a univentricular CHD, or low IQ. Conclusion A quarter of mothers of children with CHD have chronically low m-HRQoL throughout their child’s development, especially those mothers with poor social support. Further studies of family-oriented approaches are needed to identify and support these mothers and reinforce parental well-being.

Cardiovascular magnetic resonance (CMR) has taken on an increasingly important role in the diagnostic evaluation and pre-procedural planning for patients with congenital heart disease. This article provides guidelines for the performance of CMR in children and adults with congenital heart disease. The first portion addresses preparation for the examination and safety issues, the second describes the primary techniques used in an examination, and the third provides disease-specific protocols. Variations in practice are highlighted and expert consensus recommendations are provided. Indications and appropriate use criteria for CMR examination are not specifically addressed.

Objectives 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. Methods 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 (EL v ) were calculated. Results 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 EL v . Conclusions 4D-flow MRI pathline tracking reveals disordered ventricular flow patterns and associated EL v in d-TGA patients. Key Points • 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.

Background and AimFontan 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.MethodsCardiovascular 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).ResultsDaily 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.ConclusionOnly 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.

We sought to establish normal values for the diameters of the main (MPA), right (RPA), and left (LPA) pulmonary arteries and for the angles describing the geometry of the pulmonary artery bifurcation in children by using contrast-enhanced magnetic resonance angiography (CE-MRA). CE-MRA was performed in 69 children without cardiovascular disease. The median age was 10 ± 4.9 years (range 2–20), weight 37.4 ± 18.5 kg (10–82), body surface area (BSA) 1.18 ± 0.4 m 2 (0.48–2.07). The pulmonary artery diameters and angles were measured at standardized sites and projections. Regression analysis of diameters and angles in relation to BSA demonstrated linear relationship between the cross-sectional diameters of the pulmonary arteries and the square root of BSA (BSA 0.5 ). Normalized mean diameters were for the MPA 17.6 ± 5.1 mm/m 2 , origin of RPA 13.1 ± 2.9 mm/m 2 , origin of LPA 14.2 ± 2.9 mm/m 2 . The MPA showed a mean antero-posterior inclination of 33° ± 8° and a lateral leftward angulation of 18° ± 5°. The mean angle of the bifurcation was 99.5° ± 10.3°. Both side branches showed a supero-inferior course of the proximal segments, steeper for the RPA (7.7° ± 6.5°) than for the LPA (2.1° ± 7.8°). Normative curves in relation to BSA are presented for all measurements. This study provides normative values by CE-MRA for the main pulmonary artery and its side branches in children during somatic growth. These data can be used for identifying pulmonary arteries anomalies in children, and evaluate the need and the modality for treatment.

We sought to evaluate the impact of prenatal diagnosis on morbidity and mortality in single ventricle (SV) lesions. All consecutive patients with pre- or postnatally diagnosed SV physiology admitted to our centre between January 2001 and June 2013 were reviewed. Primary endpoints included survival until 30 days after bidirectional cavopulmonary connection (BCPC) without transplant or BCPC takedown. Prenatal diagnosis was performed in 160 of 259 cases (62%). After excluding all cases with termination of pregnancy, intrauterine demise or treated with comfort care, a total of 180 neonates were admitted to our centre for treatment, including 87 with a prenatal and 93 with a postnatal diagnosis. Both groups showed similar distribution regarding diagnosis, dominant ventricle and risk factors such as restrictive foramen or some form of atrial isomerism. A larger proportion of postnatally diagnosed children presented at admission with elevated lactate > 10 mmol/l (p = 0.02), a higher dose of prostaglandin (p = 0.0013) and need for mechanical ventilation (p < 0.0001). Critical lesions such as hypoplastic left heart syndrome were an important determinant for morbidity and mortality. Thirty-days survival after BCPC was better in patients with prenatal diagnosis (p = 0.025). Prenatal diagnosis is associated with higher survival in neonates with SV physiology.

Morphological and functional parameters such as chamber size and function, aortic diameters and distensibility, flow and T1 and T2* relaxation time can be assessed and quantified by cardiovascular magnetic resonance (CMR). Knowledge of normal values for quantitative CMR is crucial to interpretation of results and to distinguish normal from disease. In this review, we present normal reference values for morphological and functional CMR parameters of the cardiovascular system based on the peer-reviewed literature and current CMR techniques and sequences.