Accelerated cardiac magnetic resonance imaging using deep learning for volumetric assessment in children.

BACKGROUND: 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. OBJECTIVE: To assess the diagnostic performance of accelerated 3-RR cine MRI sequences using deep learning reconstruction compared with standard 2-D cine bSSFP sequences. MATERIAL AND METHODS: 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). RESULTS: 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). CONCLUSION: 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.

Multivendor Evaluation of Automated MRI Postprocessing of Biventricular Size and Function for Children With and Without Congenital Heart Defects.

BACKGROUND: Manually segmenting cardiac structures is time-consuming and produces variability in MRI assessments. Automated segmentation could solve this. However, current software is developed for adults without congenital heart defects (CHD). PURPOSE: To evaluate automated segmentation of left ventricle (LV) and right ventricle (RV) for pediatric MRI studies. STUDY TYPE: Retrospective comparative study. POPULATION: Twenty children per group of: healthy children, LV-CHD, tetralogy of Fallot (ToF), and univentricular CHD, aged 11.7 [8.9-16.0], 14.2 [10.6-15.7], 14.6 [11.6-16.4], and 12.2 [10.2-14.9] years, respectively. SEQUENCE/FIELD STRENGTH: Balanced steady-state free precession at 1.5 T. ASSESSMENT: Biventricular volumes and masses were calculated from a short-axis stack of images, which were segmented manually and using two fully automated software suites (Medis Suite 3.2, Medis, Leiden, the Netherlands and SuiteHeart 5.0, Neosoft LLC, Pewaukee, USA). Fully automated segmentations were manually adjusted to provide two further sets of segmentations. Fully automated and adjusted automated segmentation were compared to manual segmentation. Segmentation times and reproducibility for each method were assessed. STATISTICAL TESTS: Bland Altman analysis and intraclass correlation coefficients (ICC) were used to compare volumes and masses between methods. Postprocessing times were compared by paired t-tests. RESULTS: Fully automated methods provided good segmentation (ICC > 0.90 compared to manual segmentation) for the LV in the healthy and left-sided CHD groups (eg LV-EDV difference for healthy children 1.4 ± 11.5 mL, ICC: 0.97, for Medis and 3.0 ± 12.2 mL, ICC: 0.96 for SuiteHeart). Both automated methods gave larger errors (ICC: 0.62-0.94) for the RV in these populations, and for all structures in the ToF and univentricular CHD groups. Adjusted automated segmentation agreed well with manual segmentation (ICC: 0.71-1.00), improved reproducibility and reduced segmentation time in all patient groups, compared to manual segmentation. DATA CONCLUSION: Fully automated segmentation eliminates observer variability but may produce large errors compared to manual segmentation. Manual adjustments reduce these errors, improve reproducibility, and reduce postprocessing times compared to manual segmentation. Adjusted automated segmentation is reasonable in children with and without CHD. EVIDENCE LEVEL: 3. TECHNICAL EFFICACY: Stage 2.

Mental health-related quality of life in mothers of children with surgically repaired congenital heart disease: a 13-year longitudinal study.

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 (ß = -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: ß 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.

Feasibility of Non-Gated Dynamic Fetal Cardiac MRI for Identification of Fetal Cardiovascular Anatomy.

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.

Assessment of ventricular flow dynamics by 4D-flow MRI in patients following surgical repair of d-transposition of the great arteries.

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 (ELv) 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 ELv. CONCLUSIONS: 4D-flow MRI pathline tracking reveals disordered ventricular flow patterns and associated ELv 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.

Additional value and new insights by four-dimensional flow magnetic resonance imaging in congenital heart disease: application in neonates and young children.

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.

Flow quantification dependency on background phase correction techniques in 4D-flow MRI.

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.

In-vivo validation of interpolation-based phase offset correction in cardiovascular magnetic resonance flow quantification: a multi-vendor, multi-center study.

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.

Prenatal Diagnosis of Single Ventricle Physiology Impacts on Cardiac Morbidity and Mortality.

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.

Normal values for cardiovascular magnetic resonance in adults and children.

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.

Guidelines and protocols for cardiovascular magnetic resonance in children and adults with congenital heart disease: SCMR expert consensus group on congenital heart disease.

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.

Imaging the right heart: the use of integrated multimodality imaging.

During recent years, right ventricular (RV) structure and function have been found to be an important determinant of outcome in different cardiovascular and also pulmonary diseases. Currently, echocardiography and cardiac magnetic resonance (CMR) imaging are the two imaging modalities most commonly used to visualize the RV. Most structural abnormalities of the RV can be reliably described by echocardiography but due its complex geometrical shape, echocardiographic assessment of RV function is more challenging. Newer promising echocardiographic techniques are emerging but lack of validation and limited normal reference data influence their routine clinical application. Cardiac magnetic resonance is generally considered the clinical reference technique due to its unlimited imaging planes, superior image resolution, and three-dimensional volumetric rendering. The accuracy and reliability of CMR measurements make it the ideal tool for serial examinations of RV function. Multidetector computed tomography (MDCT) plays an important role in the diagnosis of pulmonary emboli but can also be used for assessing RV ischaemic disease or as an alternative for CMR if contra-indicated. Radionuclide techniques have become more obsolete in the current era. The different imaging modalities should be considered complimentary and each plays a role for different indications.

Management and outcome of Ebstein's anomaly in children.

OBJECTIVES: To assess clinical presentation, treatment, and outcome of children with Ebstein's anomaly. BACKGROUND: Data on long-term outcome of children with Ebstein's anomaly are scarce. METHODS: Retrospective analysis of all children with Ebstein's anomaly treated between February, 1979 and January, 2009 in a single tertiary institution. Primary outcomes included patient survival and need for intervention, either cardiac surgery or catheter intervention. RESULTS: A total of 42 patients were diagnosed with Ebstein's anomaly at a median age of 5 days ranging from 1 day to 11.7 years. Symptoms included cyanosis, heart murmur, and/or dyspnoea. Associated cardiac anomalies occurred in 90% of the patients. Average follow-up was 9.5 plus or minus 7.0 years. The overall mortality rate was 14%. Of the six patients, three died postnatally before treatment. Cardiac surgery and/or catheter-guided interventions were required in 33 patients (79%). Cardiac surgery was performed in 21 (50%) patients at a median age of 9.1 years (range 0.1-16.5 years), including biventricular repair in 13 (62%), one-and-a-half chamber repair in seven (33%), and a staged single-ventricle repair in one. Peri-operative mortality was 4%. Catheter-guided interventions consisted of device closure of an atrial septal defect in three cases and radiofrequency ablation of accessory pathways in nine patients. The estimated 10-year survival was 85.3 plus or minus 5.6%. CONCLUSION: In children, Ebstein's anomaly is usually diagnosed in the first year of age. Even though children with Ebstein's anomaly often require an intervention, their peri-operative mortality is low and long-term survival is good. Symptomatic newborns requiring an intervention may have a worse outcome.

Outcome of biventricular repair in infants with multiple left heart obstructive lesions.

The decision to perform biventricular repair for infants with multiple obstructive or hypoplastic left heart lesions (LHL) and borderline left ventricle (LV) may be controversial. This study sought to assess the mortality and morbidity of patients with LHL after biventricular repair and to determine the growth of the left-sided cardiac structures. Retrospective analysis of 39 consecutive infants with LHL who underwent biventricular repair was performed. The median age at surgery was 7 days (range 1-225 days), and the median follow-up period was 34 months (range 1-177 months). Between diagnosis and the end of the follow-up period, the size of the aortic annulus (z-score -4.1 ± 2.8 vs. -0.1 ± 2.7) and the LV (LV end-diastolic diameter z-score -1.7 ± 2.8 vs. 0.21 ± 1.7) normalized. During the follow-up period, 23 patients required 39 reinterventions (62%) consisting of redo surgery for 21 patients (57%) and catheter-guided reinterventions for 8 patients (22%). At the end of the follow-up period, 25 of 34 patients were doing subjectively well; 10 children (29%) received cardiac medication; 12 (35%) presented with failure to thrive (weight ≤ P3) and 5 (15%) with pulmonary hypertension. The overall mortality rate was 13%. Biventricular repair for patients with multiple LHL results in sufficient growth of the left-sided cardiac structures. Nevertheless, residual or newly developing obstructive lesions and pulmonary hypertension are frequent, causing significant morbidity that requires reintervention.

Physical Activity in Fontan Patients Relates to Quality of Life and Sleep Quality.

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.

Social and Behavioral Difficulties in 10-Year-Old Children With Congenital Heart Disease: Prevalence and Risk Factors.

Children with congenital heart disease (CHD) may be at increased risk for neurodevelopmental impairments. Long-term behavioral profiles and respective risk factors are less frequently described. The aim of this study was to evaluate multidimensional behavioral outcomes and associated medical, psychological, and social risk factors in children with complex CHD. At 10-years of age, 125 children with CHD were assessed for general behavioral difficulties, attention deficit hyperactivity disorder (ADHD)-related behavior, and social interaction problems and were compared to normative data. Medical and cardiac factors, IQ, maternal mental health at 4 years of age and parental socioeconomic status were tested as predictors for all behavioral outcomes. Children with CHD showed no significant differences in general behavioral difficulties. However, increased ADHD-related symptoms (p < 0.05) and difficulties in social interaction (p < 0.05) were observed. In 23% of the children, a combination of ADHD-related symptoms and social interaction problems was reported by parents. In multivariate analyses, IQ (p < 0.01) and maternal mental health (p < 0.03) at 4 years of age were found to be predictive for all behavioral outcomes at 10 years while medical and cardiac risk factors were not. Our findings reveal significant difficulties in ADHD-related symptoms and social interaction problems with a significant comorbidity. Behavioral difficulties were not detected with a screening tool but with disorder-specific questionnaires. Furthermore, we demonstrate the importance of maternal mental health during early childhood on later behavioral outcomes of children with CHD. This underlines the importance of identifying and supporting parents with mental health issues at an early stage in order to support the family and improve the child's neurodevelopment.

Right ventricular dilatation in patients with pulmonary regurgitation after repair of tetralogy of Fallot: How fast does it progress?

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.

Health-related quality of life in children and adolescents after open-heart surgery.

OBJECTIVE: To assess health-related quality of life (HRQOL) in children with congenital heart disease (CHD) after open-heart surgery and to identify medical, individual, and family-related predictors of outcome. STUDY DESIGN: Cross-sectional cohort study. 155 children with CHD (40% cyanotic type) who underwent open-heart surgery between 1995 and 1998 were eligible. One hundred and ten patients (response rate 71%) were investigated at a mean age of 10.4 years. Mean age at operation was 2.3 years (range, 0-8.7 years), mean duration of cardiopulmonary bypass was 95 minutes (range, 5-206 minutes), and circulatory arrest was performed in 9% of the children. Child- and parent-rated HRQOL was evaluated by the TNO-AZL Child Quality of Life Questionnaire. Scores were compared with healthy references. RESULTS: Most dimensions of self-reported HRQOL were impaired, including autonomy and motor, social, and emotional functioning. In addition to the children's ratings, parents rated cognitive functioning of their children as diminished. Multivariate analyses revealed that duration of cardiopulmonary bypass, length of hospitalization, need for current cardiac medication, and adverse family relationships had a negative impact on parent- but not on child-rated HRQOL. CONCLUSIONS: HRQOL is impaired in children with CHD after open-heart surgery. Medical variables and the quality of family relationships are important determinants.

Normal values for aortic diameters in children and adolescents--assessment in vivo by contrast-enhanced CMR-angiography.

BACKGROUND: Contrast-enhanced CMR angiography (CE-CMRA) is being increasingly used for diagnosing aortic arch anomalies, planning interventions and follow-up assessment. We sought to establish normal values for the diameters of the thoracic aorta and reference curves related to body growth in children using CE-CMRA. RESULTS: CE-CMRA was performed in 53 children without cardiovascular disease. The median age was 9 years (range 2 - 20 years), weight 30 kg (range 12 - 75 kg), height 131 cm (range 81 - 184 cm), body surface area (BSA) 1.05 m2 (range 0.52-1.9 m2). Aortic diameters were measured at nine standardized sites on oblique maximum-intensity projection (MIP) images. Regression analysis of diameters in relation to BSA demonstrated linear relationship between the cross-sectional aortic diameters and the square root of BSA (BSA0.5). Normative diameters were (0.57 + 19.37*BSA0.5) mm for the aortic sinus, (-3.52 + 18.66*BSA0.5) mm for the first segment of the aortic arch, (-3.37 + 16.52*BSA0.5) mm for the isthmic region and (-1.27 + 9.89*BSA0.5) mm for the descending aorta at the level of the diaphragm. Normative curves are presented. CONCLUSION: This study provides normative values for aortic diameters in children measured by CE-CMRA. These data may serve for making the diagnosis of pediatric arch anomalies, assessing the need for treatment and planning interventions.

Imaging the adult with congenital heart disease: a multimodality imaging approach-position paper from the EACVI.

Advances in the diagnosis and management of congenital heart disease have led to a marked improvement in the survival of adult with congenital heart disease (ACHD) patients. However, ACHD patients are a heterogeneous population, with a large spectrum of anatomic substrates even within specific lesions. In addition, the nature of previous surgery and other intervention is highly variable rendering each patient unique and residual anatomic and haemodynamic abnormalities are very common. As the ACHD population continues to age, acquired heart disease will also require cardiac imaging assessment. It is increasingly recognized in ACHD community that the diagnostic utility of a multimodality cardiovascular approach is greater than the sum of individual tests. In ACHD patients, diagnostic information can be obtained using a variety of diagnostic tools. The aims of this document are to describe the role of each diagnostic modality in the care of ACHD patients and to provide guidelines for a multimodality approach. The goal should be to provide the most appropriate and cost-effective diagnostic pathway for each individual ACHD patient.