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Cardiovascular magnetic resonance (CMR) has established itself as the gold standard for serial assessment of systemic right ventricular (RV) performance but due to the lack of standardized RV reference values for hypoplastic left heart syndrome (HLHS) patients, the interpretation of RV volumetric data in HLHS remains difficult. Therefore, this study aimed to close this gap by providing CMR reference values for the systemic RV in HLHS patients. CMR scans of 160 children, adolescents, and young adults (age range 2.2–25.2 years, 106 males) with HLHS were retrospectively evaluated. All patients were studied following total cavopulmonary connection. Short-axis stacks were used to measure RV end-diastolic and end-systolic volumes (RVEDV, RVESV), RV stroke volume (RVSV), RV ejection fraction (RVEF), and RV end-diastolic myocardial mass (RVEDMM). Univariable and multiple linear regression analyses were performed to assess associations between RV parameters and demographic and anthropometric characteristics. Following the results of the regression analysis, reference graphs and tables were created with the Lambda-Mu-Sigma method. Multiple linear regression analysis showed strong associations between body height and RVEDV, RVESV as well as RVSV. Age was highly associated with RVEDMM. Therefore, percentile curves and tables were created with respect to body height (RVEDV, RVESV, RVSV) and age (RVEDMM). The influence of demographic and anthropometric parameters on RVEF was mild, thus no percentile curves and tables for RVEF are provided. We were able to define CMR reference values for RV volumetric variables for HLHS patients. These data might be useful for the assessment and interpretation of CMR scans in these patients and for research in this field. [Display omitted]

Staged palliation with the creation of a Fontan circulation is the standard surgical approach in patients with a single ventricle. The Fontan circulation is a complex circuit that is associated with various complications that may present early or later in life and can limit life quality and expectancy. In this context, a good understanding of the Fontan physiology is important to improve outcomes for single-ventricle patients. Cardiovascular magnetic resonance (CMR) is recommended for the long-term follow-up of Fontan patients, as it provides functional and haemodynamic information. Four-dimensional (4D) Flow MRI is a time-resolved, three-dimensional, velocity-encoded cardiovascular magnetic resonance technique that is increasingly used in Fontan patients because it not only enables measuring blood flow within a three-dimensional (3D) volume, but also allows for assessing more advanced haemodynamic parameters that may help in understanding the Fontan physiology and pathophysiology. Furthermore, 4D Flow is used for image-based simulations using computational fluid dynamics. In this review, we provide an overview of the use of cardiovascular magnetic resonance flow assessment, with a focus on four-dimensional flow (‘4D Flow’).

ObjectiveTo test the hypothesis that ventricular and atrial function are different between patients with transposition of the great arteries (TGA) after arterial switch operation (ASO) and healthy controls.Methods103 consecutive patients with TGA (median age: 16.7 years, 4.3–39.6 years, 71.8% male) were compared with 77 controls (median age: 15.4 years, 6.3–43.2 years, 66.2% male). Biventricular and biatrial function were assessed using standard cardiovascular magnetic resonance (CMR) techniques and feature tracking. Group comparison was performed with conventional non-parametrical statistics and machine learning methods to find the variables most discriminative between patients and controls. These variables were used to build a multivariable logistic regression model to assess the case–control status.ResultsMarkers of left and right ventricular function (LV; RV) (ejection fraction, MAPSE, TAPSE, LV long-axis strain) as well as LV global longitudinal (−20.7 (−24.1; −17.9) vs −23.7 (−26.1; −21.6), p<0.001), circumferential (−29.4 (−32.2; −26.5) vs −30.5 (−33.6; 29), p=0.001) and atrial longitudinal strain (left atrium (LA): 23.3 (18.6; 28.8) vs 36.7 (30.7; 44), p<0001; right atrium: 21.7 (18.2; 27.8) vs 34.9 (26.9; 40.3), p<0.001) were reduced in patients compared with controls using non-parametrical testing. The logistic regression model including the most discriminative variables from univariate and machine learning analysis demonstrated significant differences between patients and controls only for TAPSE and LA global longitudinal strain.ConclusionsBiventricular and biatrial function are largely preserved after ASO for TGA. Using a comprehensive CMR protocol along with statistical machine learning methods and a regression approach, only RV longitudinal function and LA function are significantly impaired.

The number of patients with congenital heart disease (CHD) is increasing worldwide and most of them will require cardiac surgery, once or more, during their lifetime. The total volume of cardiac surgery in CHD patients at a national level and the associated mortality and predictors of death associated with surgery are not known. We aimed to investigate the surgical volume and associated mortality in CHD patients in England. Using a national hospital episode statistics database, we identified all CHD patients undergoing cardiac surgery in England between 1997 and 2015. We evaluated 57,293 patients (median age 11.9years, 46.7% being adult, 56.7% female). There was a linear increase in the number of operations performed per year from 1,717 in 1997 to 5,299 performed in 2014. The most common intervention at the last surgical event was an aortic valve procedure (9,276; 16.2%), followed by repair of atrial septal defect (9,154; 16.0%), ventricular septal defect (7,746; 13.5%), tetralogy of Fallot (3,523; 6.1%) and atrioventricular septal defect (3,330; 5.8%) repair. Associated mortality remained raised up to six months following cardiac surgery. Several parameters were predictive of post-operative mortality, including age, complexity of surgery, need for emergency surgery and socioeconomic status. The relationship of age with mortality was \"U\"-shaped, and mortality was highest amongst youngest children and adults above 60 years of age. The number of cardiac operations performed in CHD patients in England has been increasing, particularly in adults. Mortality remains raised up to 6-months after surgery and was highest amongst young children and seniors.

ObjectivesPatients with Ebstein’s anomaly of the tricuspid valve (EA) are at risk of tachyarrhythmia, congestive heart failure and sudden cardiac death. We sought to determine the value of cardiovascular magnetic resonance (CMR) for predicting these outcomes.MethodsSeventy-nine consecutive adult patients (aged 37±15 years) with unrepaired EA underwent CMR and were followed prospectively for a median 3.4 (range 0.4–10.9) years for clinical outcomes, namely major adverse cardiovascular events (MACEs: sustained ventricular tachycardia/heart failure hospital admission/cardiac transplantation/death) and first-onset atrial tachyarrhythmia (AT).ResultsCMR-derived variables associated with MACE (n=6) were right ventricular (RV) or left ventricular (LV) ejection fraction (EF) (HR 2.06, 95% CI 1.168 to 3.623, p=0.012 and HR 2.35, 95% CI 1.348 to 4.082, p=0.003, respectively), LV stroke volume index (HR 2.82, 95% CI 1.212 to 7.092, p=0.028) and cardiac index (HR 1.71, 95% CI 1.002 to 1.366, p=0.037); all remained significant when tested solely for mortality. History of AT (HR 11.16, 95% CI 1.30 to 95.81, p=0.028) and New York Heart Association class >2 (HR 7.66, 95% CI 1.54 to 38.20, p=0.013) were also associated with MACE; AT preceded all but one MACE, suggesting its potential role as an early marker of adverse outcome (p=0.011).CMR variables associated with first-onset AT (n=17; 21.5%) included RVEF (HR 1.55, 95% CI 1.103 to 2.160, p=0.011), total R/L volume index (HR 1.18, 95% CI 1.06 to 1.32, p=0.002), RV/LV end diastolic volume ratio (HR 1.55, 95% CI 1.14 to 2.10, p=0.005) and apical septal leaflet displacement/total LV septal length (HR 1.03, 95% CI 1.00 to 1.07, p=0.041); the latter two combined enhanced risk prediction (HR 6.12, 95% CI 1.67 to 22.56, p=0.007).ConclusionCMR-derived indices carry prognostic information regarding MACE and first-onset AT among adults with unrepaired EA. CMR may be included in the periodic surveillance of these patients.

Background Single ventricle (SV) Fontan patients are at risk for the development of pathological cervicothoracic lymphatic drainage pathways that are involved in the development of serious conditions such as plastic bronchitis or chylothorax. Visualization and categorization of cervicothoracic lymphatic drainage pathways might therefore help to stratify prognosis and to individualize therapy and follow-up for Fontan patients. This study aimed to show that the 3-dimensional (3D) modified Dixon (mDixon) steady state magnetic resonance (MR) angiography, commonly used to image cardiovascular anatomy, can visualize cervicothoracic lymphatic drainage pathways in high resolution in Fontan patients. Methods 3D mDixon steady state MR angiography of 88 pediatric and young adult patients with a Fontan circulation were retrospectively analysed. The pattern of cervicothoracic lymphatic pathways and image quality according to diagnostic value were assessed. Furthermore, ventricular volumes, mass and ejection fraction from cine imaging were measured. Results Image quality was assessed as very good or good in > 90% of the cases. Six patients had a lymphatic complication of which five (83.3%) had a higher cervicothoracic lymphatic pathway type (type 3 or 4). Conclusions 3D mDixon steady state MR angiography is an established method to assess cardiovascular anatomy and function in Fontan patients. The method also allows to visualize and evaluate the cervicothoracic lymphatic anatomy with high image quality. 3D mDixon steady state MR angiography is therefore particularly useful to comprehensively assess Fontan patients, a patient group prone not only to cardiac but also lymphatic failure.

ObjectivePulmonary valve replacement (PVR) is often required in patients with congenital heart disease. We aimed to describe temporal trends in PVR in a nationwide English cohort between 1997 and 2014, survival and the need for re-PVR.MethodsPatients were identified in the Hospital Episode Statistics Database. Survival data were retrieved from the UK Office for National Statistics.ResultsA total of 2733 patients underwent PVR (2845 procedures) over the study period. Median age at first procedure increased from 20.1 years in 1997–2005 to 24.7 years in 2006–2014. The annual number of PVRs increased from 23 in 1997 to 251 in 2014. Homografts were the most common choice in the early years, but the use of xenografts increased after 2005. During a median follow-up of 5.8 years, 176 patients died and 108 required redo PVR. Early (30 day) survival was 98% for all PVRs and was similar for all types of prostheses but longer-term mortality dropped to 92% at 10 years and 90% at 15 years. Age >16 years and percutaneous PVR were risk factors for death. The cumulative incidence for re-PVR at 10 years was 8% for all PVRs and 11% at 15 years. Risk factors for re-PVR were complex diagnosis, male gender and black ethnicity.ConclusionThere was a significant increase in the number of PVRs performed in England over the last two decades and a significant change in the type of prosthesis employed. While early mortality was low across the board, longer-term mortality was not negligible in this young population.

Rare genetic variants in KDR, encoding the vascular endothelial growth factor receptor 2 (VEGFR2), have been reported in patients with tetralogy of Fallot (TOF). However, their role in disease causality and pathogenesis remains unclear. We conducted exome sequencing in a familial case of TOF and large-scale genetic studies, including burden testing, in >1,500 patients with TOF. We studied gene-targeted mice and conducted cell-based assays to explore the role of KDR genetic variation in the etiology of TOF. Exome sequencing in a family with two siblings affected by TOF revealed biallelic missense variants in KDR. Studies in knock-in mice and in HEK 293T cells identified embryonic lethality for one variant when occurring in the homozygous state, and a significantly reduced VEGFR2 phosphorylation for both variants. Rare variant burden analysis conducted in a set of 1,569 patients of European descent with TOF identified a 46-fold enrichment of protein-truncating variants (PTVs) in TOF cases compared to controls (P = 7 × 10-11). Rare KDR variants, in particular PTVs, strongly associate with TOF, likely in the setting of different inheritance patterns. Supported by genetic and in vivo and in vitro functional analysis, we propose loss-of-function of VEGFR2 as one of the mechanisms involved in the pathogenesis of TOF.

Ventricular dysfunction is a well-known complication in single ventricle patients in Fontan circulation. As studies exclusively examining patients with a single left ventricle (SLV) are sparse, we assessed left ventricular (LV) function in SLV patients by using 2D-cardiovascular magnetic resonance (CMR) feature tracking (2D-CMR-FT) and 2D-speckle tracking echocardiography (2D-STE). 54 SLV patients (11.4, 3.1–38.1 years) and 35 age-matched controls (12.3, 6.3–25.8 years) were included. LV global longitudinal, circumferential and radial strain (GLS, GCS, GRS) and strain rate (GLSR, GCSR, GRSR) were measured using 2D-CMR-FT. LV volumes, ejection fraction (LVEF) and mass were determined from short axis images. 2D-STE was applied in patients to measure peak systolic GLS and GLSR. In a subgroup analysis, we compared double inlet left ventricle (DILV) with tricuspid atresia (TA) patients. The population consisted of 19 DILV patients, 24 TA patients and 11 patients with diverse diagnoses. 52 patients were in NYHA class I and 2 patients were in class II. Most SLV patients had a normal systolic function but median LVEF in patients was lower compared to controls (55.6% vs. 61.2%, p = 0.0001). 2D-CMR-FT demonstrated reduced GLS, GCS and GCSR values in patients compared to controls. LVEF correlated with GS values in patients (p < 0.05). There was no significant difference between GLS values from 2D-CMR-FT and 2D-STE in the patient group. LVEF, LV volumes, GS and GSR (from 2D-CMR-FT) were not significantly different between DILV and TA patients. Although most SLV patients had a preserved EF derived by CMR, our results suggest that, LV deformation and function may behave differently in SLV patients compared to healthy subjects.

Background Pediatric patients are becoming increasingly referred for cardiovascular magnetic resonance (CMR). Measurement of ventricular wall thickness is typically part of the assessment and can be of diagnostic importance, e.g. in arterial hypertension. However, normal values for left ventricular (LV) and right ventricular (RV) wall thickness in pediatric patients are lacking. The aim of this study was to establish pediatric centile charts for segmental LV and RV myocardial thickness in a retrospective multicenter CMR study. Methods CMR was performed in 161 healthy children and adolescents with an age range between 6 and 18 years from two centers in the UK and Germany as well as from a previously published CMR project of the German Competence Network for Congenital Heart Defects. LV myocardial thickness of 16 segments was measured on the short axis stack using the American Heart Association segmentation model. In addition, the thickness of the RV inferior and anterior free wall as well as biventricular mass was measured. Results The mean age (standard deviation) of the subjects was 13.6 (2.9) years, 64 (39.7%) were female. Myocardial thickness of the basal septum (basal antero- and inferoseptal wall) was 5.2 (1.1) mm, and the basal lateral wall (basal antero- and inferolateral) measured 5.1 (1.2) mm. Mid-ventricular septum (antero- and inferoseptal wall) measured 5.5 (1.2) mm, and mid-ventricular lateral wall (antero- and inferolateral wall) was 4.7 (1.2) mm. Separate centile charts for boys and girls for all myocardial segments and myocardial mass were created because gender was significantly correlated with LV myocardial thickness (p < 0.001 at basal level, p = 0.001 at midventricular level and p = 0.005 at the apex) and biventricular mass (LV, p < 0.001; RV, p < 0.001). Conclusion We established CMR normal values of segmental myocardial thickness and biventricular mass in children and adolescents. Our data are of use for the detection of abnormal myocardial properties and can serve as a reference in future studies and clinical practice.