Cardiovascular magnetic resonance reference values of right ventricular volumetric variables in patients with hypoplastic left heart syndrome.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Detection of hypoplastic left heart syndrome anatomy from cardiovascular magnetic resonance images using machine learning.

OBJECTIVE: The prospect of being able to gain relevant information from cardiovascular magnetic resonance (CMR) image analysis automatically opens up new potential to assist the evaluating physician. For machine-learning-based classification of complex congenital heart disease, only few studies have used CMR. MATERIALS AND METHODS: This study presents a tailor-made neural network architecture for detection of 7 distinctive anatomic landmarks in CMR images of patients with hypoplastic left heart syndrome (HLHS) in Fontan circulation or healthy controls and demonstrates the potential of the spatial arrangement of the landmarks to identify HLHS. The method was applied to the axial SSFP CMR scans of 46 patients with HLHS and 33 healthy controls. RESULTS: The displacement between predicted and annotated landmark had a standard deviation of 8-17 mm and was larger than the interobserver variability by a factor of 1.1-2.0. A high overall classification accuracy of 98.7% was achieved. DISCUSSION: Decoupling the identification of clinically meaningful anatomic landmarks from the actual classification improved transparency of classification results. Information from such automated analysis could be used to quickly jump to anatomic positions and guide the physician more efficiently through the analysis depending on the detected condition, which may ultimately improve work flow and save analysis time.

Pediatric Cardiac Magnetic Resonance Reference Values for Biventricular Volumes Derived From Different Contouring Techniques.

BACKGROUND: Measurement of ventricular volumes and function using MRI is an important tool in pediatric congenital heart disease. However, normal values for children are sparce and analysis methods are inconsistent. PURPOSE: To propose biventricular reference values in children for two MRI postprocessing (contouring) techniques. STUDY TYPE: Retrospective. SUBJECTS: A total of 154 children from two institutions (13.9 ± 2.8 years; 101 male) that were referred for a clinical MRI study. FIELD STRENGTH/SEQUENCE: 1.5 T; balanced steady-state free precession (bSSFP) sequence. ASSESSMENT: Left ventricular (LV) and right ventricular (RV) end-diastolic and end-systolic volumes (LVEDV, LVESV, RVEDV, RVESV) and end-diastolic and end-systolic myocardial mass (LVEDMM, LVESMM, RVEDMM, RVESMM) were measured from short-axis images using two contouring techniques: 1) papillary muscles, trabeculations and the moderator band were included in the ventricular blood volume and excluded from the myocardial mass, 2) papillary muscles, trabeculations and the moderator band were excluded from the ventricular volume and included in the ventricular mass. STATISTICAL TESTS: Univariable and multivariable linear regression models were used to evaluate relationships between sex, weight, height, body surface area (BSA) and age and volumetric results. Reference graphs and tables were created with the LMS-method. Contouring techniques were compared by intraclass correlation, regression analysis and Bland-Altman plots. A P value < 0.05 was considered statistically significant. RESULTS: Height and BSA were significantly associated with LVESV (method 1) and with LVEDV and RVEDV (method 2). LVESV (method 2), RVESV (both methods), RVEDV (method 1), and LVEDMM and RVEDMM (both methods), showed significant associations with height and weight. LVSV and RVSV (both methods) were significantly associated with BSA and weight. RVESV (method 1) was significantly associated with age. Gender showed significant associations for all parameters. DATA CONCLUSION: The proposed pediatric reference values can be used in the diagnosis and follow-up of congenital or acquired heart disease and for research purposes. EVIDENCE LEVEL: 3 TECHNICAL EFFICACY: Stage 2.

Serial Assessment of Right Ventricular Deformation in Patients With Hypoplastic Left Heart Syndrome: A Cardiovascular Magnetic Resonance Feature Tracking Study.

Background As right ventricular dysfunction is a major cause of adverse outcome in patients with hypoplastic left heart syndrome, the aim was to assess right ventricular function and deformation after Fontan completion by performing 2-dimensional cardiovascular magnetic resonance feature tracking in serial cardiovascular magnetic resonance studies. Methods and Results Cardiovascular magnetic resonance examinations of 108 patients with hypoplastic left heart syndrome (female: 31) were analyzed. Short-axis cine images were used for right ventricular volumetry. Two-dimensional cardiovascular magnetic resonance feature tracking was performed using long-axis and short-axis cine images to measure myocardial global longitudinal, circumferential, and radial strain. All patients had at least 2 cardiovascular magnetic resonance examinations after Fontan completion and 41 patients had 3 examinations. Global strain values and right ventricular ejection fraction decreased from the first to the third examination with a significant decline in global longitudinal strain from the first examination to the second examination (median, first, and third quartile: -18.8%, [-20.5;-16.5] versus -16.9%, [-19.3;-14.7]) and from the first to the third examination in 41 patients (-18.6%, [-20.9;-15.7] versus -15.8%, [-18.7;-12.6]; P-values <0.004). Right ventricular ejection fraction decreased significantly from the first to the third examination (55.4%, [49.8;59.3] versus 50.2%, [45.0;55.9]; P<0.002) and from the second to the third examination (53.8%, [47.2;58.7] versus 50.2%, [45.0;55.9]; P<0.0002). Conclusions Serial assessment of cardiovascular magnetic resonance studies in patients with hypoplastic left heart syndrome after Fontan completion demonstrates a significant reduction in global strain values and right ventricular ejection fraction at follow-up. The significant reduction in global longitudinal strain between the first 2 examinations with non-significant changes in right ventricular ejection fraction suggest that global longitudinal strain measured by 2-dimensional cardiovascular magnetic resonance feature tracking might be a superior technique for the detection of changes in myocardial function.

Comprehensive analysis of haemodynamics in patients with physiologically curved prostheses of the ascending aorta.

OBJECTIVES: This is a comprehensive analysis of haemodynamics after valve-sparing aortic root replacement (VSARR) with anatomically curved prosthesis (CP) compared to straight prosthesis (SP) and age-matched volunteers (VOL) using 4D flow MRI (time-resolved three-dimensional magnetic resonance phase-contrast imaging). METHODS: Nine patients with 90° CP, nine patients with SP, and twelve VOL were examined with 4D flow MRI. Analyses included various characteristic anatomical, qualitative and quantitative haemodynamic parameters. RESULTS: Grading of secondary flow patterns was lower in CP patients than in SP patients (P = 0.09) and more comparable to VOL, albeit not reaching statistical significance. However, it was easy to differentiate between VSARR patients and healthy volunteers: Patients more often had angular aortic arches (CP: 89%, SP: 100%; VOL: 17%; P ≤ 0.002), increased average curvature (CP: 0.17/cm [0.15, 0.18]; SP: 0.15/cm [0.14, 0.16]; VOL: 0.14/cm [0.13, 0.16]; P ≤ 0.007; values given as median [interquartile range]), and more secondary flow patterns (CP: 3 [2, 4] SP: 3 [2, 3] VOL: 2 [1, 2]; P < 0.01). Maximum circulation (CP: 142.7 cm2/s [116.1, 187.3]; SP: 101.8 cm2/s [77.7, 132.5]; VOL: 42.8cm2/s [39.3, 65.6]; P ≤ 0.002), maximum helicity density (CP: 9.6 m/s2 [9.3, 23.9]; SP: 9.7 m/s2 [8.6, 12.5]; VOL 4.9 m/s2 [4.2, 7.7]; P ≤ 0.007), and wall shear stress gradient (e.g., proximal ascending aorta CP: 0.97 N/m2 [0.54, 1.07]; SP: 1.08 N/m2 [0.74, 1.24]; VOL: 0.41 N/m2 [0.32, 0.60]; P ≤ 0.01) were increased in patients. One CP patient had a round aortic arch with physiological haemodynamic parameters. CONCLUSIONS: The restoration of physiological aortic configuration and haemodynamics was not fully achieved with the curved prostheses in our study cohort. However, there was a tendency towards improved haemodynamic conditions in the patients with curved prostheses overall but without statistical significance. A single patient with a CP and near-physiological configuration of the thoracic aorta underlines the importance of optimizing postoperative geometric conditions for allowing for physiological haemodynamics and cardiovascular energetics after VSARR.

Myocardial Perfusion in Hypoplastic Left Heart Syndrome.

BACKGROUND: The status of the systemic right ventricular coronary microcirculation in hypoplastic left heart syndrome (HLHS) is largely unknown. It is presumed that the systemic right ventricle's coronary microcirculation exhibits unique pathophysiological characteristics of HLHS in Fontan circulation. The present study sought to quantify myocardial blood flow by cardiac magnetic resonance imaging and evaluate the determinants of microvascular coronary dysfunction and myocardial ischemia in HLHS. METHODS: One hundred nineteen HLHS patients (median age, 4.80 years) and 34 healthy volunteers (median age, 5.50 years) underwent follow-up cardiac magnetic resonance imaging ≈1.8 years after total cavopulmonary connection. Right ventricle volumes and function, myocardial perfusion, diffuse fibrosis, and late gadolinium enhancement were assessed in 4 anatomic HLHS subtypes. Myocardial blood flow (MBF) was quantified at rest and during adenosine-induced hyperemia. Coronary conductance was estimated from MBF at rest and catheter-based measurements of mean aortic pressure (n=99). RESULTS: Hyperemic MBF in the systemic ventricle was lower in HLHS compared with controls (1.89±0.57 versus 2.70±0.84 mL/g per min; P<0.001), while MBF at rest normalized by the rate-pressure product, was similar (1.25±0.36 versus 1.19±0.33; P=0.446). Independent risk factors for a reduced hyperemic MBF were an HLHS subtype with mitral stenosis and aortic atresia (P=0.017), late gadolinium enhancement (P=0.042), right ventricular diastolic dysfunction (P=0.005), and increasing age at total cavopulmonary connection (P=0.022). The coronary conductance correlated negatively with systemic blood oxygen saturation (r, -0.29; P=0.02). The frequency of late gadolinium enhancement increased with age at total cavopulmonary connection (P=0.014). CONCLUSIONS: The coronary microcirculation of the systemic ventricle in young HLHS patients shows significant differences compared with controls. These hypothesis-generating findings on HLHS-specific risk factors for microvascular dysfunction suggest a potential benefit from early relief of frank cyanosis by total cavopulmonary connection.

Ventricular and atrial function and deformation is largely preserved after arterial switch operation.

OBJECTIVE: To 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. METHODS: 103 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. RESULTS: Markers 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. CONCLUSIONS: Biventricular 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.

Serial right ventricular assessment in patients with hypoplastic left heart syndrome: a multiparametric cardiovascular magnetic resonance study.

OBJECTIVES: The aim of this study was to assess single right ventricular (RV) function in a large cohort of hypoplastic left heart syndrome (HLHS) patients after the completion of total cavopulmonary connection by analysing serial cardiovascular magnetic resonance (CMR) studies. METHODS: CMR studies from 95 HLHS patients were analysed. RV end-diastolic and end-systolic volumes (RVEDV, RVESV), ejection fraction (RVEF) and long-axis strain (LAS) were measured from cine images. RESULTS: All 95 patients had at least 2 CMR scans and 35 patients had 3 CMR scans. The median age (first quartile-third quartile) at the 3 examinations was 4.2 (3.3-6.1), 9.4 (6.1-11.4) and 14.6 (11.8-16.8) years. RV indexed volumes (RVEDVi and RVESVi) increased from first to the second and from the first and second examination to the third examination in patients with >10 years of age (P < 0.05). There was a slight decrease in RVEF and LAS throughout the examinations, but this was not statistically significant. Correlations were found between RVEF and LAS (r = -0.23; P < 0.01). Both RVEF and LAS correlated with RVEDVi and RVESVi (r = -0.17 to 0.43; P < 0.05). CONCLUSIONS: Serial assessment of CMR studies in HLHS patients after total cavopulmonary connection completion demonstrate an increase in indexed RV volumes in older HLHS patients but only mild reduction in RVEF and LAS. The correlation of indexed RV volumes with RVEF and LAS together with the significant increase in RV volumes over time suggests that indexed RV volumes might be superior to RV functional markers to monitor the RV in HLHS patients.

Myocardial deformation in patients with a single left ventricle using 2D cardiovascular magnetic resonance feature tracking: a case-control study.

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.

Reference Values for Ventricular Volumes and Pulmonary Artery Dimensions in Pediatric Patients with Transposition of the Great Arteries After Arterial Switch Operation.

BACKGROUND: Pulmonary artery (PA) anatomy in patients with transposition of the great arteries (TGA) after arterial switch operation (ASO) with Lecompte manoeuvre is different compared to healthy subjects, and stenoses of the PA are common. Magnetic resonance imaging (MRI) is an excellent imaging modality to assess PA anatomy in TGA patients. However, disease-specific reference values for PA size are scarce. PURPOSE: To establish disease-specific reference ranges for PA dimensions and for biventricular volumes and mass. STUDY TYPE: Retrospective. SUBJECTS: A total of 69 pediatric patients with TGA after ASO (median age 12.6 years; range 5-17.8 years; 13 females and 56 males). FIELD STRENGTH/SEQUENCE: 3.0 T, steady-state free precession (SSFP) and gradient echo cine sequences and four-dimensional time-resolved magnetic resonance angiography with keyhole. ASSESSMENT: Right and left PA (RPA, LPA) were each measured at three locations during its course around the aorta. Ventricular volumes, mass, and ejection fraction were measured from a stack of short axis cine images. STATISTICAL TESTS: The lambda-mu-sigma (LMS) method of Cole and Green, univariate and multivariate linear models, and t-test. RESULTS: Centile graphs and tables for PA dimensions, biventricular volumes, mass, and ejection fraction were created. Univariate linear analysis showed significant associations (P < 0.05) between body surface area (BSA), height, and weight with systolic MPA and RPA diameter. In multivariate linear analysis, only BSA remained a strong predictor for main PA and RPA diameters. For biventricular volumes, the univariate linear model revealed a strong influence of BSA, height, weight, and age (all P < 0.05). On multivariate linear analysis, only body height remained associated. DATA CONCLUSION: Uni- and multivariate linear analyses showed a strong association between BSA and PA diameters, as well as between height and biventricular volumes, and therefore, centile tables and graphs are presented accordingly. Our data may improve MR image interpretation and may serve as a reference in future studies. LEVEL OF EVIDENCE: 4 TECHNICAL EFFICACY STAGE: 2.

3D-printed, patient-specific intracranial aneurysm models: From clinical data to flow experiments with endovascular devices.

PURPOSE: Flow models of intracranial aneurysms (IAs) can be used to test new and existing endovascular treatments with flow modulation devices (FMDs). Additionally, 4D flow magnetic resonance imaging (MRI) offers the ability to measure hemodynamics. This way, the effect of FMDs can be determined noninvasively and compared to patient data. Here, we describe a cost-effective method for producing flow models to test the efficiency of FMDs with 4D flow MRI. METHODS: The models were based on human radiological data (internal carotid and basilar arteries) and printed in 3D with stereolithography. The models were printed with three different printing layers (25, 50, and 100 µm thickness). To evaluate the models in vitro, 3D rotational angiography, time-of-flight MRI, and 4D flow MRI were employed. The flow and geometry of one model were compared with in vivo data. Two FMDs (FMD1 and FMD2) were deployed into two different IA models, and the effect on the flow was estimated by 4D flow MRI. RESULTS: Models printed with different layer thicknesses exhibited similar flow and little geometric variation. The mean spatial difference between the vessel geometry measured in vivo and in vitro was 0.7 ± 1.1 mm. The main flow features, such as vortices in the IAs, were reproduced. The velocities in the aneurysms were similar in vivo and in vitro (mean velocity magnitude: 5.4 ± 7.6 and 7.7 ± 8.6 cm/s, maximum velocity magnitude: 72.5 and 55.1 cm/s). By deploying FMDs, the mean velocity was reduced in the IAs (from 8.3 ± 10 to 4.3 ± 9.32 cm/s for FMD1 and 9.9 ± 12.1 to 2.1 ± 5.6 cm/s for FMD2). CONCLUSIONS: The presented method allows to produce neurovascular models in approx. 15 to 30 h. The resulting models were found to be geometrically accurate, reproducing the main flow patterns, and suitable for implanting FMDs as well as 4D flow MRI.

MRI-based comprehensive analysis of vascular anatomy and hemodynamics.

BACKGROUND: Standardized methods for mapping the complex blood flow in vessels are essential for processing the large data volume acquired from 4D Flow MRI. We present a method for systematic and efficient analysis of anatomy and flow in large human blood vessels. To attain the best outcomes in cardiac surgery, vascular modifications that lead to secondary flow patterns such as vortices should be avoided. In this work, attention was paid to the undesired cancelation of vortices with opposite directions of rotation, known as Dean flow patterns, using hemodynamic parameters such as circulation and helicity density. METHODS: Our approach is based on the multiplanar reconstruction (MPR) of a multi-dimensional feature-space along the blood vessel's centerline. Hemodynamic parameters and anatomic information were determined in-plane from the reconstructed feature-space and from the blood vessel's centerline. A modified calculation of circulation and helicity density and novel parameters for quantifying Dean flow were developed. To test the model performance, we applied our methods to three test cases. RESULTS: Comprehensive information on position, magnitude and interrelation of vascular anatomy and hemodynamics were extracted from 4D Flow MRI datasets. The results show that the Dean flow patterns can be efficiently assessed using the novel parameters. CONCLUSIONS: Our approach to comprehensively and simultaneously quantify multiple parameters of vascular anatomy and hemodynamics from 4D Flow MRI provides new insights to map complex hemodynamic conditions.

Abnormal torsion and helical flow patterns of the neo-aorta in hypoplastic left heart syndrome assessed with 4D-flow MRI.

BACKGROUND: The Norwood procedure is the first stage of correction for patients with hypoplastic left heart syndrome (HLHS) and may lead to an abnormal neoaortic anatomy. We prospectively studied the neoaorta's fluid dynamics and the abnormal twist of the neoaorta by MRI examinations of HLHS patients in Fontan circulation. This study for the first time investigates the hypothesis that the neoaorta twist is associated with increased helical flow patterns, which may lead to an increased workload for the systemic right ventricle (RV) and ultimately to RV hypertrophy. METHODS: A group of forty-two HLHS patients with a median age of 4.9 (2.9-17.0) years, at NYHA I was studied along with a control group of eleven subjects with healthy hearts and a median age of 12.1 (4.0-41.6). All subjects underwent MRI of the thoracic aorta including ECG-gated 2D balanced SSFP cine for an axial slice stack and 4D-flow MRI for a sagittal volume slab covering the thoracic aorta. The twist of the neoaortic arch was quantified by the effective geometric torsion, defined as the product of curvature and geometric torsion. Fluid dynamics and geometry in the neoaorta, including the flow helicity index, were evaluated using an in-house analysis software (MeVisLab-based). Myocardial mass of the systemic ventricle at end-diastole was estimated by planimetry of the short-axis stack. RESULTS: Compared to the control group, the neoaorta in the HLHS patients shows an increased twist (P=0.04) and higher peak helicity density (P=0.03). The maximum helicity density was correlated with maximum effective torsion of the ascending neoaorta (P<0.001). The degree of maximum twist correlated with the increase in RV myocardial mass (P<0.01). CONCLUSIONS: This study shows that the abnormal twist of the neoaortic arch in HLHS patients is associated with abnormal helical flow patterns, which may contribute to increased RV afterload and may adversely affect the systemic RV by stimulation of myocardial hypertrophy. These findings suggest that further improvements of surgical aortic reconstruction, guided by insights from 4D-flow MRI, could lead to better neoaortic fluid dynamics in patients with HLHS.

Abnormal aortic arch shape and vortical flow patterns are associated with descending aortic dilatation in patients with hypoplastic left heart syndrome.

INTRODUCTION: The descending aorta (DAo) in patients with hypoplastic left heart syndrome (HLHS) after Norwood procedure is frequenty enlarged and exhibits increased stiffness. Such findings of previous studies still remain unexplained given the fact that the DAo is not involved in the Norwood operation. METHODS: We studied five HLHS patients with DAo dilatation (aged: 2.9-15.1 years (y), median 9.0 y), four HLHS patients without DAo dilatation (aged: 3.5-9.7 years, median 6.45 y) and 7 healthy controls (aged 6.3-41.6 y, median 26.0 y) using cardiovascular magnetic resonance imaging with acquisition of cine images, contrast-enhanced angiograms and 4D flow. 4D flow data were analyzed based on in-house developed analysis software to quantify vortical flow patterns in terms of vorticity. RESULTS: All patients with DAo dilatation presented with a caliber reduction between the proximal and distal aortic arch of more than 40% (median reduction 71%, range 43%-79%) and with increased z-scores of the DAo. Vorticity in the DAo of patients with DAo dilatation (median: -24 s-1, range: -26 s-1 - -8 s-1) was significantly increased in magnitude compared to controls (median: 0 s-1, range: -2 - +2) (p < 0.01). Vorticity in the DAo of patients without DAo dilatatation was not significantly increased compared to controls. DAo z-scores were associated with increased vorticity. CONCLUSIONS: The findings of signficiantly increased vorticity and its association with increased DAo z-scores can potentially explain vascular alterations in the DAo of HLHS patients. This study gives motivation for further investigations and may ultimately lead to future Norwood procedure modifications.

Publisher Correction: Heart beat but not respiration is the main driving force of the systemic venous return in the Fontan circulation.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Determinants of Left Ventricular Dysfunction and Remodeling in Patients With Corrected Tetralogy of Fallot.

Background The aim of this study was to identify in asymptomatic patients with repaired tetralogy of Fallot the prevalence and determinants of impaired left-sided cardiac function and adverse ventricular remodeling and the relation of left ventricular (LV) dysfunction and remodeling with cardiopulmonary exercise capacity. Methods and Results In a cross-sectional study, 103 patients with tetralogy of Fallot (median age, 16.3 years) in New York Heart Association class 1, with surgical repair at a median age of 1.1 years, and 63 age-matched controls were studied. LV, right ventricular function and geometry, LV myocardial extracellular volume (n=57), and left atrial function were quantified with cardiac magnetic resonance. Peak oxygen consumption was measured by a standardized cardiopulmonary exercise test (n=70). Patients with tetralogy of Fallot had lower LV ejection fraction (P=0.001; 49% below age-adjusted fifth percentile for controls), lower LV mass index (P=0.003), lower LV mass/volume ratio (P<0.01), and impaired left atrial function. Right ventricular mass/volume ratio was the best predictor for LV systolic dysfunction and for a lower LV mass/volume ratio. Compared with controls, LV extracellular volume was higher (P<0.001), particularly in female patients, and associated with subnormal peak oxygen consumption (P=0.037). A peak oxygen consumption below the third percentile reference level was more likely with decreasing LV ejection fraction (P=0.008), and lower LV mass index (P=0.024), but independent of right ventricular ejection fraction. Conclusions In New York Heart Association class 1 patients with tetralogy of Fallot, frequent impaired systolic and diastolic LV function, LV adverse remodeling with LV atrophy, a decreased mass/volume ratio, and extracellular matrix expansion suggest cardiomyopathic changes. The best predictor for LV systolic dysfunction was the right ventricular mass/volume ratio. The subnormal peak oxygen consumption indicates that monitoring of LV status may be important for long-term prognosis.

Heart beat but not respiration is the main driving force of the systemic venous return in the Fontan circulation.

The Fontan procedure provides relief from cyanosis in patients with univentricular hearts. A major clinical unmet need is to understand whether the venous flow patterns of the Fontan circulation lead to the development of congestive hepatopathy and other life-threatening complications. Currently, there is no consensus on whether heart beat or respiration is the main driving force of venous return and which one affects the periodic flow changes for the most (i. e., pulsatility). The present study, for the first time, quantified respiratory and cardiac components of the venous flow in the inferior vena cava (IVC) of 14 Fontan patients and 11 normal controls using a novel approach ("physio-matrix"). We found that in contrast to the normal controls, respiration in Fontan patients had a significant effect on venous flow pulsatility, and the ratio of respiration-dependent to the cardiac-dependent pulsatility was positively associated with the retrograde flow. Nevertheless, the main driving force of net IVC flow was the heart beat and not respiration. The separate analysis of the effects of respiration and heart beat provides new insights into the abnormal venous return patterns that may be responsible for adverse effects on liver and bowel of the patients with Fontan circulation.

Longitudinal Deformation of the Right Ventricle in Hypoplastic Left Heart Syndrome: A Comparative Study of 2D-Feature Tracking Magnetic Resonance Imaging and 2D-Speckle Tracking Echocardiography.

In hypoplastic left heart syndrome (HLHS), long-term outcome is closely related to right ventricular function. Echocardiography and magnetic resonance imaging (MRI) are routinely used for functional assessment. MRI 2D-tissue feature tracking (2D-FT) allows quantification of myocardial deformation but has not yet been applied to HLHS patients. We sought to investigate the feasibility of this technique and to compare the results to 2D-speckle tracking echocardiography (2D-STE). In routine MRI 2D anatomical four chamber view, cine images were recorded in 55 HLHS patients (median age 4.9 years [1.6, 17.0]). Regional and global peak systolic longitudinal strain (LS) and strain rate (LSR) were determined using 2D-FT software. Echocardiographic four chamber view was analyzed with 2D-STE. Visualization of all myocardial segments with MRI was excellent, regional, and global LS and LSR could be assessed in all data sets. In 2D-STE, 28% of apical segments could not be analyzed due to poor image quality. Agreement of 2D-FT MRI and 2D-STE was acceptable for global LS, but poor for global LSR. In MRI, regional LS was lower in the septal segments, while LSR was not different between the segments. GLS and GLSR correlated with ejection fraction (GLS: r = - 0.45 and r < 0.001, GLSR: r = - 0.34 and p = 0.01). With new post-processing options, the assessment of regional and global LS and LSR is feasible in routine MRI of HLHS patients. For LS, results were comparable with 2D-STE. The agreement was poor for LSR, which might relate to differences in temporal resolution between the two imaging modalities.