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Background: Four-dimensional cardiovascular magnetic resonance flow imaging (4D flow CMR) plays an important role in assessing cardiovascular diseases. However, the manual or semi-automatic segmentation of aortic vessel boundaries in 4D flow data introduces variability and limits the reproducibility of aortic hemodynamics visualization and quantitative flow-related parameter computation. This paper explores the potential of deep learning to improve 4D flow CMR segmentation by developing models for automatic segmentation and analyzes the impact of the training data on the generalization of the model across different sites, scanner vendors, sequences, and pathologies., Methods: The study population consists of 260 4D flow CMR datasets, including subjects without known aortic pathology, healthy volunteers, and patients with bicuspid aortic valve (BAV) examined at different hospitals. The dataset was split to train segmentation models on subsets with different representations of characteristics, such as pathology, gender, age, scanner model, vendor, and field strength. An enhanced three-dimensional U-net convolutional neural network (CNN) architecture with residual units was trained for time-resolved two-dimensional aortic cross-sectional segmentation. Model performance was evaluated using Dice score, Hausdorff distance, and average symmetric surface distance on test data, datasets with characteristics not represented in the training set (model-specific), and an overall evaluation set. Standard diagnostic flow parameters were computed and compared with manual segmentation results using Bland-Altman analysis and interclass correlation., Results: The representation of technical factors, such as scanner vendor and field strength, in the training dataset had the strongest influence on the overall segmentation performance. Age had a greater impact than gender. Models solely trained on BAV patients' datasets performed well on datasets of healthy subjects but not vice versa., Conclusion: This study highlights the importance of considering a heterogeneous dataset for the training of widely applicable automatic CNN segmentations in 4D flow CMR, with a particular focus on the inclusion of different pathologies and technical aspects of data acquisition., Competing Interests: Declaration of competing interests Chiara Manini reports financial support was provided by German Research Foundation. Sebastian Kelle reports financial support was provided by German Center for Cardiovascular Disease. Anja Hennemuth reports financial support was provided by German Research Foundation. Titus Kuehne reports financial support was provided by German Research Foundation. Sebastian Kelle reports financial support was provided by German Research Foundation. Andreas Harloff reports financial support was provided by University of Freiburg Faculty of Medicine. Sebastian Kelle reports a relationship with Philips Healthcare that includes funding grants. The other authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: In congenital aortic valve disease, quantifying aortic regurgitation (AR) varies by the measurement site. Our study aimed to identify the optimal site for AR assessment using 2D and 4D MR flow measurements, with a focus on vortices., Methods: We retrospectively analysed 31 patients with congenital aortic valve disease, performing 2D and 4D MR flow measurements at the aortic valve, sinotubular junction (STJ), ascending aorta (AAo), and using midpulmonary artery measurements as a reference. We assessed percentage AR and net forward volumes, calculated linear correlations, and plotted Bland-Altman plots. Net forward flow at all aortic sites were correlated with the main pulmonary artery. Differences in AR between 2D and 4D flows were linked to vortices detected by 4D streamlines., Results: The best agreement in % AR between 2D and 4D flows was at the aortic valve (mean difference 4D2D -2.9%, limits of agreement 8.7% to -14.3%; r 2  = 0.7). Correlations weakened at STJ and AAo. Vortices in the ascending aorta led to AR overestimation in 2D measurements. Net forward flow at the aortic valve by 4D flow correlated closer with main pulmonary artery than did 2D flow. (Mean difference for 2D and 4D MR flow 7.5 ml and 4.2 ml, respectively)., Conclusions: For congenital aortic valve disease, the most accurate AR quantification occurs at the aortic valve using 2D and 4D MR flow. Notably, vortices in the ascending aorta can result in AR overestimation with 2D MR flow., Competing Interests: Declaration of competing interest None. Use of generative AI an AI assisted technologies: none., (Copyright © 2023. Published by Elsevier B.V.)

Introduction: In infants and young children, good image quality in MRI and CT requires sedation or general anesthesia to prevent motion artefacts. This study aims to determine the safety of ambulatory sedation for children with CHD in an outpatient setting as a feasible alternative to in-hospital management., Methods: We recorded 91 consecutive MRI and CT examinations of patients with CHD younger than 6 years with ambulatory sedation. CHD diagnoses, vital signs, applied sedatives, and adverse events during or after ambulatory sedation were investigated., Results: We analysed 91 patients under 72 months (6 years) of age (median 26.0, range 1-70 months; 36% female). Sixty-eight per cent were classified as ASA IV, 25% as ASA III, and 7% as ASA II (American Society of Anesthesiologists Physical Status Classification). Ambulatory sedation was performed by using midazolam, propofol, and/or S-ketamine. The median sedation time for MRI was 90 minutes (range 35-235 minutes) and 65 minutes for CT (range 40-280 minutes). Two male patients (age 1.5 months, ASA II, and age 17 months, ASA IV) were admitted for in-hospital observation due to unexpected severe airway obstruction. The patients were discharged without sequelae after 1 and 3 days, respectively. All other patients were sent home on the day of examination., Conclusion: In infants and young children with CHD, MRI or CT imaging can be performed under sedation in an outpatient setting by a well-experienced team. In-hospital backup should be available for unexpected events.

Background: Bicuspid aortic valve (BAV) disease leads to deviant helical flow patterns especially in the mid-ascending aorta (AAo), potentially causing wall alterations such as aortic dilation and dissection. Among others, wall shear stress (WSS) could contribute to the prediction of long-term outcome of patients with BAV. 4D flow in cardiovascular magnetic resonance (CMR) has been established as a valid method for flow visualization and WSS estimation. The aim of this study is to reevaluate flow patterns and WSS in patients with BAV 10 years after the initial evaluation., Methods: Fifteen patients (median age 34.0 years) with BAV were re-evaluated 10 years after the initial study from 2008/2009 using 4D flow by CMR. Our particular patient cohort met the same inclusion criteria as in 2008/2009, all without enlargement of the aorta or valvular impairment at that time. Flow patterns, aortic diameters, WSS and distensibility were calculated in different aortic regions of interest (ROI) with dedicated software tools., Results: Indexed aortic diameters in the descending aorta (DAo), but especially in the AAo did not change in the 10-year period. Median difference 0.05 cm/m 2 (95% CI: 0.01 to 0.22; P=0.06) for AAo and median difference -0.08 cm/m 2 (95% CI: -0.12 to 0.01; P=0.07) for DAo. WSS values were lower in 2018/2019 at all measured levels. Aortic distensibility decreased by median 25.6% in the AAo, while stiffness increased concordantly (median +23.6%)., Conclusions: After a ten years' follow-up of patients with isolated BAV disease, indexed aortic diameters did not change in this patient cohort. WSS was lower compared to values generated 10 years earlier. Possibly a drop of WSS in BAV could serve as a marker for a benign long-term course and implementation of more conservative treatment strategies., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://cdt.amegroups.com/article/view/10.21037/cdt-22-477/coif). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part V” was commissioned by the editorial office without any funding or sponsorship. The authors have no other conflicts of interest to declare., (2023 Cardiovascular Diagnosis and Therapy. All rights reserved.)

Background: MR or CT scans are often required in the treatment of pediatric intensive care patients. During the therefore needed intrahospital transport continuous ventilation of the patient must be maintained. Intrahospital transport is considered safe regarding changes in hemodynamics or adverse events (AEs). As those studies cover inhomogeneous patient groups, we analyzed the safety for ventilated pediatric patients with congenital heart disease (CHD) focusing on differences between manual and mechanical ventilation during transport and examination., Methods: Retrospective monocentric case-control study covering a 10 years' period in a tertiary cardiac center for CHD. Sixty-three critically ill ventilator-dependent patients, median 2 (0-37) months, were included. Fifty-one patients got ventilated manually and 12 patients got ventilated with a mobile ventilator. The data include vital parameters and blood gas data, as well as catecholamine support, occurrence of AEs and total duration of transport and examination., Results: In both groups we found minor changes of vital parameters or blood gas data. Regarding the HR we found a drop from median 135/min before leaving the ICU to 130/min after returning to the ICU (P=0.0072) in the manually ventilated group and a drop from median 133/min to 123/min (P=0.0703) in the mechanically ventilated group. The median transport time including scan was higher in the manually ventilated group (P=0.0098) with a median duration of transport and scan time of 100 minutes (30-245 minutes) in the manually ventilated group and of 97.5 minutes (60-224 minutes) in the mechanically ventilated group. A total of 9 AEs was recorded, 7 (13.7%) of them in the manually and 2 (16.7%) in the mechanically ventilated group with a drop of the mean arterial pressure (MAP) and an increase in catecholamine support., Conclusions: We consider both manual ventilation and mechanical ventilation for intrahospital transport safe for pediatric intensive care patients with CHD. Using a mechanical ventilator might have the advantage to react faster to changes in hemodynamics., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://cdt.amegroups.com/article/view/10.21037/cdt-22-384/coif). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part V” was commissioned by the editorial office without any funding or sponsorship. The authors have no other conflicts of interest to declare., (2022 Cardiovascular Diagnosis and Therapy. All rights reserved.)

Background: Cardiovascular magnetic resonance feature-tracking analysis (CMR-FT) provides a quantitative assessment of myocardial contraction with potential for diagnostic and prognostic ability in a wide spectrum of diseases. Ebstein's anomaly (EA) is a rare congenital heart disease characterized by apical displacement of the tricuspid valve. However, it is also considered a disorder of development affecting the global right ventricular myocardium. Aim of our study is to describe the complex contractile mechanics of the functional right ventricle (RV) in patients affected by EA through CMR-FT., Methods: Fifty surgery-free EA patients who had undergone a complete CMR protocol at our institution between January 2017 and December 2020 were selected for the retrospective study. A historical control group of twenty-five healthy subjects was also included. CMR-FT analysis was performed at a dedicated workstation by manually tracing RV endo- end epicardial borders on steady-state-free-precession (SSFP) cine images. Strain values were calculated. Apical displacement of the tricuspid valve (TV) was measured on a 4-chamber cine image from the right atrio-ventricular junction to the functional annulus of the TV., Results: EA patients presented significantly impaired RV global radial strain (GRS) and global circumferential strain (GCS) compared to controls (P<0.0001 and P=0.0008, respectively). In a subgroup analysis, GRS was significantly compromised in patients with a severely displaced TV (>16 mm/m 2 ) compared to milder forms (P=0.03) and to controls (P<0.0001). Among EA patients with a preserved ejection fraction, 12 (48%) vs. 6 (24%) controls had reduced both GRS and GCS., Conclusions: The contractile pattern of the functional RV in EA is characterised by prevalent alterations in the short-axis direction as indicated by reduced GRS and GCS. Strain values might be reduced prior to routine used functional parameters like RV ejection fraction (RVEF) and can possibly serve as an early predictor of myocardial dysfunction in EA patients., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://cdt.amegroups.com/article/view/10.21037/cdt-22-82/coif). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part V” was commissioned by the editorial office without any funding or sponsorship. The authors have no other conflicts of interest to declare., (2022 Cardiovascular Diagnosis and Therapy. All rights reserved.)

Background: Adolescents and adults with native Ebstein's anomaly (EA) are at the benign part of the Ebstein spectrum, having survived infancy without surgery. In this population, surgical indication and timing remain objects of controversy and depend, among other factors, on exercise capacity., Purpose: To better understand the pathophysiology of exercise adaptation in native EA., Study Type: Retrospective., Population: Ten patients with unoperated EA (age range 18-61 years) and 13 healthy subjects as controls., Field Strength/sequence: Balanced steady-state free precession cine and phase contrast flow sequences at 1.5 T., Assessment: We measured volumes and flows at rest and during submaximal exercise. Hemodynamic parameters including stroke volume (SV), cardiac index (CI), ejection fraction (EF), and tricuspid regurgitation (TR) were calculated., Statistical Tests: We used nonparametric Mann-Whitney U-test and Wilcoxon signed-rank test. A P-value of <0.05 was considered statistically significant., Results: Rest CI and SV were significantly higher in controls; rest heart rate (HR) was similar in the two groups (median 71 bpm by patients and 65 bpm by controls, P = 0.448). During exercise, CI increased significantly in both groups: from 2.40 to 3.35 L/min/m 2 in the patient group and from 3.60 to 4.20 L/min/m 2 in controls; HR increased significantly in both groups. SV increased significantly in the patient group, whereas it remained stable in controls (P = 0.5284). Patients' median TR decreased significantly: median 42% at rest and 30% during exercise; concomitantly, left ventricular (LV) preload increased significantly (+3% indexed LV end-diastolic volume) as did LVEF (median 59% at rest vs. 65% during exercise)., Data Conclusion: During submaximal exercise, patients with mild to moderate EA improved their cardiovascular system's total efficiency by increasing CI; this was obtained by an increase in HR and by the recruitment of volume, as shown by an increased LV end-diastolic volume and SV, with simultaneous decrease in TR. This was different from healthy subjects in which CI increased only due to HR increase., Level of Evidence: 3 TECHNICAL EFFICACY STAGE: 3., (© 2021 The Authors. Journal of Magnetic Resonance Imaging published by Wiley Periodicals LLC on behalf of International Society for Magnetic Resonance in Medicine.)

Background: Cardiovascular magnetic resonance (CMR) is widely used for aortic diameter assessment but there is no consensus on the sequence or cardiac cycle phase in which the measures should be taken. The most used sequence is contrast-enhanced-magnetic-resonance-angiography (angiography), usually non-ECG-triggered. An alternative is a navigated 3D-whole-heart-steady-state-free-precession sequence which is contrast-free and breath- and ECG-gated (mostly diastolic gating), producing very sharp anatomical rendering. Nonetheless, its routine use has not yet spread. Our aim was evaluating aortic diameters by a systolic-gated 3D and put additional effort in the validation of diastolic-gated 3D as alternative to angiography., Methods: We retrospectively analysed 30 patients scheduled for routine Angiography. We measured the aorta at 9 standard positions by three different sequences (angiography, 3D-diastole and 3D-systole) and compared the diameters obtained by calculating the differences and by paired t -test analysis., Results: Diameters by 3D-systole were larger than by 3D-diastole and angiography (P<0.01). In the ascending aorta we found the maximal differences between systole and diastole and between systole and angiography which were 1.7± SD 1.02 mm and 1.5± SD 1.07 mm respectively. There was no significant difference between diastolic and angiography measurements (mean difference 0.2± SD 0.16 mm, P not significant)., Conclusions: Our results support the use of navigated 3D-whole-heart CMR to evaluate aortic diameters. Systolic-gated 3D produces larger diameter, especially in the ascending aorta., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/cdt-20-868). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part IV” was commissioned by the editorial office without any funding or sponsorship. The authors have no other conflicts of interest to declare., (2021 Cardiovascular Diagnosis and Therapy. All rights reserved.)

Background: The aim of this study was to test a CE-certified MR-conditional guidewire to facilitate blood pressure measurement in cardiovascular magnetic resonance (CMR) using fluid-filled catheters in patients with congenital heart disease (CHD). The main purpose was to determine procedural success in a post market clinical follow-up (PMCF) for routine procedure in a diagnostic and interventional workflow. Real-time CMR provides high quality imaging without the risk of exposing the patient to X-rays, especially for patients with irregular heart anatomy and patients who are susceptible to radiation and iodinated contrast media. To date, the assessment of blood pressure gradients is not a common feature of CMR, as these gradients cannot be accurately evaluated in routine CMR., Methods: Twenty-five CHD patients who were planned for combined clinical CMR and diagnostic and/or interventional catheterization were enrolled in the trial. Prior to inclusion, a specific procedure for catheterization in CMR was defined, encompassing the assessment of pressure and pressure gradients in the heart and great vessels., Results: By the use of an MR-conditional guidewire we successfully measured specific pressure and pressure gradients in up to 92% of cases with liquid-filled catheters which were guided exclusively under CMR guidance. There were no guidewire-related adverse events, and guidewire guidance and manipulation of catheters were successful., Conclusions: Using a MR-conditional guidewire assists in easily reaching targets in the heart and great vessels and makes the catheter itself visible, so that invasive blood pressure assessment by CMR guidance with liquid-filled catheters can be improved., Keywords: Cardiovascular magnetic resonance (CMR); congenital heart disease (CHD); cardiac catheterization; magnetic resonance; pressure; guidewire., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/cdt-20-575). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part IV” was commissioned by the editorial office without any funding or sponsorship. Dr. Meierhofer reports personal fees and other from Nano4Imaging, Düsseldorf, Germany, during the conduct of the study; personal fees from Nano4Imaging, outside the submitted work. Dr. Belker reports other from Nano4Imaging, Düsseldorf, Germany, during the conduct of the study. Dr. Shehu reports other from Nano4Imaging, Düsseldorf, Germany, during the conduct of the study. Dr. Latus reports other from Nano4Imaging, Düsseldorf, Germany, during the conduct of the study. Dr. Mkrtchyan reports other from Nano4Imaging, Düsseldorf, Germany, during the conduct of the study. Dr. Naumann reports other from Nano4Imaging, Düsseldorf, Germany, during the conduct of the study. Dr. Stern reports other from Nano4Imaging, Düsseldorf, Germany, during the conduct of the study. Dr. Eicken reports other from Nano4Imaging, Düsseldorf, Germany, during the conduct of the study. Dr. Ewert reports other from Nano4Imaging, Düsseldorf, Germany, during the conduct of the study. The authors have no other conflicts of interest to declare., (2021 Cardiovascular Diagnosis and Therapy. All rights reserved.)

Background Right atrial (RA) dilatation and impaired right ventricular (RV) filling are common in patients with RV outflow tract dysfunction. We aimed to study potential correlations between atrial function with clinically relevant hemodynamic parameters and to assess the predictive impact of atrial performance on the recovery of exercise capacity and RV pump function after percutaneous pulmonary valve implantation (PPVI). Methods and Results Altogether, 105 patients with right ventricular outflow tract dysfunction (median age at PPVI, 19.2 years; range, 6.2-53.4 years) who underwent cardiac magnetic resonance imaging before and 6 months after PPVI were included. RA and left atrial maximal and minimal volumes as well as atrial passive and active emptying function were assessed from axial cine slices. RA emptying function was inversely related to invasive RV end-diastolic pressure, and RA passive emptying correlated significantly with peak oxygen uptake. After PPVI, a significant decrease in RA minimum volume was observed, whereas RA passive emptying function improved, and RA active emptying function decreased significantly. Patients with predominant right ventricular outflow tract stenosis showed more favorable changes in RA active and left atrial passive emptying than those with primary volume overload. None of the RA and left atrial emptying parameters was predictive for recovery of peak oxygen uptake or RV ejection fraction. Conclusions In patients with right ventricular outflow tract dysfunction, impaired RA emptying assessed by cardiac magnetic resonance imaging was associated with increased RV filling pressures and lower exercise capacity. PPVI leads to a reduction in RA size and improved passive RA emptying function. However, RA function was not associated with improved exercise performance and RV pump function.

Sudden cardiac death (SCD) is the most common cause of late mortality in tetralogy of Fallot (TOF). Pulmonary regurgitation (PR) was previously found to be the most common hemodynamic abnormality associated with ventricular arrhythmias (VA), but cardiovascular magnetic resonance (CMR)-based studies did not show this association. The aim of this study is to investigate the risk factors for VA in TOF using CMR. Electronic records of TOF patients and their CMR studies between July 2006 and October 2018 in one center were retrospectively reviewed. Demographic, clinical and CMR data of patients were collected. Outcome was defined as sustained ventricular tachycardia (VT), aborted SCD and SCD. From a total of 434 TOF patients with complete CMR studies, 19 (4.4%) patients developed a positive outcome (12 sustained VT, 4 aborted SCD, 3 SCD) at a median age of 24 years. The number of surgical interventions was significantly greater in patients who developed VA. Right ventricular volumes were significantly larger in patients who suffered a positive outcome. Odds ratio for developing VA was 6.905 for RVEDVI ≥ 160 ml/m 2 and 6.141 for RVESVI ≥ 80 ml/m 2 (P = 0.0014 and 0.0012, respectively). Event-free survival was longer in patients with smaller right ventricular volumes. In conclusion, right ventricular dimensions are the most significant factors associated with the development of VA in TOF. The number of surgical interventions is also related to an increased risk.

Percutaneous pulmonary valve replacement (PPVI) in native or patched right ventricular outflow tract (RVOT) has proven to be feasible. The procedure is highly dependent on the size of the RVOT. Several methods exist to evaluate the size of the RVOT by cardiovascular magnetic resonance (CMR). We evaluated different CMR modalities for measuring RVOT diameters. Thirty-one consecutive patients with native or patched RVOT were retrospectively evaluated. CMR was part of follow-up of patients with corrected Tetralogy of Fallot or pulmonary stenosis with significant pulmonary regurgitation (PR). CMR included 3D-SSFP whole-heart in systole, diastole, and contrast-enhanced MR angiography (ceMRA). Diameters of the RVOT were assessed by the three sequences. Additionally, in patients who underwent cardiac catheterization (n = 11) for PPVI, vessel diameters assessed by cine-angiography were compared to CMR. Systolic diameters of RVOT were significantly larger compared to the diameters taken in diastole and ceMRA (median difference 5.0 mm and 3.8 mm). Diastolic and ceMRA diameters did not differ significantly. CMR diameters taken in systole showed no statistical difference to systolic diameters taken by cine-angiography, while diastolic and ceMRA diameters were significantly smaller. PPVI was feasible to a maximal CMR diameter of 31 mm measured by SSFP whole-heart sequence in systole. Absolute diameters of native RVOT differ depending on the CMR sequence and timing of acquisition (systolic vs diastolic gating). Diameters taken during heart catheterization by cine-angiography best correlate to systolic CMR values. Data may help to select RVOTs suitable for PPVI.

Objective: To evaluate the incidence of cancer within the first 15 years of life in children who underwent cardiac catheterization under the age of one year., Methods: In this retrospective, single center study, 2770 infants (7.8% with trisomy 21) were studied. All infants underwent cardiac catheterization under one year of age between January 1980 and December 1998. Newly diagnosed cancer in the first 15 years of life was assessed through record linkage to the German Childhood Cancer Registry (GCCR). Cancer risk in study patients was compared to the GCCR population of children less than 15 years. Patients with trisomy 21 were compared to the Danish Cytogenic Register for trisomy 21. Effective radiation doses were calculated for each tumor patient and 60 randomly selected patients who did not develop cancer., Results: In total, 24,472.5 person-years were analyzed. Sixteen children developed cancer, while 3.64 were expected (standardized incidence ratio (SIR) = 4.4, 95% confidence interval (CI): 2.5-7.2, p < 0.001). There was no preferred cancer type. The observed incidence of leukemia and solid tumors in trisomy 21 was only slightly higher (1 in 476 py) than expected (1 in 609 py, p = 0.64). There was no direct relationship between the radiation dose and the incidence of cancer., Conclusion: Cardiac catherization in the first year of life was associated with a significantly increased cancer risk in a population with congenital heart disease., Competing Interests: The authors declare no conflict of interest.

Background: Alterations in wall shear stress (WSS) assessed using 4D flow MRI have been shown to play a role in various vascular pathologies, such as bicuspid aortic valve aortopathy. Most studies have focused on systolic WSS, whereas altered diastolic hemodynamics in regurgitant semilunar valvular lesions have not so far been well characterized., Purpose: To investigate diastolic WSS in aortic and pulmonary regurgitation., Study Type: Retrospective data analysis., Population: Thirty tetralogy of Fallot patients, 19 bicuspid aortic valve patients, 11 healthy volunteers., Field Strength/sequence: 5 T, 3D time-resolved phase-contrast MRI with 3D velocity encoding., Assessment: Estimation of WSS and its axial and circumferential vector components along cardiac cycle timeframes in the proximal main pulmonary artery in pulmonary regurgitation (PR) and in the proximal ascending aorta in aortic regurgitation (AR) as well as in healthy volunteers., Statistical Tests: Wilcoxon matched pairs test was used for intra-group comparisons and Mann-Whitney test for intergroup comparisons. Correlations were assessed using Spearman correlation., Results: WSS along the entire cardiac cycle was higher in PR and AR in comparison with controls (mean WSS 0.381 ± 0.070 vs. 0.220 ± 0.018, P < 0.0001; 0.361 ± 0.099 vs. 0.212 ± 0.030, P < 0.0001; respectively). Peak diastolic WSS was significantly higher than the mean WSS in AR and PR (P < 0.0001-0.005). The severity of PR correlated with the peak diastolic axial WSS (Spearman's r s  = 0.454, P = 0.018), whereas the severity of AR correlated with both peak systolic and diastolic tangential WSS (Spearman's r s  = 0.458, P = 0.049; r s  = 0.539, P = 0.017, respectively)., Data Conclusion: Elevated diastolic WSS is a component of the altered flow hemodynamics in AR and PR. This may give more insight into the pathophysiologic role of WSS in vascular remodeling in AR and PR., Level of Evidence: 4 Technical Efficacy Stage: 1 J. Magn. Reson. Imaging 2019;50:763-770., (© 2019 International Society for Magnetic Resonance in Medicine.)

Background: Exercise capacity is a well-defined marker of outcome in congenital heart disease. We analyzed seventeen cardiovascular magnetic resonance (CMR) derived parameters and their correlation to exercise capacity in patients with Ebstein's anomaly (EA)., Methods: Fifty-four surgery free patients, age 5 to 69 years (median 30 years) prospectively underwent CMR examination and cardiopulmonary exercise testing (CPET). The following volume/flow parameters were compared with peak oxygen uptake as the percentage of normal (peakVO 2 %) using univariate and multivariate analysis: right and left ventricular ejection fraction (RVEF and LVEF), the indexed end-diastolic and end-systolic volumes (RVEDVi, RVESVi, LVEDVi, and LVESVi), the indexed stroke volumes (RVSVi and LVSVi), the total normalized right and left heart volumes; the total right to left heart volume ratio (R/L-ratio). The indexed antegrade flow (ante), indexed net flow (net) as well as cardiac index (CI) in the aorta (Ao) and pulmonary artery (PA) were used., Results: RVEF (R 2 0.2788), indexed flow PA net (R 2 0.2330), and PA ante (R 2 0.1912) showed the best correlation with peakVO 2 % (all p < 0.001) in the univariate model. Further significant correlation could also be demonstrated with CI-PA, LVEF, LVSVi, Aorta net, RVESVi, and Aorta ante. Multivariate analysis for RVEF and indexed net flow PA revealed a R 2 of 0.4350., Conclusion: Functional CMR parameters as RVEF and LVEF and flow data of cardiac forward flow correlate to peakVO 2 %. Evaluation of the indexed net flow in the pulmonary artery and the overall function of the right ventricle best predicts the maximal exercise capacity in patients with EA.

In adult patients with transposition of the great arteries (TGA) late after atrial switch operation (AtSO), each of the ventricles is faced with a profoundly different pressure regimen from the one they are meant to support in normal conditions. The extent of diffuse fibrosis of the right ventricle (RV) and left ventricle (LV) in these patients remains incompletely investigated. Aim of this study was to quantify the degree of fibrosis of the unloaded LV and of the overloaded RV by determining the myocardial extracellular volume (ECV) with non-invasive techniques as T1 mapping. We determined ECV by cardiac magnetic resonance (CMR) in 10 patients (36.8 ± 5.3 years old) with TGA late after AtSO, without relevant pulmonary stenosis, by acquiring T1-maps of the myocardium before and 10 min after injection of Gadolinium-based contrast agent. ECV of the inferior wall (36% (33-41%)) and of the lateral wall (37% (35-39%)) of the LV was significantly increased compared to the ECV of the RV (27% (25-29%)), in both comparisons P < 0.0001. Long-time LV unloading following atrial switch procedures leads to severe myocardial fibrosis of the subpulmonary LV. T1 mapping CMR might be useful for selection of patients with atrial switch operation, in whom reestablishment of the LV as a systemic ventricle by staged arterial switch operation is planned. However larger studies and newer higher resolution methods for T1-mapping are needed to determine the role of ECV in the decision of a surgical intervention in this kind of population.

Background: Aortopulmonary collateral flow is considered to have significant impact on the outcome of patients with single ventricle circulation and total cavopulmonary connection (TCPC). There is little information on collateral flow during exercise., Purpose: To quantify aortopulmonary collateral flow at rest and during continuous submaximal exercise in clinical patients doing well with TCPC., Study Type: Prospective, case controlled., Population: Thirteen patients with TCPC (17 (11-37) years) and 13 age and sex-matched healthy controls (18 (11-38) years)., Field Strength: 1.5T; free breathing; phase sensitive gradient echo sequence., Assessment: Blood flow in the ascending and descending aorta and superior vena cava were measured at rest and during continuous submaximal physical exercise in patients and controls. Systemic blood flow (Q s ) was assumed to be represented by the sum of flow in the superior caval vein (Q svc ) and the descending aorta (Q AoD ) at the diaphragm level. Aortopulmonary collateral flow (Q coll ) was calculated by subtracting Q s from flow in the ascending aorta (Q AoA )., Statistics: Mann-Whitney U-test and Wilcoxon test for comparison between groups and between rest and exercise., Results: Absolute collateral flow in TCPC patients at rest was 0.4 l/min/m 2 (-0.1-1.2), corresponding to 14% (-2-42) of Q s . Collateral flow did not change during exercise (difference -0.01 (-0.7-1.0) l/min/m 2 , P = 0.97). TCPC patients had significantly lower Q s at rest (2.5 (1.6-4.1) vs. 3.5 (2.6-4.8) l/min/m 2 , P = 0.001) and during submaximal exercise (3.2 (2.0-6.0) vs. 4.8 (3.3-6.9) l/min/m 2 , P = 0.001), compared to healthy controls. The increase in Q s with exercise was also significantly lower in patients than in healthy controls (median 0.6 vs. 1.2 l/min/m 2 , P < 0.02)., Data Conclusion: Clinical patients doing well with TCPC have significant aortopulmonary collateral flow at rest (14% of Q s ) compared to healthy controls, which does not change during submaximal exercise., Level of Evidence: 2 Technical Efficacy: Stage 3 J. Magn. Reson. Imaging 2018;47:1509-1516., (© 2017 International Society for Magnetic Resonance in Medicine.)

Background: Wall shear stress (WSS) presents an important parameter for assessing blood flow characteristics and evaluating flow-mediated lesions in the aorta., Purpose: To investigate the robustness of WSS and oscillatory shear index (OSI) estimation based on 4D flow MRI against vessel wall motion, spatiotemporal resolution, and velocity encoding (VENC)., Study Type: Simulated and prospective., Population: Synthetic 4D flow MRI data of the aorta, simulated using the Lattice-Boltzmann method; in vivo 4D flow MRI data of the aorta from healthy volunteers (n = 11) and patients with congenital heart defects (n = 17)., Field Strength/sequence: 1.5T; 4D flow MRI with PEAK-GRAPPA acceleration and prospective electrocardiogram triggering., Assessment: Predicated upon 3D cubic B-splines interpolation of the image velocity field, WSS was estimated in mid-systole, early-diastole, and late-diastole and OSI was derived. We assessed the impact of spatiotemporal resolution and phase noise, and compared results based on tracked-using deformable registration-and static vessel wall location., Statistical Tests: Bland-Altman analysis to assess WSS/OSI differences; Hausdorff distance (HD) to assess wall motion; and Pearson's correlation coefficient (PCC) to assess correlation of HD with WSS., Results: Synthetic data results show systematic over-/underestimation of WSS when different spatial resolution (mean ± 1.96 SD up to -0.24 ± 0.40 N/m 2 and 0.5 ± 1.38 N/m 2 for 8-fold and 27-fold voxel size, respectively) and VENC-depending phase noise (mean ± 1.96 SD up to 0.31 ± 0.12 N/m 2 and 0.94 ± 0.28 N/m 2 for 2-fold and 4-fold VENC increase, respectively) are given. Neglecting wall motion when defining the vessel wall perturbs WSS estimates to a considerable extent (1.96 SD up to 1.21 N/m 2 ) without systematic over-/underestimation (Bland-Altman mean range -0.06 to 0.05)., Data Conclusion: In addition to sufficient spatial resolution and velocity to noise ratio, accurate tracking of the vessel wall is essential for reliable image-based WSS estimation and should not be neglected if wall motion is present., Level of Evidence: 2 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2018., (© 2018 International Society for Magnetic Resonance in Medicine.)

Objectives: Surgical septal myectomy is performed to relieve left ventricular outflow tract narrowing in severe drug-refractory obstructive hypertrophic cardiomyopathy. The objective of this study was to assess the perioperative and long-term clinical outcome of this procedure performed during infancy., Methods: Clinical, transthoracic echocardiographic, electrocardiographic, 24-h Holter, cardiopulmonary exercise test and genetic data were extracted by medical record review. A subset of patients underwent additional prospective detailed clinical evaluation including cardiac magnetic resonance imaging with contrast., Results: Surgery was performed in 23 paediatric patients between 1978 and 2015 at the German Heart Centre Munich. Twelve patients had undergone surgery during infancy (≤ 1 year) (Group A), 11 between 1 and 18 years of age (Group B). The underlying genetic diagnosis was Noonan syndrome spectrum and non-syndromic hypertrophic cardiomyopathy. As compared to Group B, patients in Group A showed more concomitant cardiac procedures and received more homologous transfusions. One perioperative death occurred in Group A, and none in Group B. Two patients in Group A but no patient in Group B required redo septal myectomy. The long-term clinical outcome was similar between the 2 groups. One patient in Group B required cardioverter-defibrillator/pacemaker implantation for higher degree atrioventricular block and none in Group A. There was no evidence of differences in myocardial fibrosis between groups on long-term follow-up magnetic resonance imaging., Conclusions: Surgical septal myectomy can be performed safely during infancy with favourable perioperative and long-term clinical outcome but with a trend towards a higher reoperation rate later in life., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

The assumption of the presence of diffuse myocardial fibrosis in long-standing cyanotic congenital heart disease (CHD) inspired us to noninvasively determine the myocardial extracellular volume (ECV) using contrast CMR. T1 maps were measured pre and 10 min after the injection of 0.15 mmol/kg of gadolinium in 25 subjects. Seven patients with long-standing cyanotic CHD and no previous cardiac surgery (aged 16-53 years and oxygen saturations of 69-90%), nine normal subjects (aged 14-49 years), and nine patients with previously cyanotic CHD, who had been corrected by open heart surgery (aged 2 months-58 years, mean 9 years). Late gadolinium enhancement was performed to exclude scar areas. The T1 values were measured in the interventricular septum and in the left lateral or inferior ventricular wall, such that same areas were assessed in every patient in the pre- and post-contrast T1 scan. ECV was calculated according to ΔR1myocardium/ΔR1blood * (1 - hematocrit). Cyanotic patients had significantly lower ECV percentage than the previous cyanotic patients (septum: 22 ± 2.7% vs 35 ± 4.6%, p = 0.002; LV wall: 22 ± 2.2% vs 30 ± 3.7%, p = 0.01, respectively). No significant differences were found between cyanotic patients and normal controls (septum: 22 ± 2.7% vs 24 ± 1.4%, p = 0.44; LV wall: 22 ± 2.2% vs 24 ± 2%, p = 0.57, respectively). Long-standing cyanosis in CHD without cardiac surgery does not cause diffuse myocardial fibrosis or expansion of the myocardial ECV.

Purpose: To compare the quantification of pulmonary stroke volume (SV) by phase contrast magnetic resonance (PC-MR) in the main pulmonary artery (MPA) to the sum of SVs in both peripheral pulmonary arteries (PPA) in different right ventricular (RV) outflow pathologies., Materials and Methods: Pulmonary SV was determined by PC-MR in the MPA and the PPA in healthy individuals (H, n = 54), patients after correction for tetralogy of Fallot with significant pulmonary regurgitation and without pulmonary or RV outflow tract stenosis (PR, n = 50), and in patients with RV outflow tract or pulmonary valve stenosis (PS, n = 50). Resulting SVs were compared to aortic SV in the ascending aorta., Results: Mean age was similar between the groups: H 28 ± 17 vs. PR 24 ± 11 vs. PS 22 ± 10 years. Bland-Altman analyses revealed in all groups a relatively small systemic (bias) but large random error (limits of agreement) for pulmonary SV determined in the MPA as compared to summed SVs in the PPA. The largest limits of agreement were present in PS patients: H: MPA 3.9% (-11, + 19) vs. PPA 0.4% (-15, + 15); PR: MPA 5.2% (-25, + 36) vs. PPA 0.6% (-24, + 26); PS: MPA 5% (-36; + 46), PPA -0.03% (-34, + 35)., Conclusion: The accuracy of PC-MR in the MPA is reasonable; however, a large random error (precision) is observed that is most pronounced in PS patients. This potential error should be taken into consideration when interpreting MPA flow measurements., Level of Evidence: 3 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2017;46:1839-1845., (© 2017 International Society for Magnetic Resonance in Medicine.)

Good quality of life correlates with a good exercise capacity in daily life in patients with tetralogy of Fallot (ToF). Patients after correction of ToF usually develop residual defects such as pulmonary regurgitation or stenosis of variable severity. However, the importance of different hemodynamic parameters and their impact on exercise capacity is unclear. We investigated several hemodynamic parameters measured by cardiovascular magnetic resonance (CMR) and echocardiography and evaluated which parameter has the most pronounced effect on maximal exercise capacity determined by cardiopulmonary exercise testing (CPET). 132 patients with ToF-like hemodynamics were tested during routine follow-up with CMR, echocardiography and CPET. Right and left ventricular volume data, ventricular ejection fraction and pulmonary regurgitation were evaluated by CMR. Echocardiographic pressure gradients in the right ventricular outflow tract and through the tricuspid valve were measured. All data were classified and correlated with the results of CPET evaluations of these patients. The analysis was performed using the Random Forest model. In this way, we calculated the importance of the different hemodynamic variables related to the maximal oxygen uptake in CPET (VO 2 %predicted). Right ventricular pressure showed the most important influence on maximal oxygen uptake, whereas pulmonary regurgitation and right ventricular enddiastolic volume were not important hemodynamic variables to predict maximal oxygen uptake in CPET. Maximal exercise capacity was only very weakly influenced by right ventricular enddiastolic volume and not at all by pulmonary regurgitation in patients with ToF. The variable with the most pronounced influence was the right ventricular pressure.

Aims: It is commonly believed that pulmonary regurgitation (PR) after surgical repair of tetralogy of Fallot (TOF) leads to progressive right ventricular (RV) enlargement. However, progressive RV dilatation has never clearly been documented in this patient population. Therefore, we studied the size of the RV over time in patients after surgical TOF repair., Methods and Results: Fifty-one consecutive patients after surgical TOF repair underwent at least two cardiovascular magnetic resonance (CMR) exams using a single CMR scanner. Patients with RV outflow tract obstruction, interventions other than the initial repair and CMR exams with use of sedation were excluded. Three subgroups with different repair techniques were studied: transannular patch repair [n = 22, age 17 ± 10 years], subvalvular patch repair [n = 15, age 22 ± 8 years], or non-patch repair/infundibulectomy (n = 14, age 28 ± 11 years). Right ventricular end-diastolic volume index (RVEDVI) and PR fraction did not change during the 37 ± 21 months follow-up between first and last CMR in the whole group (RVEDVI: 118 ± 23 mL/m2 vs. 119 ± 23 mL/m2, P = 0.720; PR fraction: 33% (23-40%) vs. 32% (24-39%), P = 0.268). RVEDVI remained stable in all subgroups (transannular patch: 120 ± 21 mL/m2 vs. 122 ± 22 mL/m2, subvalvular patch: 112 ± 23 mL/m2 vs. 111 ± 23 mL/m2, non-patch: 123 ± 28 mL/m2 vs. 123 ± 23 mL/m2, P = 0.827). RVEDVI at last CMR did not differ between groups (P = 0.301)., Conclusions: This study shows no progression of RV dilatation in patients after surgical repair of TOF with moderately dilated RVs and significant PR during a 3-year follow-up. RV dilatation in our patient group seems to be independent from surgical repair techniques., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2016. For permissions, please email: journals.permissions@oup.com.)

Objectives: Patients younger than 8 years are usually examined by cardiovascular magnetic resonance (CMR) under general anesthesia (GA) or sedation without intubation. Therefore, we sought to study the feasibility of CMR in patients aged 3 to 8years without GA or sedation., Patients: Data sets of 71 consecutive patients aged 3 to 8years were studied retrospectively., Design: The total cohort was divided into 2 groups: a no-GA or sedation without intubation group (no-GA or sedation) and a GA or sedation without intubation group (GA or sedation)., Measurements: The patients' age, scan durations for each group, successfully answered clinical question, and number of sequences per study were compared between both groups., Main Results: Scan duration in the no-GA or sedation group (n=44) was 35± 20minutes, and that in the GA or sedation group (n=27) was 60± 31minutes (P<.001). The percentage of successful reports was 95% (42/44) in the no-GA or sedation group and 89% (24 of 27) in the GA or sedation group (P=.29)., Conclusion: CMR in patients aged 3 to 8years is usually successfully feasible without GA or sedation., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Introduction: Velocity offset errors may influence flow measurement in phase-contrast cardiovascular magnetic resonance (CMR). By using a stationary gel phantom, offset errors probably may be corrected. We tested its impact on flow measurement and, in particular, on shunt calculation in patients proven not to have any shunt., Methods: Flow measurements were carried out in 24 patients with congenital heart disease. Baseline correction was performed by using a stationary gel phantom., Results: Significantly more patients without shunts incorrectly showed a calculated shunt after baseline correction., Conclusions: Baseline correction did not improve flow measurement and was clinically not relevant for routine CMR., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Aims: We compared flow and wall shear stress (WSS) patterns in the ascending aorta of individuals with either bicuspid aortic valve (BAV) or tricuspid aortic valve (TAV) using four-dimensional cardiovascular magnetic resonance (4D-CMR). BAV are known to be associated with dilation and dissection of the ascending aorta. However, the cause of vessel disease in patients with BAVs is unknown. Inborn connective tissue disease and also dilation secondary to increased WSS because of altered blood flow patterns in the ascending aorta are discussed as causes for dilation of the aorta. WSS can be estimated non-invasively by 4D-CMR., Methods and Results: Eighteen, otherwise, healthy individuals with functionally normal BAVs were compared prospectively with an age- and sex-matched control group of healthy individuals with TAV. Blood flow data were obtained by 4D-CMR visualization and WSS was calculated with specific software tools. Eighty-five per cent of the individuals with BAVs showed a high-grade helical flow pattern in the ascending aorta compared with 6% of the individuals with TAV. WSS in the ascending aorta was significantly altered in individuals with BAVs compared with TAV., Conclusion: WSS and flow patterns in the ascending aorta in patients with BAVs without concomitant valve or vessel disease are significantly different compared with TAV. The significantly higher shear forces may have an impact on the development of aortic dilation in patients with BAVs.

Background: The size of the functional right ventricle of patients with Ebstein's anomaly is, according to contemporary opinion, small. However, the in vivo size of the functional right ventricle in patients with Ebstein's anomaly is unknown. Furthermore, it is unknown how the apical displacement and regurgitant fraction of the tricuspid valve in Ebstein's anomaly affects the size of the functional right ventricle. Therefore the primary aim of this study was to determine the volume of the functional right ventricle in patients with Ebstein's anomaly. The secondary aim of this study was to assess correlation of the volume of the functional right ventricle to the apical displacement and regurgitant fraction of the tricuspid valve., Methods: Thirty-two consecutive patients with Ebstein's anomaly without previous cardiac surgery or shunts were studied prospectively by cardiovascular magnetic resonance. Functional right ventricle, left ventricle, and atrialized right ventricle volumes and tricuspid valve regurgitation were measured., Results: Functional right ventricle end diastolic volumes were median 127 ml/m² (range: 76-339 ml/m²) and were median 2.5 (range: 1.3-8.8) times larger than the left ventricle volumes. Furthermore, functional right ventricle volumes correlated in a strong positive fashion with tricuspid valve regurgitation (p<0.001, R(2)=0.65) and modestly with the atrialized right ventricle volumes (p=0.027, R(2)=0.16)., Conclusion: Patients with untreated Ebstein's anomaly have large functional right ventricles. The size of the enlarged functional right ventricle seems to depend on the degree of tricuspid valve regurgitation and not on the size of the atrialized right ventricle or the age of the patient., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Background: Optimal ECG triggering is of paramount importance for correct blood flow quantification during cardiovascular magnetic resonance (CMR). However, optimal ECG triggering and therefore blood flow quantification is impaired in many patients with congenital heart disease (CHD) due to complex QRS patterns. Therefore, a new ECG-trigger algorithm was developed to address triggering problems due to complex QRS patterns.The aim of this study was to test this new ECG-trigger algorithm in routine patients with CHD and its impact on blood flow quantification., Methods: 35 consecutive routine patients with CHD undergoing CMR were included in the study. (40% Fallot's Tetralogy, 20% aortic arch pathology, 14% transposition of the great arteries, 26% others; age 26+/-11 yrs).In all patients, blood flow in the ascending aorta was quantified using the old ECG-trigger algorithm and the new ECG-trigger algorithm in random order. Blood flow quantified using the old or new ECG-trigger algorithm was compared by Bland-Altman analysis.Three blinded investigators evaluated the vector clouds and trigger points of both ECG-trigger methods. Evaluation criteria were false positive and false negative triggered QRS complexes (specificity and sensitivity), and accuracy of detection. Accuracy of detection was defined as time scatter of the trigger around the correct trigger point., Results: Specificity, sensitivity, and accuracy of detection significantly increased using the new ECG-trigger algorithm compared to the old ECG-trigger algorithm.Blood flow quantification using the old or new ECG-trigger algorithm differed more than 5% in 31% of the cases., Conclusions: Our results suggest that optimizing ECG triggering during CMR using our new algorithm can avoid errors of >5% in approximately 1/3 of routine patients with congenital heart disease (CHD). We furthermore suggest that incorrect ECG triggering appears to be problematic for blood flow quantification of many patients with CHD undergoing routine CMR.

Purpose: To assess by cardiovascular magnetic resonance (CMR) and CMR tagging if the Amplatzer Septal Occluder affects right ventricular (RV) and left ventricular (LV) motion pattern., Materials and Methods: Sixteen consecutive patients with significant atrial septal defect (ASD) and nine consecutive patients with persistent foramen ovale (PFO) as controls were studied before and a median of 14 days after defect closure by an Amplatzer occluder. By CMR end-diastolic (EDV) and end-systolic (ESV) RV and LV volumes were determined. Aortic and pulmonary artery flow was measured for assessment of left-to-right shunt (Qp/Qs). By CMR tagging circumferential strain and radial shortening, maximal rotation and torsion were measured,, Results: In ASD patients RV-EDV and RV-ESV decreased (P < 0.05). LV-EDV and LV-ESV increased after ASD closure (P < 0.005). Qp/Qs dropped from 1.8 to 1.0 (P < 0.001). PFO patients showed no ventricular volume change after PFO closure. In ASD patients circumferential strain and radial shortening and maximal rotation of the RV decreased by ASD closure (P < 0.01). In LV only maximal rotation at the base and apex decreased significantly (P < 0.05). Torsion remained constant. In PFO patients no tagging parameter changed after defect closure., Conclusion: The Amplatzer occluder itself does not change the ventricular contraction pattern. All volume and myocardial deformation changes were caused by ventricular loading shifts., (Copyright © 2012 Wiley Periodicals, Inc.)

Background: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a very rare disease. Accordingly, the incidence and distribution of myocardial scarring in long-term follow-up of patients after successful surgery of ALCAPA is unknown. Therefore, the aim of this study was to describe the distribution of left ventricular (LV) myocardial scarring by late gadolinium enhancement magnetic resonance in these patients., Methods: Fourteen consecutive patients over 12 years of age with ALCAPA without any further cardiac abnormalities, and after corrective surgery at our center, participated prospectively in this study. All patients underwent cardiac magnetic resonance including LV function analysis and late gadolinium enhancement magnetic resonance. Of these 14 patients, 12 patients also underwent cardiopulmonary exercise testing., Results: Four patients had no evidence of myocardial scarring. The median extent of the myocardial scar of all patients by late gadolinium enhancement magnetic resonance, expressed as a percentage of the total LV myocardial volume, was only 2% (range, 0% to 11%). Median LV ejection fraction was 0.55 (range, 0.31 to 0.73). Median LV end-diastolic volume indexed to body surface area was 84 mL/m(2) (39 to 120 mL/m(2)). Median peak oxygen uptake during cardiopulmonary exercise was 24 mL · kg(-1) · minute(-1) (range, 19 to 51 mL/kg/minute)., Conclusions: We conclude that despite often severely compromised LV function and evidence of scarring before corrective surgery of patients with ALCAPA, in long-term follow-up scar tissue is relatively scarce., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Recent reports provide indirect evidence of myocardial injury and ventricular dysfunction after prolonged exercise. However, existing data is conflicting and lacks direct verification of functional myocardial alterations by CMR [cardiac MR (magnetic resonance)]. The present study sought to examine structural myocardial damage and modification of LV (left ventricular) wall motion by CMR imaging directly after a marathon. Analysis of cTnT (cardiac troponin T) and NT-proBNP (N-terminal pro-brain natriuretic peptide) serum levels, echocardiography [pulsed-wave and TD (tissue Doppler)] and CMR were performed before and after amateur marathon races in 28 healthy males aged 41 ± 5 years. CMR included LGE (late gadolinium enhancement) and myocardial tagging to assess myocardial injury and ventricular motion patterns. Echocardiography indicated alterations of diastolic filling [decrease in E/A (early transmitral diastolic filling velocity/late transmitral diastolic filling velocity) ratio and E' (tissue Doppler early transmitral diastolic filling velocity)] postmarathon. All participants had a significant increase in NT-proBNP and/or cTnT levels. However, we found no evidence of LV LGE. MR tagging demonstrated unaltered radial shortening, circumferential and longitudinal strain. Myocardial rotation analysis, however, revealed an increase of maximal torsion by 18.3% (13.1 ± 3.8 to 15.5 ± 3.6 °; P=0.002) and maximal torsion velocity by 35% (6.8 ± 1.6 to 9.2 ± 2.5 °·s-1; P<0.001). Apical rotation velocity during diastolic filling was increased by 1.23 ± 0.33 °·s-1 after marathon (P<0.001) in a multivariate analysis adjusted for heart rate, whereas peak untwist rate showed no relevant changes. Although marathon running leads to a transient increase of cardiac biomarkers, no detectable myocardial necrosis was observed as evidenced by LGE MRI (MR imaging). Endurance exercise induces an augmented systolic wringing motion of the myocardium and increased diastolic filling velocities. The stress of marathon running seems to be better described as a burden of myocardial overstimulation rather than cardiac injury.

The best method to measure right (RV) and left (LV) ventricle volumes of patients with corrected tetralogy of Fallot is considered cardiac magnetic resonance (CMR). However, to date, no standard protocol to measure RV volumes by CMR exists. RV volumes can be measured from a stack of short-axis slices or a stack of axial slices through the patient's chest. Therefore, the aim of this study was to determine whether short-axis or axial slices are more reliable for routine measurement of RV and LV volumes in patients with corrected tetralogy of Fallot. We studied consecutive patients with corrected tetralogy of Fallot (n = 46) undergoing routine CMR. The end-diastolic and end-systolic RV and LV volumes were measured by 2 investigators unaware of the results of the other measurements using short-axis and axial slices, and the inter- and intraobserver variances were compared. The design of the study was based on the Standards for Reporting of Diagnostic Accuracy. Interobserver variance was significantly smaller using axial slices than using short-axis slices for the RV end-systolic volumes (127.9%(2) vs 315.1%(2); p = 0.003), LV end-diastolic volumes (11.4%(2) vs 36.1%(2); p <0.001), and LV end-systolic volumes (31.9%(2) vs 176.1%(2); p <0.001). Intraobserver variance was significantly smaller using axial slices than using short-axis slices for the RV end-diastolic volumes (26.7%(2) vs 51.1%(2); p = 0.032), LV end-diastolic volumes (11.0%(2) vs 23.5%(2); p = 0.012), and LV end-systolic volumes (34.3%(2) vs 86.1%(2); p = 0.003). In conclusion, axial slices are more reproducible than short-axis slices for measuring ventricular volumes of patients with corrected tetralogy of Fallot by CMR.

Background: Cardiovascular magnetic resonance (CMR) of patients with congenital heart disease (CHD) has become routine clinical practice. However, existing CMR protocols focus predominantly on patients with ischemic heart disease, and information is limited on the types of patient with CHD who benefit from CMR investigation, and in what ways. Therefore the aim of this study was to answer the questions: What type of patients were studied by CMR in a centre specializing in paediatric and adult CHD management? What questions were asked, which protocols were used and were the questions successfully answered? To answer these questions, we conducted a cohort study of all 362 patients that received routine clinical CMR during 2007 at the Department of Paediatric Cardiology and Congenital Heart Disease at the Deutsches Herzzentrum München., Results: Underlying diagnosis was in 33% Fallot's tetralogy, 17% aortic coarctation, 8% Ebstein's disease, 6% Marfan's disease, 4% single ventricle with Fontan-like circulation, and 32% others. Median age was 26 years (7 days - 75 years). Ventricular volumes were assessed in 67% of the patients; flow in 74%; unknown anatomy only in 9%; specific individual morphology of known anatomy in 83%; myocardial fibrosis in 8%; stress-induced myocardial perfusion defects in 1%. Only in 3% of the cases the question could not be fully answered., Conclusion: Contrary to common belief, routine CMR of patients with CHD was not requested to address global anatomical questions so much as to clarify specific questions of morphology and function of known anatomy. The CMR protocols used differed markedly from those widely used in patients with ischemic heart disease.

Background: Patients after atrial switch operation for transposition of the great arteries have limited exercise performance. Rigid atrial baffles may cause a relative preload reduction. Previous studies have had suboptimal control groups, which ideally should consist of patients with congenitally corrected transposition of the great arteries (ccTGA) without previous heart surgery, having a systemic right ventricle, but lacking rigid atrial baffles. Therefore the aim of this study was to test the impact of atrial baffles by comparing 12 atrial switch patients with 11 ccTGA patients., Methods and Results: Systemic right ventricular stroke volume (SV), heart rate, cardiac index, and other parameters were assessed during rest and dobutamine stress magnetic resonance imaging. The most important difference between the groups was that the atrial switch patients could not increase SV during stress, whereas ccTGA patients increased it significantly. There was no difference between groups in the rise of the cardiac index. Heart rate increased significantly more in atrial switch patients than in ccTGA patients., Conclusions: The results support the hypothesis that atrial baffles restrict a rise in SV under dobutamine stress in patients after atrial switch operation for transposition of the great arteries.

Background: After Fontan surgery there is no subpulmonary ventricle to modify pulmonary blood flow. The influence of the cardiac cycle on pulmonary blood flow patterns in various types of Fontan patients is unknown., Methods: Blood flow patterns were investigated using phase-velocity cine magnetic resonance imaging in the pulmonary artery of 17 patients (21.1 +/- 7.3 years old, 6 females) with Fontan circulation. These patterns were compared with those of 12 healthy volunteers (26.3 +/- 6.0 years old, 10 females) obtained in the superior vena cava and the main pulmonary artery. Measurements were sampled for a period of about 3 minutes to rule out respiratory effects. Blood flow patterns were depicted by interpolating the variable number of measured phases in every patient to 100 phases and normalizing flow to mean blood flow in that vessel. Then, average flow patterns were calculated throughout the patient groups to depict a typical pattern., Results: In Fontan patients, peaks and troughs are highly variable. In averaged flow patterns for the whole Fontan group, only a slight late diastolic flow acceleration could be detected. This is in contrast to the pattern of the control subjects in whom typical systolic peaks and late diastolic troughs could be found in both the superior vena cava and in the pulmonary artery., Conclusions: There are no typical pulmonary blood flow patterns of cardiac origin in patients with Fontan circulation, except for slight late diastolic flow acceleration representing diastolic inflow restriction.

Unlabelled: The clinical diagnosis of Marfan syndrome in childhood is difficult, because symptoms may not have developed to their full expression until adulthood. The Ghent nosology for the diagnosis of Marfan syndrome classifies dural ectasia as a major diagnostic criterion. More than two thirds of adult patients with Marfan syndrome show dural ectasia, while the frequency in childhood is unknown. This prospective multicenter observational patient-control study was performed to identify pathologic changes of the lumbosacral spine in young patients with Marfan syndrome., Design: Prospective clinical trial, multicentric, cross-sectional., Setting: MRI of the lumbosacral spine., Patients: Twenty patients with proven Marfan syndrome, 20 patients suspicious for Marfan syndrome and 38 healthy controls., Outcome Measures: Vertebral body diameter (VBD) from L1 to S1, dural sac diameter (DSD) from L1 to S1, dural sac ratio (DSR), qualitative assessment of the lumbosacral spine., Results: DSD and VBD in different age groups were higher in patients with proven or suspected Marfan syndrome than in healthy controls (DSD: L1, 6-8 years, P < 0.05). VBD related to body height showed a similar growth related increase in patients with proven or suspected Marfan syndrome and controls. DSD related to body height was elevated in patients with proven or suspected Marfan syndrome at different levels of the lumbar spine. DSD at levels L1, L5, and S1, and DSR at levels L5 and S1 of patients with proven Marfan syndrome were significantly higher (P < 0.05) than in controls., Conclusion: Even during childhood pathologic changes inside the lumbosacral spine of patients with Marfan syndrome can be observed. Dural ectasia, which occurs at different levels of the lumbar spine, can be detected at levels L5 and S1 in up to 40% of patients with Marfan syndrome., (Copyright 2006 Wiley-Liss, Inc.)

Objectives: To compare images of the aorta obtained with helical CT (HCT) scanning and MRI for the follow-up of adults with coarctation of the aorta (CoA)., Design: Longitudinal study., Setting: Department of adult congenital heart disease in a tertiary university hospital., Patients: A total of 37 adults (age range, 16 to 68 years; women, 13) with CoA (after surgery, 34 patients; native, 2 patients; after balloon-angioplasty, 1 patient), Measurements and Results: All patients underwent both HCT and MRI of the thoracic aorta within a mean (+/- SD) time interval of 1.86 +/- 1.11 years. Aortic diameters measured at six intrathoracic levels showed a high correlation (r = 0.79 to 0.94). On average, slightly lower diameters were measured with MRI (1.2 mm). But there was a substantial variation between the two measurements with differences of up to 9 mm. All other pathomorphologic abnormalities were detected and classified similarly with both methods., Conclusions: HCT and MRI are similarly useful for the noninvasive evaluation of the thoracic aorta in patients with CoA. But there can be a substantial variation in two subsequent measurements without an overall substantial bias toward larger diameter in one of the two methods. In repetitive studies, changes of the diameters should be interpreted with care, especially when assessing the progression of aortic diameters.

Objectives: The purpose of this study was to assess ventricular mass, volume, and systolic function in patients late after Fontan operation by cardiac magnetic resonance imaging., Background: An assessment of determinants for ventricular function in post-Fontan patients was intended., Methods: Twenty-three unselected patients (9 female, 14 male) at a median age of 19.4 years (range, 7.8 to 31.3 years), at a median time of 10.5 years (range, 4.1 to 18 years) after Fontan operation were studied. A standard 1.5-T scanner was used, and analysis was performed using dedicated software. Ten healthy volunteers (median age 26.4, range 18 to 39.3 years) served as the control group., Results: Median end-systolic mass index was 72.2 g/m(2) (range, 43 to 138 g/m(2)) and 86.6 g/m(2) (range, 52 to 123 g/m(2)) in the control group (p = NS). Median end-diastolic ventricular volume was 64 ml/m(2) (range, 32 to 117 ml/m(2)) compared with 67.7 ml/m(2) (range, 59 to 75 ml/m(2)) in the control group (p = NS). Median ejection fraction was 49.3% (range, 20% to 63%) compared with 64.8% (range, 57% to 79%) in normals (p = 0.00001)., Conclusions: We conclude that long-term survivors of a Fontan operation have normal ventricular mass, normal volume, but reduced systolic ventricular function.

Background: Quantitative evaluation of pulmonary perfusion using lung perfusion scintigraphy in patients with atriopulmonary anastomosis (APA) or total cavopulmonary connection (TCPC) or partial cavopulmonary connection (PCPC) is difficult because of preferential draining of the venae cavae to one lung. Scintigraphy is the gold standard. Phase-velocity MRI (PV-MRI) is a new technique for determining pulmonary perfusion. The aim of this study was therefore to determine whether PV-MRI is more accurate than scintigraphy for quantitative evaluation of pulmonary perfusion ratios in patients with APA, TCPC, or PCPC., Methods and Results: We studied 15 patients with APA, TCPC, or PCPC (16+/-7 years old, 4 female). Twelve patients (15+/-8 years old, 3 female) with a single pulmonary blood source supplied by a subpulmonary ventricle, ensuring complete mixing of the radioactive tracer before entering the pulmonary circulation, served as controls. Pulmonary scintigraphy and PV-MRI were performed in all patients. Bland-Altman analysis showed a clinically unacceptable difference of 7.1% right pulmonary blood flow (27.2% upper and -13.0% lower limit of agreement) between the two methods in the study group. The two methods agreed excellently in the control group (difference, 1.6%; 4.0% upper and -7.2% lower limit of agreement), showing that the bad agreement in the study group was caused by the problems encountered using pulmonary scintigraphy in patients with APA, TCPC, or PCPC., Conclusions: Because of preferential caval flow into either lung, PV-MRI is more accurate for evaluating pulmonary perfusion ratios than lung perfusion scintigraphy in patients with Fontan-like circulation.