AIMS:Preeclampsia is a pregnancy disorder characterized by hypertension and multiorgan failure related to endothelial damage. It is associated with increased risk of cardiovascular disease later in life, but pathophysiological mechanisms are unclear, and strategies to identify women at risk are missing. The aim was to investigate cardiovascular impact 5 years after severe preeclampsia, compared to normotensive pregnancies. METHODS:The study was powered to detect approximately 5% difference between groups by cardiac magnetic resonance (CMR). Ten women experiencing severe preeclampsia, and 20 women with normotensive pregnancies were included: 10 lifestyle-matched and 10 physically active. Brachial blood pressure, echocardiography and CMR were performed 4-7 years after pregnancy. RESULTS:There was no difference in systolic or diastolic blood pressure, and all women were normotensive (103 vs. 106 vs. 108 mmHg, p = 0.67; and 66 vs. 66 vs. 64 mmHg, p = 0.78). Indexed left ventricular volumes were larger for active controls than for women with prior preeclampsia (98 vs. 84 mL/m2, p = 0.004; and 41 vs. 32 mL/m2, p = 0.01). Left ventricular mass, cardiac function and pulse wave velocity did not differ. No participant fulfilled the criteria for diastolic dysfunction. CONCLUSION:The difference in left ventricular volumes is most likely related to a physiological adaptation to exercise. The lack of persistent hypertension may be the factor explaining the absence of clinically significant differences between groups using routine investigations for cardiovascular structure and function, despite severe preeclampsia. Structured follow-up is essential to enable early diagnosis of hypertension after preeclampsia, as well as supporting an active and potentially protective lifestyle.

Cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) imaging shows myocardial fibrosis and is associated with outcome in repaired Tetralogy of Fallot (rToF). However, patients with rToF have surgical material implanted, also appearing bright in LGE images. Therefore, right ventricular myocardial fibrosis could be overestimated or falsely diagnosed, which may affect prognostic value and serial examinations. The aim of this study was to assess right ventricular LGE in patients with rToF and to compare this with placement of surgical material. This retrospective cohort study included patients with rToF operated in Lund between 1981–2022 in whom CMR with LGE had been performed. The LGE images were scored by two blinded observers and matched against patient records for surgical material placement. Ventricular volumes were measured in cine CMR images. Fifty-four patients with rToF (median age 18 [13–22] years) with LGE imaging 17 [12–21] years after repair were analyzed. Increased signal intensity in LGE images was only found at the site of surgical material or surgical incision and in right ventricular insertion points. There was no progression of LGE in patients with serial examinations. Knowing the surgical history is vital to avoid misinterpretation of LGE images since presence of surgical material may lead to overestimation or false diagnosis of right ventricular myocardial fibrosis. Serial LGE examinations could likely be avoided in most patients with rToF.

Introduction. The ESCAPER project explores cardiovascular resilience in individuals who, despite a high-risk factor burden-longstanding Type 1 Diabetes (T1D), obesity, or kidney failure-avoid or delay macrovascular complications. This suggests underlying protective mechanisms. Initiated in September 2022, this exploratory study aims to uncover and define these mechanisms, potentially leading to novel therapeutic targets in preventive medicine. Research design and methods. Participants from the Skåne region, Southern Sweden, are divided into three subgroups: (1) T1D patients (>30 years duration) without macrovascular complications or macroalbuminuria, (2) obese individuals with normal cardiac function and no cardiovascular medications, and (3) kidney failure patients awaiting transplantation with no arterial calcification, alongside respective controls. Comprehensive phenotyping includes 24-h blood pressure, ECG monitoring, vascular ultrasound, cardiac MRI, and ergospirometry (in a subgroup), along with laboratory investigations, including biomarker and omics analyses. Arterial biopsies are collected from kidney failure patients. The study leverages Swedish national medical registries for detailed follow-up of healthcare utilization, diagnoses, and prescriptions, enabling longitudinal outcome assessments. Results. Initial findings from 90 T1D patients and 31 obese individuals indicate well-managed cardiovascular risk factors. The T1D subgroup shows a mean BMI of 25.6 kg/m2 and HbA1c of 52 mmol/mol, while the obesity subgroup presents a BMI of 32.9 kg/m2 with normal glucose levels. Conclusions. ESCAPER has the potential to advance understanding of cardiovascular resilience and refine prevention strategies. Its comprehensive methodology and registry-based follow-up provide robust insights into protective mechanisms and long-term outcomes.

A hydraulic force aids diastolic filling of the left ventricle (LV) and is proportional to the difference in short-axis area between the left ventricle and atrium; the atrioventricular area difference (AVAD). Patients with repaired Tetralogy of Fallot (rToF) and pulmonary regurgitation (PR) have reduced LV filling which could lead to a negative AVAD and a hydraulic force impeding diastolic filling. The aim was to assess AVAD and to determine whether the hydraulic force aids or impedes diastolic filling in patients with rToF and PR, compared to controls. Twelve children with rToF (11.5 [9–13] years), 12 pediatric controls (10.5 [9–13] years), 12 adults with rToF (21.5 [19–27] years) and 12 adult controls (24 [21–29] years) were retrospectively included. Cine short-axis images were acquired using cardiac magnetic resonance imaging. Atrioventricular area difference was calculated as the largest left ventricular short-axis area minus the largest left atrial short-axis area at beginning of diastole and end diastole and indexed to height (AVADi). Children and adults with rToF and PR had higher AVADi (0.3 cm2/m [− 1.3 to 0.8] and − 0.6 [− 1.5 to − 0.2]) at beginning of diastole compared to controls (− 2.7 cm2/m [− 4.9 to − 1.7], p = 0.015) and − 3.3 cm2/m [− 3.8 to − 2.8], p = 0.017). At end diastole AVADi did not differ between patients and controls. Children and adults with rToF and pulmonary regurgitation have an atrioventricular area difference that do not differ from controls and thus a net hydraulic force that contributes to left ventricular diastolic filling, despite a small underfilled left ventricle due to pulmonary regurgitation.

BACKGROUND:Cardiac diffusion tensor imaging (cDTI) is an emerging technique for microstructural characterization of the heart and has shown clinical potential in a range of cardiomyopathies. However, there is substantial variation reported for in vivo cDTI results across the literature, and sensitivity of cDTI to differences in imaging sites, scanners, acquisition protocols, and post-processing methods remains incompletely understood. METHODS:SIGNET is a prospective multi-center, observational study in traveling and non-traveling healthy volunteers. The study was initiated by the executive board of the Society of Cardiovascular Magnetic Resonance (SCMR) Cardiac Diffusion Special Interest Group (SIG) as a follow-up to a previous multi-center study on phantom validation of cardiac DTI and a recently published SCMR consensus statement on cardiac diffusion MRI. The study has been developed by the Project Management Committee in consultation with the SCMR cardiac diffusion SIG, which includes international experts in cardiac diffusion MRI. To date, more than 20 international institutions have engaged with the study, including sites that are new to cardiac DTI, making this the largest collaborative effort in the field. DISCUSSION:SIGNET will provide important information about the key sources of variation in cardiac DTI. This will help rationalize strategies for addressing and minimizing such variation. Harmonization of protocols in this and future studies will underpin efforts to translate cardiac DTI for clinical application.

BackgroundRecurrent coarctation of the aorta (re-CoA) is a well-known although not fully understood complication after surgical repair, typically occurring in 10%–20% of cases within months after discharge.ObjectivesTo (1) characterize geometry of the aortic arch and blood flow from pre-discharge magnetic resonance imaging (MRI) in neonates after CoA repair; and (2) compare these measures between patients that developed re-CoA within 12 months after repair and patients who did not.MethodsNeonates needing CoA repair, without associated major congenital heart defects, were included. Transthoracic echocardiography (echo) and 4D phase-contrast MRI were performed prior to discharge after CoA repair to assess 3D arch geometry, flow velocity and flow pattern in the distal aortic arch corresponding to the area at risk for re-CoA. Arch geometry was assessed by measuring angles of the aortic arch and its branches using 3D patient-specific geometries segmented from MRI. Continuous data are presented as median and interquartile range.ResultsThe median age at CoA surgery was 9 days. Four out of the included 28 patients (14%) developed re-CoA within the first 12 months after surgery. Re-CoA was associated with repair technique (lateral thoracotomy 100% vs. 33%, p = 0.02), higher postoperative isthmic flow velocity by echocardiography (1.9 [0. 9] m/s vs. 1.25 [0.5] m/s, p = 0.04) and postoperative crenel aortic arch (100% vs. 21%, p = 0.007) with a larger distance between the first and last branching points (12.6 [3.1] mm vs. 7.3 [7.0] mm; p = 0.01). A smaller angle between the ascending aorta and the brachiocephalic artery (89 [58]° vs. 122 [37]°, p = 0.05) and between the proximal aortic arch and the left carotid artery (75° vs. 97 [37]°, p = 0.04), with a more pronounced caliber change between the ascending aorta and the proximal (1.85 vs. 0.86 [0.76]; p = 0.03) and distal aortic arch (2.19 [2.42] vs. 1.01 [0.94]; p = 0.03) were observed in re-CoA patients. Patients who developed re-CoA had more left-handed helical flow in systole (p = 0.045), more right-handed helical flow in diastole (p = 0.02), and less vortical flow (p = 0.05).ConclusionSubtle changes in aortic arch geometry and flow pattern early after neonatal CoA repair may contribute to the risk of re-CoA.

BACKGROUND:The mitral valve is essential for proper heart function. Patient-specific simulations may provide insights into valve function and provide hemodynamic information for treatment planning. This study presents a framework for patient-specific mitral valve hemodynamic prediction and the validation of the simulation model against echocardiographic and MRI data. METHODS:Ten healthy volunteers (age range/median: 1-37/13 years) underwent echocardiographic exams, of which five also underwent cardiac MRI. Patient-specific mitral valve geometries were segmented from 3D echocardiograms, while mass flow boundary conditions were derived from left ventricular volume data obtained via echocardiography and MRI. The mitral apparatus, including the chordae, was modeled in a simplified left heart and simulated in a computational model using fluid-structure interaction. RESULTS:The simulations captured the valvular behavior and hemodynamics of the left heart throughout the cardiac cycle. We found an average difference of 3.6 ± 29 % and 8.3 ± 22 % in the maximum and mean transvalvular velocity compared to Doppler data. Echocardiography underestimated end-systolic and end-diastolic volumes by 1.8 ± 20 % and 19 ± 3.8 % compared to MRI. The average maximum principal strain over the mitral valve was 5.8 % during systole and 6.7 % during diastole, consistent with literature. CONCLUSIONS:A computational framework for clinically feasible patient-specific prediction of mitral valve hemodynamics was developed and validated in vivo in the largest cohort presented to date. Computational time was acceptable for clinical planning. This is a step towards personalized surgical planning of valve repair and an increased understanding of the hemodynamics and mitral valve function after intervention.

The "nutmeg lung pattern" on fetal magnetic resonance imaging (MRI) indicates pulmonary lymphangiectasia. This is associated with adverse outcomes, particularly in fetuses with congenital heart defects and impaired pulmonary venous return. Whereas lymphedema is common in fetuses with Turner syndrome, pulmonary lymphangiectasia is not. A 26-year-old woman presented with a fetus with hypoplastic left heart syndrome (HLHS) without restrictive atrial septum (RAS). The family declined amniocentesis, yet non-invasive prenatal testing showed an increased risk for Turner syndrome. The patient underwent a fetal MRI as part of a blinded research protocol. Postnatal echocardiogram confirmed the fetal echocardiographic findings without evidence of RAS. Norwood stage I palliation was performed at two days of age. Significant neonatal respiratory morbidity including pneumonia, diaphragmatic and vocal cord pareses and chylothorax occurred. Subsequent review of fetal MRI revealed a prominent thoracic duct and mild pulmonary lymphangiectasia. Turner syndrome was confirmed by genetic testing. After one month, the patient was discharged to her home hospital with respiratory support, which was discontinued a few weeks later. Respiratory problems continued, but the vocal cord paresis resolved over time. This is a unique case of a fetus with HLHS/non-RAS with mild fetal pulmonary lymphangiectasia, and significant neonatal respiratory morbidity, probably in part due to Turner syndrome. The infant survived the neonatal period and underwent uneventful Glenn surgery. The patient's tolerance to the total cavopulmonary connection (TCPC) procedure is yet to be seen. An MRI lymphography should precede it to assess residual lymphatic abnormalities and serve as baseline for post-TCPC changes.

Aims:4D blood flow measurements by cardiac magnetic resonance imaging (CMR) can be used to simplify blood flow assessment. Compressed sensing (CS) can provide better flow measurements than conventional parallel imaging (PI), but clinical validation is needed. This study aimed to validate stroke volume (SV) measurements by 4D-CS in healthy volunteers and patients while also investigating the influence of the CS image reconstruction parameter λ on haemodynamic parameters. Methods and results:Healthy participants (n = 9; 20-62 years) underwent CMR with 2D, 4D-CS, and 4D-PI flow. Patients (n = 30, 17 with congenital heart defect; 2-75 years) had 4D-CS added to their clinical examination. Impact of λ was assessed by reconstructing 4D-CS data for six different λ values. In healthy volunteers, 4D-CS and 4D-PI SV differed by 0.4 ± 6.5 mL [0.6 ± 9.1%; intraclass correlation coefficient (ICC) 0.98], and 4D-CS and 2D flow by 0.9 ± 7.0 mL (0.9 ± 10.6%; ICC 0.98). In patients, 4D-CS and 2D flow differed by -1.3 ± 6.0 mL (-7.2 ± 20%; ICC 0.97). SV was not dependent on λ in patients (P = 0.75) but an increase in λ by 0.001 led to increased differences between 4D-CS and 4D-PI of -0.4% (P = 0.0021) in healthy participants. There were significant differences for ventricular kinetic energy (systole: P < 0.0001; diastole: P < 0.0001) and haemodynamic forces (systole: P < 0.0001; diastole: P < 0.0001), where error increased with increasing λ values in both healthy participants and patients. Conclusion:4D flow CMR with CS can be used clinically to assess SV in paediatric and adult patients. Ventricular kinetic energy and haemodynamic forces are however sensitive to the change in reconstruction parameter λ, and it is therefore important to validate advanced blood flow measurements before comparing data between scanners and centres.

Background The geometrical relationship between atrial and ventricular short‐axis cross‐sectional area determines the hydraulic forces acting on intracardiac blood. This is important for diastolic filling. In patients undergoing heart transplantation (HTx), the left atrium is often enlarged as a result of the standard surgical technique. We hypothesized that diastolic filling in HTx patients is affected by the surgery altering the geometrical relationship between atrium and ventricle. Methods and Results This retrospective, cross‐sectional study included 25 HTx patients (median age, 52 [range, 25–70] years), 15 patients with heart failure with reduced ejection fraction (median age, 63 [range, 52–75] years), 15 patients with heart failure with preserved ejection fraction (median age, 74 [range, 56–82] years), and 15 healthy controls (median age, 64 [range, 58–67] years) who underwent cardiac magnetic resonance imaging. Left ventricular, atrial, and total heart volumes (THV) were obtained. Atrioventricular area difference at end diastole and end systole was calculated as the largest ventricular short‐axis area minus the largest atrial short‐axis area. Left atrial minimum volume normalized for THV (LA min /THV) was larger in HTx patients (median, 0.13 [range, 0.07–0.19]) compared with controls (median, 0.05 [range, 0.03–0.08], P <0.001), whereas left ventricular volume normalized for THV (left ventricular end‐diastolic volume/THV) was similar between HTx and controls (median, 0.19 [range, 0.12–0.24] and median, 0.22 [range, 0.20–0.25], respectively). At end diastole, when atrioventricular area difference reached its largest positive value in controls, 11 HTx patients (44%) had a negative atrioventricular area difference, indicating impaired diastolic filling. Conclusions Diastolic filling is impaired in HTx patients due to an altered geometrical relationship between the left atrium and ventricle. When performing cardiac transplantation, a surgical technique that creates a smaller left atrium may improve diastolic filling by aiding hydraulic forces.

BackgroundChildren with repaired tetralogy of Fallot (rToF) often have pulmonary regurgitation with right ventricular (RV) dilatation and dysfunction, whereas less is known about the effect on the left ventricle (LV). The aim was to investigate LV haemodynamic variables derived from non-invasive pressure-volume loops in children with rToF and how they compare to controls and previous research on adults.Materials and methodsTen children with rToF and pulmonary regurgitation (12 years [10-13], 6 males) and 10 age- and sex-matched healthy controls (12 years [10-14], 6 males) underwent brachial blood pressure in conjunction with cardiac magnetic resonance imaging. Pressure-volume loops were derived by brachial blood pressure together with LV volumes throughout the cardiac cycle in short-axis cine images yielding several haemodynamic variables, including arterial elastance. The RV endocardial border was delineated in end-diastole and end-systole.ResultsChildren with rToF and pulmonary regurgitation had larger RV end-diastolic volume (136 [114-156]) than controls (100 [94-112] ml/m2; p = 0.0015) and smaller LV end-diastolic volume (83 [58-91] ml/m2) than controls (101 [92-110] ml/m2; p = 0.002). Arterial elastance was higher in children with rToF (1.5 [1.3-2.7] mmHg/ml) than in controls (1.1 [1.0-1.5] mmHg/ml; p = 0.02). Heart rate was higher in children with rToF (77 [74-81] bpm) than in controls (69 [65-75] bpm; p = 0.027).ConclusionChildren with rToF had higher arterial elastance and heart rate than controls, likely due to increased sympathetic tone to compensate for impaired LV filling following pulmonary regurgitation. If this contributes to increased risk of adverse cardiovascular and cerebrovascular events remains to be studied.

Photon-counting computed tomography (PCCT) is a new clinical method that may show better diagnostic quality at lower radiation doses than conventional CT. To investigate the diagnostic quality and radiation dose of paediatric cardiovascular PCCT for diagnosis of congenital heart defects at 70 kV and 90 kV. This retrospective assessment included clinical non-gated paediatric PCCT examinations for assessment of congenital heart defects. Radiation doses were recorded, and overall and specific diagnostic quality (1–4) were scored by four paediatric radiologists. Agreement, differences, and trends were assessed by percent rater agreement, intraclass correlation, Mann–Whitney tests, and Jonckheere-Terpstra tests. Seventy children with congenital heart defects were examined at 70 kV (n = 35; age 2 days–16 years; 63