Design and implementation of multicenter pediatric and congenital studies with cardiovascular magnetic resonance: Big data in smaller bodies.

Cardiovascular magnetic resonance (CMR) has become the reference standard for quantitative and qualitative assessment of ventricular function, blood flow, and myocardial tissue characterization. There is a preponderance of large CMR studies and registries in adults; However, similarly powered studies are lacking for the pediatric and congenital heart disease (PCHD) population. To date, most CMR studies in children are limited to small single or multicenter studies, thereby limiting the conclusions that can be drawn. Within the PCHD CMR community, a collaborative effort has been successfully employed to recognize knowledge gaps with the aim to embolden the development and initiation of high-quality, large-scale multicenter research. In this publication, we highlight the underlying challenges and provide a practical guide toward the development of larger, multicenter initiatives focusing on PCHD populations, which can serve as a model for future multicenter efforts.

Impact of 4D-Flow CMR Parameters on Functional Evaluation of Fontan Circulation.

We sought to evaluate the potential clinical role of 4D-flow cardiac magnetic resonance (CMR)-derived energetics and flow parameters in a cohort of patients' post-Fontan palliation. In patients with Fontan circulation who underwent 4D-Flow CMR, streamlines distribution was evaluated, as well a 4D-flow CMR-derived energetics parameters as kinetic energy (KE) and energy loss (EL) normalized by volume. EL/KE index as a marker of flow efficiency was also calculated. Cardiopulmonary exercise test (CPET) was also performed in a subgroup of patients. The population study included 55 patients (mean age 22 ± 11 years). The analysis of the streamlines revealed a preferential distribution of the right superior vena cava flow for the right pulmonary artery (62.5 ± 35.4%) and a mild preferential flow for the left pulmonary artery (52.3 ± 40.6%) of the inferior vena cave-pulmonary arteries (IVC-PA) conduit. Patients with heart failure (HF) presented lower IVC/PA-conduit flow (0.75 ± 0.5 vs 1.3 ± 0.5 l/min/m2, p = 0.004) and a higher mean flow-jet angle of the IVC-PA conduit (39.2 ± 22.8 vs 15.2 ± 8.9, p < 0.001) than the remaining patients. EL/KE index correlates inversely with VO2/kg/min: R: - 0.45, p = 0.01 peak, minute ventilation (VE) R: - 0.466, p < 0.01, maximal voluntary ventilation: R:0.44, p = 0.001 and positively with the physiological dead space to the tidal volume ratio (VD/VT) peak: R: 0.58, p < 0.01. From our data, lower blood flow in IVC/PA conduit and eccentric flow was associated with HF whereas higher EL/KE index was associated with reduced functional capacity and impaired lung function. Larger studies are needed to confirm our results and to further improve the prognostic role of the 4D-Flow CMR in this challenging population.

Organisation of paediatric echocardiography laboratories and governance of echocardiography services and training in Europe: current status, disparities, and potential solutions. A survey from the Association for European Paediatric and Congenital Cardiology (AEPC) imaging working group.

BACKGROUND: There is limited data on the organisation of paediatric echocardiography laboratories in Europe. METHODS: A structured and approved questionnaire was circulated across all 95 Association for European Paediatric and Congenital Cardiology affiliated centres. The aims were to evaluate: (1) facilities in paediatric echocardiography laboratories across Europe, (2) accredited laboratories, (3) medical/paramedical staff employed, (4) time for echocardiographic studies and reporting, and (5) training, teaching, quality improvement, and research programs. RESULTS: Respondents from forty-three centres (45%) in 22 countries completed the survey. Thirty-six centres (84%) have a dedicated paediatric echocardiography laboratory, only five (12%) of which reported they were European Association of Cardiovascular Imaging accredited. The median number of echocardiography rooms was three (range 1-12), and echocardiography machines was four (range 1-12). Only half of all the centres have dedicated imaging physiologists and/or nursing staff, while the majority (79%) have specialist imaging cardiologist(s). The median (range) duration of time for a new examination was 45 (20-60) minutes, and for repeat examination was 20 (5-30) minutes. More than half of respondents (58%) have dedicated time for reporting. An organised training program was present in most centres (78%), 44% undertake quality assurance, and 79% perform research. Guidelines for performing echocardiography were available in 32 centres (74%). CONCLUSION: Facilities, staffing levels, study times, standards in teaching/training, and quality assurance vary widely across paediatric echocardiography laboratories in Europe. Greater support and investment to facilitate improvements in staffing levels, equipment, and governance would potentially improve European paediatric echocardiography laboratories.

4D Flow cardiovascular magnetic resonance consensus statement: 2023 update.

Hemodynamic assessment is an integral part of the diagnosis and management of cardiovascular disease. Four-dimensional cardiovascular magnetic resonance flow imaging (4D Flow CMR) allows comprehensive and accurate assessment of flow in a single acquisition. This consensus paper is an update from the 2015 '4D Flow CMR Consensus Statement'. We elaborate on 4D Flow CMR sequence options and imaging considerations. The document aims to assist centers starting out with 4D Flow CMR of the heart and great vessels with advice on acquisition parameters, post-processing workflows and integration into clinical practice. Furthermore, we define minimum quality assurance and validation standards for clinical centers. We also address the challenges faced in quality assurance and validation in the research setting. We also include a checklist for recommended publication standards, specifically for 4D Flow CMR. Finally, we discuss the current limitations and the future of 4D Flow CMR. This updated consensus paper will further facilitate widespread adoption of 4D Flow CMR in the clinical workflow across the globe and aid consistently high-quality publication standards.

Multi-morbidity and blood pressure trajectories in hypertensive patients: A multiple landmark cohort study.

BACKGROUND: Our knowledge of how to better manage elevated blood pressure (BP) in the presence of comorbidities is limited, in part due to exclusion or underrepresentation of patients with multiple chronic conditions from major clinical trials. We aimed to investigate the burden and types of comorbidities in patients with hypertension and to assess how such comorbidities and other variables affect BP levels over time. METHODS AND FINDINGS: In this multiple landmark cohort study, we used linked electronic health records from the United Kingdom Clinical Practice Research Datalink (CPRD) to compare systolic blood pressure (SBP) levels in 295,487 patients (51% women) aged 61.5 (SD = 13.1) years with first recorded diagnosis of hypertension between 2000 and 2014, by type and numbers of major comorbidities, from at least 5 years before and up to 10 years after hypertension diagnosis. Time-updated multivariable linear regression analyses showed that the presence of more comorbidities was associated with lower SBP during follow-up. In hypertensive patients without comorbidities, mean SBP at diagnosis and at 10 years were 162.3 mm Hg (95% confidence interval [CI] 162.0 to 162.6) and 140.5 mm Hg (95% CI 140.4 to 140.6), respectively; in hypertensive patients with ≥5 comorbidities, these were 157.3 mm Hg (95% CI 156.9 to 157.6) and 136.8 mm Hg (95% 136.4 to 137.3), respectively. This inverse association between numbers of comorbidities and SBP was not specific to particular types of comorbidities, although associations were stronger in those with preexisting cardiovascular disease. Retrospective analysis of recorded SBP showed that the difference in mean SBP 5 years before diagnosis between those without and with ≥5 comorbidities was -9 mm Hg (95% CI -9.7 to -8.3), suggesting that mean recorded SBP already differed according to the presence of comorbidity before baseline. Within 1 year after the diagnosis, SBP substantially declined, but subsequent SBP changes across comorbidity status were modest, with no evidence of a more rapid decline in those with more or specific types of comorbidities. We identified factors, such as prescriptions of antihypertensive drugs and frequency of healthcare visits, that can explain SBP differences according to numbers or types of comorbidities, but these factors only partly explained the recorded SBP differences. Nevertheless, some limitations have to be considered including the possibility that diagnosis of some conditions may not have been recorded, varying degrees of missing data inherent in analytical datasets extracted from routine health records, and greater measurement errors in clinical measurements taken in routine practices than those taken in well-controlled clinical study settings. CONCLUSIONS: BP levels at which patients were diagnosed with hypertension varied substantially according to the presence of comorbidities and were lowest in patients with multi-morbidity. Our findings suggest that this early selection bias of hypertension diagnosis at different BP levels was a key determinant of long-term differences in BP by comorbidity status. The lack of a more rapid decline in SBP in those with multi-morbidity provides some reassurance for BP treatment in these high-risk individuals.

Myocardial involvement in children with post-COVID multisystem inflammatory syndrome: a cardiovascular magnetic resonance based multicenter international study-the CARDOVID registry.

BACKGROUND: Recent evidence shows an association between coronavirus disease 2019 (COVID-19) infection and a severe inflammatory syndrome in children. Cardiovascular magnetic resonance (CMR) data about myocardial injury in children are limited to small cohorts. The aim of this multicenter, international registry is to describe clinical and cardiac characteristics of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 using CMR so as to better understand the real extent of myocardial damage in this vulnerable cohort. METHODS AND RESULTS: Hundred-eleven patients meeting the World Health Organization criteria for MIS-C associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), having clinical cardiac involvement and having received CMR imaging scan were included from 17 centers. Median age at disease onset was 10.0 years (IQR 7.0-13.8). The majority of children had COVID-19 serology positive (98%) with 27% of children still having both, positive serology and polymerase chain reaction (PCR). CMR was performed at a median of 28 days (19-47) after onset of symptoms. Twenty out of 111 (18%) patients had CMR criteria for acute myocarditis (as defined by the Lake Louise Criteria) with 18/20 showing subepicardial late gadolinium enhancement (LGE). CMR myocarditis was significantly associated with New York Heart Association class IV (p = 0.005, OR 6.56 (95%-CI 1.87-23.00)) and the need for mechanical support (p = 0.039, OR 4.98 (95%-CI 1.18-21.02)). At discharge, 11/111 (10%) patients still had left ventricular systolic dysfunction. CONCLUSION: No CMR evidence of myocardial damage was found in most of our MIS-C cohort. Nevertheless, acute myocarditis is a possible manifestation of MIS-C associated with SARS-CoV-2 with CMR evidence of myocardial necrosis in 18% of our cohort. CMR may be an important diagnostic tool to identify a subset of patients at risk for cardiac sequelae and more prone to myocardial damage. CLINICAL TRIAL REGISTRATION: The study has been registered on ClinicalTrials.gov, Identifier NCT04455347, registered on 01/07/2020, retrospectively registered.

Fast-track virtual reality for cardiac imaging in congenital heart disease.

BACKGROUND AND AIM OF THE STUDY: We sought to evaluate the appropriateness of cardiac anatomy renderings by a new virtual reality (VR) technology, entitled DIVA, directly applicable to raw magnetic resonance imaging (MRI) data without intermediate segmentation steps in comparison to standard three-dimensional (3D) rendering techniques (3D PDF and 3D printing). Differences in post-processing times were also evaluated. METHODS: We reconstructed 3D (STL, 3D-PDF, and 3D printed ones) and VR models of three patients with different types of complex congenital heart disease (CHD). We then asked a senior pediatric heart surgeon to compare and grade the results obtained. RESULTS: All anatomical structures were well visualized in both VR and 3D PDF/printed models. Ventricular-arterial connections and their relationship with the great vessels were better visualized with the VR model (Case 2); aortic arch anatomy and details were also better visualized by the VR model (Case 3). The median post-processing time to get VR models using DIVA was 5 min in comparison to 8 h (range 8-12 h including printing time) for 3D models (PDF/printed). CONCLUSIONS: VR directly applied to non-segmented 3D-MRI data set is a promising technique for 3D advanced modeling in CHD. It is systematically more consistent and faster when compared to standard 3D-modeling techniques.

Society for Cardiovascular Magnetic Resonance (SCMR) recommended CMR protocols for scanning patients with active or convalescent phase COVID-19 infection.

The aim of this document is to provide specific recommendations on the use of cardiovascular magnetic resonance (CMR) protocols in the era of the COVID-19 pandemic. In patients without COVID-19, standard CMR protocols should be used based on clinical indication as usual. Protocols used in patients who have known / suspected active COVID-19 or post COVID-19 should be performed based on the specific clinical question with an emphasis on cardiac function and myocardial tissue characterization. Short and dedicated protocols are recommended.

Deep learning for electronic health records: A comparative review of multiple deep neural architectures.

Despite the recent developments in deep learning models, their applications in clinical decision-support systems have been very limited. Recent digitalisation of health records, however, has provided a great platform for the assessment of the usability of such techniques in healthcare. As a result, the field is starting to see a growing number of research papers that employ deep learning on electronic health records (EHR) for personalised prediction of risks and health trajectories. While this can be a promising trend, vast paper-to-paper variability (from data sources and models they use to the clinical questions they attempt to answer) have hampered the field's ability to simply compare and contrast such models for a given application of interest. Thus, in this paper, we aim to provide a comparative review of the key deep learning architectures that have been applied to EHR data. Furthermore, we also aim to: (1) introduce and use one of the world's largest and most complex linked primary care EHR datasets (i.e., Clinical Practice Research Datalink, or CPRD) as a new asset for training such data-hungry models; (2) provide a guideline for working with EHR data for deep learning; (3) share some of the best practices for assessing the "goodness" of deep-learning models in clinical risk prediction; (4) and propose future research ideas for making deep learning models more suitable for the EHR data. Our results highlight the difficulties of working with highly imbalanced datasets, and show that sequential deep learning architectures such as RNN may be more suitable to deal with the temporal nature of EHR.

Idiopathic, heritable and veno-occlusive pulmonary arterial hypertension in childhood: computed tomography angiography features in the initial assessment of the disease.

BACKGROUND: In children, idiopathic and heritable pulmonary arterial hypertension present echocardiographic and heart catheterization findings similar to findings in pulmonary veno-occlusive disease. OBJECTIVE: To provide a systematic analysis of CT angiography anomalies in children with idiopathic or heritable pulmonary arterial hypertension, or pulmonary veno-occlusive disease. We also sought to identify correlations between CT findings and patients' baseline characteristics. MATERIALS AND METHODS: We retrospectively analyzed CT features of children with idiopathic and heritable pulmonary arterial hypertension or pulmonary veno-occlusive disease and 30 age-matched controls between 2008 and 2014. We compared CT findings and patient characteristics, including gene mutation type, and disease outcome until 2017. RESULTS: The pulmonary arterial hypertension group included idiopathic (n=15) and heritable pulmonary arterial hypertension (n=11) and pulmonary veno-occlusive disease (n=4). Median age was 6.5 years. Children with pulmonary arterial hypertension showed enlargement of pulmonary artery and right cardiac chambers. A threshold for the ratio between the pulmonary artery and the ascending aorta of ≥1.2 had a sensitivity of 90% and a specificity of 100% for pulmonary arterial hypertension. All children with pulmonary veno-occlusive disease had thickened interlobular septa, centrilobular ground-glass opacities, and lymphadenopathy. In children with idiopathic and heritable pulmonary arterial hypertension, presence of intrapulmonary neovessels and enlargement of the right atrium were correlated with higher mean pulmonary artery pressure (P=0.011) and pulmonary vascular resistance (P=0.038), respectively. Mediastinal lymphadenopathy was associated with disease worsening within the first 2 years of follow-up (P=0.024). CONCLUSION: CT angiography could contribute to early diagnosis and prediction of severity in children with pulmonary arterial hypertension.

Aortic atresia and interrupted aortic arch communicating through external carotid anastomosis.

We describe the case of a newborn girl who displayed association of aortic atresia and interrupted aortic arch, with retrograde flow in ascending aorta, through extracranial anastomoses between vertebral arteries (arisen from descending aorta) and external carotids.

Predicting the risk of emergency admission with machine learning: Development and validation using linked electronic health records.

BACKGROUND: Emergency admissions are a major source of healthcare spending. We aimed to derive, validate, and compare conventional and machine learning models for prediction of the first emergency admission. Machine learning methods are capable of capturing complex interactions that are likely to be present when predicting less specific outcomes, such as this one. METHODS AND FINDINGS: We used longitudinal data from linked electronic health records of 4.6 million patients aged 18-100 years from 389 practices across England between 1985 to 2015. The population was divided into a derivation cohort (80%, 3.75 million patients from 300 general practices) and a validation cohort (20%, 0.88 million patients from 89 general practices) from geographically distinct regions with different risk levels. We first replicated a previously reported Cox proportional hazards (CPH) model for prediction of the risk of the first emergency admission up to 24 months after baseline. This reference model was then compared with 2 machine learning models, random forest (RF) and gradient boosting classifier (GBC). The initial set of predictors for all models included 43 variables, including patient demographics, lifestyle factors, laboratory tests, currently prescribed medications, selected morbidities, and previous emergency admissions. We then added 13 more variables (marital status, prior general practice visits, and 11 additional morbidities), and also enriched all variables by incorporating temporal information whenever possible (e.g., time since first diagnosis). We also varied the prediction windows to 12, 36, 48, and 60 months after baseline and compared model performances. For internal validation, we used 5-fold cross-validation. When the initial set of variables was used, GBC outperformed RF and CPH, with an area under the receiver operating characteristic curve (AUC) of 0.779 (95% CI 0.777, 0.781), compared to 0.752 (95% CI 0.751, 0.753) and 0.740 (95% CI 0.739, 0.741), respectively. In external validation, we observed an AUC of 0.796, 0.736, and 0.736 for GBC, RF, and CPH, respectively. The addition of temporal information improved AUC across all models. In internal validation, the AUC rose to 0.848 (95% CI 0.847, 0.849), 0.825 (95% CI 0.824, 0.826), and 0.805 (95% CI 0.804, 0.806) for GBC, RF, and CPH, respectively, while the AUC in external validation rose to 0.826, 0.810, and 0.788, respectively. This enhancement also resulted in robust predictions for longer time horizons, with AUC values remaining at similar levels across all models. Overall, compared to the baseline reference CPH model, the final GBC model showed a 10.8% higher AUC (0.848 compared to 0.740) for prediction of risk of emergency admission within 24 months. GBC also showed the best calibration throughout the risk spectrum. Despite the wide range of variables included in models, our study was still limited by the number of variables included; inclusion of more variables could have further improved model performances. CONCLUSIONS: The use of machine learning and addition of temporal information led to substantially improved discrimination and calibration for predicting the risk of emergency admission. Model performance remained stable across a range of prediction time windows and when externally validated. These findings support the potential of incorporating machine learning models into electronic health records to inform care and service planning.

Neonatal management and outcomes of prenatally diagnosed CHDs.

OBJECTIVES: The aim of this study was to determine the probability of intervention at birth after prenatal diagnosis of CHD. METHODS: A 10-year retrospective study including all foetuses with a prenatally diagnosed CHD and those delivered in a tertiary-care cardiac centre between January, 2002 and December, 2011 was carried out. Patients were classified into eight groups according to the anticipated risk of neonatal intervention. RESULTS: The need for urgent intervention and/or PGE1 infusion within the first 48 hours of life was 47% (n=507/1080): 72% (n=248) for CHD at risk for a Rashkind procedure, 77% (n=72) for CHD with ductal-dependent pulmonary flow, 13% (n=22) for CHD with potentially ductal-dependent pulmonary flow, 94% (n=62) for CHD with ductal-dependent systemic flow, 29% (n=88) for CHD with potentially ductal-dependant systemic flow, 50% (n=4) for total anomalous pulmonary venous connection, and 17% (n=1) for CHD with atrio-ventricular block. In all, 34% of the patients received PGE1 infusion and 21.4% underwent urgent catheter-based or surgical interventions; 10% of patients without anticipated risk (n=10) underwent an early intervention; 6.7% (n=73) of the patients died; and 55% (n=589) had an intervention before discharge from hospital. CONCLUSION: Half of the neonates with foetal CHD benefited from an urgent intervention or PGE1 infusion at birth. We recommend scheduled delivery and in utero transfer for transposition of the great arteries, double-outlet right ventricle with sub-pulmonary ventricular septal defect, total anomalous pulmonary venous connection, CHD with atrio-ventricular block with heart rate <50, all ductal-dependant lesions, and CHD with potentially ductal-dependant systemic flow.

Spontaneous resorption of a herniated cervical disc in a dog detected by magnetic resonance imaging.

This report describes, for the first time in small animal literature, the spontaneous resorption of herniated Hansen type I intervertebral disc material in the cervical spine of a chondrodystrophic dog over a 4-month period, documented by magnetic resonance imaging. Clinical signs (cervical hyperpathia) responded to conservative treatment during the same period.

Résorption spontanée d'une hernie discale chez un chien détectée par imagerie par résonance magnétique. Cet article décrit, pour la première fois dans la littérature des petits animaux, la résorption spontanée d'une hernie Hansen de type I du matériel du disque intervertébral dans la colonne cervicale d'un chien chondrodystrophique pendant une période de 4 mois et documentée par imagerie par résonance magnétique (IRM). Les signes cliniques (hyperpathie cervicale) ont répondu à un traitement conservateur durant la même période.(Traduit par Isabelle Vallières).

Left Ventricular Mass Indexing in Infants, Children, and Adolescents: A Simplified Approach for the Identification of Left Ventricular Hypertrophy in Clinical Practice.

OBJECTIVE: To determine a simplified method to identify presence of left ventricular hypertrophy (LVH) in pediatric populations because the relationship between heart growth and body growth in children has made indexing difficult for younger ages. STUDY DESIGN: Healthy children (n = 400; 52% boys, 0-18 years of age) from 2 different European hospitals were studied to derive a simplified formula. Left ventricular mass (LVM) was calculated according to the Devereux formula. The derived approach to index LVM was tested on a validation cohort of 130 healthy children from a different hospital center. RESULTS: There was a strong nonlinear correlation between height and LVM. LVM was best related to height to a power of 2.16 with a correction factor of 0.09. Analysis of residuals for LVM/[(height(2.16)) + 0.09] showed an homoscedastic distribution in both sexes throughout the entire height range. A partition value of 45 g/m(2.16) was defined as the upper normal limit for LVM index. As opposed to formula suggested by current guidelines (ie, LVM/height(2.7)) when applying the proposed approach in the validation cohort of 130 healthy participants, no false positives for LVH were found (0% vs 8%; P < .01). CONCLUSIONS: Our data support the possibility to have a single partition (ie, 45 g/m(2.16)) value across the whole pediatric age range to identify LVH, without the time-consuming need of computing specific percentiles for height and sex.

Radiation dose reduction in paediatric coronary computed tomography: assessment of effective dose and image quality.

OBJECTIVES: To assess the impact of different protocols on radiation dose and image quality for paediatric coronary computed tomography (cCT). MATERIALS AND METHODS: From January-2012 to June-2014, 140 children who underwent cCT on a 64-slice scanner were included. Two consecutive changes in imaging protocols were performed: 1) the use of adaptive statistical iterative reconstruction (ASIR); 2) the optimization of acquisition parameters. Effective dose (ED) was calculated by conversion of the dose-length product. Image quality was assessed as excellent, good or with significant artefacts. RESULTS: Patients were divided in three age groups: 0-4, 5-7 and 8-18 years. The use of ASIR combined to the adjustment of scan settings allowed a reduction in the median ED of 58 %, 82 % and 85 % in 0-4, 5-7 and 8-18 years group, respectively (7.3 ± 1.4 vs 3.1 ± 0.7 mSv, 5.5 ± 1.6 vs 1 ± 1.9 mSv and 5.3 ± 5.0 vs 0.8 ± 2.0 mSv, all p < 0,05). Prospective protocol was used in 51 % of children. The reduction in radiation dose was not associated with reduction in diagnostic image quality as assessed by the frequency of coronary segments with excellent or good image quality (88 %). CONCLUSIONS: cCT can be obtained at very low radiation doses in children using ASIR, and prospective acquisition with optimized imaging parameters. KEY POINTS: • Using ASIR allows 25 % to 41 % reduction in the ED. • Prospective protocol is used up to 51 % of children after premedication. • Low dose is possible using ASIR and optimized prospective paediatric cCT.

Problems in the diagnosis of discordant atrioventricular with concordant ventriculo-arterial connections: anatomical considerations, surgical management, and long-term outcome.

BACKGROUND: Discordant atrioventricular with concordant ventriculo-arterial connections is a rare cardiac defect. When isolated, the haemodynamics resemble transposition of the great arteries. In complex heart defects such as heterotaxy, haemodynamics guide the surgical approach. OBJECTIVE: To report a series of eight patients with discordant atrioventricular and concordant ventriculo-arterial connections focussing on anatomical and diagnostic difficulties, surgical management, and follow-up. METHODS: A retrospective review was carried out from 1983 to 2013. Anatomical description was based on segmental analysis. Emphasis was placed on the venoatrial connections. RESULTS: Segmental arrangement was {I, D, S} in six patients, all with spiralling great vessels. There were two patients with parallel great vessels of whom one had {S, L, D} and the other had {S, L, A} arrangement. Of eight patients, five had heterotaxy syndrome. Median age at repair surgery was 1.4 years (with a range from 1.1 months to 8.1 years). The repair surgery finally performed was the atrial switch procedure in seven out of eight patients. The main post-operative complications were two cases of baffle obstruction and one sick sinus syndrome needing pacemaker implantation. There were two early post-operative deaths and six late survivors. Median follow-up was 4.2 years (with a range from 3.9 to 26.7 years) with good functional status in all survivors. Discussion Diagnosing discordant atrioventricular with concordant ventriculo-arterial connections remains challenging. There are ongoing controversies about the definition of atrial morphology and heterotaxy syndrome animating the anatomic discussion of these complex heart defects. Haemodynamically, the atrial switch procedure is the surgical method of choice with an encouraging long-term follow-up despite rhythm disturbances and baffle obstruction.

Comparative analysis of left ventricle function and deformation imaging in short and long axis plane in cardiac magnetic resonance imaging.

Background: Advancements in cardiac imaging have revolutionized our understanding of ventricular contraction. While ejection fraction (EF) is still the gold standard parameter to assess left ventricle (LV) function, strain imaging (SI) has provided valuable insights into ventricular mechanics. The lack of an integrative method including SI parameters in a single, validated formula may limit its use. Our aim was to compare different methods for evaluating global circumferential strain (GCS) and their relationship with global longitudinal strain (GLS) and EF in CMR and how the different evaluations fit in the theoretical relationship between EF and global strain. Methods: Retrospective monocenter study. Inclusion of every patient who underwent a CMR during a 15 months period with various clinical indication (congenital heart defect, myocarditis, cardiomyopathy). A minimum of three LV long-axis planes and a stack of short-axis slices covering the LV using classical steady-state free precession cine sequences. A single assessment of GLS on long axis (LAX) slices and a double assessment of GCS and EF with both short axis (SAX) and LAX slices were made by a single experienced CMR investigator. Results: GCS-SAX and GCS-LAX were correlated (r = 0.77, P < 0.001) without being interchangeable with a high reproducibility for GCS, GLS and EF. EF calculated from LAX images showed an overestimation compared to EF derived from SAX images of 7%. The correlation between calculated EF and theoretical EF derived from SI was high (r = 0.88 with EF-SAX, 0.95 with EF-LAX). Data conclusion: This study highlights the need to integrate strain imaging techniques into clinical by incorporating strain parameters into EF calculations, because it gives a deeper understanding of cardiac mechanics.