Trajectories of functional and structural myocardial parameters in post-COVID-19 syndrome-insights from mid-term follow-up by cardiovascular magnetic resonance.

Introduction: Myocardial tissue alterations in patients with post-Coronavirus disease 2019 syndrome (PCS) are often subtle and mild. Reports vary in the prevalence of non-ischemic and ischemic injuries as well as the extent of ongoing myocardial inflammation. The exact relevance of these myocardial alterations is not fully understood. This study aimed at describing the trajectories of myocardial alterations in PCS patients by mid-term follow-up with cardiovascular magnetic resonance (CMR). Methods: This study entails a retrospective analysis of symptomatic PCS patients referred for follow-up CMR between August 2020 and May 2023 due to mildly affected or reduced left or right ventricular function (LV and RV, respectively) and structural myocardial alterations, e.g., focal and diffuse fibrosis, on baseline scans. Follow-up CMR protocol consisted of cine images and full coverage native T1 and T2 mapping. Baseline and follow-up scans were compared using t-tests or Wilcoxon tests. Post-hoc analysis was carried out in a subgroup based on the change of LV stroke volume (SV) between scans. Results: In total, 43 patients [median age (interquartile range) 46 (37-56) years, 33 women] received follow-ups 347 (167-651) days after initial diagnosis. A decrease in symptoms was recorded on follow-ups (p < 0.03) with 23 patients being asymptomatic at follow-ups [symptomatic at baseline 43/43 (100%) vs. symptomatic at follow-up 21/43 (49%), p < 0.001]. Functional improvement was noted for LV-SV [83.3 (72.7-95.0) vs. 84.0 (77.0-100.3) ml; p = 0.045], global radial [25.3% (23.4%-27.9%) vs. 27.4% (24.4%-33.1%); p < 0.001], and circumferential strains [-16.5% (-17.5% to -15.6%) vs. -17.2% (-19.5% to -16.1%); p < 0.001]. In total, 17 patients had an LV-SV change >10% on follow-up scans (5 with a decrease and 12 with an increase), with LV-SV, RV-SV, and global longitudinal strain being discriminatory variables on baseline scans (p = 0.01, 0.02, and 0.04, respectively). T1- or T2-analysis revealed no changes, remaining within normal limits. Conclusion: Symptomatic load as well as blood pressures decreased on follow-up. CMR did not detect significant changes in tissue parameters; however, volumetric, specifically LV-SV, and deformation indexes improved during mid-term follow-up.

Reproducibility assessment of rapid strains in cardiac MRI: Insights and recommendations for clinical application.

PURPOSE: Studies have shown the incremental value of strain imaging in various cardiac diseases. However, reproducibility and generalizability has remained an issue of concern. To overcome this, simplified algorithms such as rapid atrioventricular strains have been proposed. This multicenter study aimed to assess the reproducibility of rapid strains in a real-world setting and identify potential predictors for higher interobserver variation. METHODS: A total of 4 sites retrospectively identified 80 patients and 80 healthy controls who had undergone cardiac magnetic resonance imaging (CMR) at their respective centers using locally available scanners with respective field strengths and imaging protocols. Strain and volumetric parameters were measured at each site and then independently re-evaluated by a blinded core lab. Intraclass correlation coefficients (ICC) and Bland-Altman plots were used to assess inter-observer agreement. In addition, backward multiple linear regression analysis was performed to identify predictors for higher inter-observer variation. RESULTS: There was excellent agreement between sites in feature-tracking and rapid strain values (ICC ≥ 0.96). Bland-Altman plots showed no significant bias. Bi-atrial feature-tracking and rapid strains showed equally excellent agreement (ICC ≥ 0.96) but broader limits of agreement (≤18.0 % vs. ≤3.5 %). Regression analysis showed that higher field strength and lower temporal resolution (>30 ms) independently predicted reduced interobserver agreement for bi-atrial strain parameters (ß = 0.38, p = 0.02 for field strength and ß = 0.34, p = 0.02 for temporal resolution). CONCLUSION: Simplified rapid left ventricular and bi-atrial strain parameters can be reliably applied in a real-world multicenter setting. Due to the results of the regression analysis, a minimum temporal resolution of 30 ms is recommended when assessing atrial deformation.

Sex-specific structural and functional cardiac remodeling during healthy aging assessed by cardiovascular magnetic resonance.

BACKGROUND: Aging as a major non-modifiable cardiac risk factor challenges future cardiovascular medicine and economic demands, which requires further assessments addressing physiological age-associated cardiac changes. OBJECTIVES: Using cardiovascular magnetic resonance (CMR), this study aims to characterize sex-specific ventricular adaptations during healthy aging. METHODS: The population included healthy volunteers who underwent CMR at 1.5 or 3 Tesla scanners applying cine-imaging with a short-axis coverage of the left (LV) and right (RV) ventricle. The cohort was divided by sex (female and male) and age (subgroups in years): 1 (19-29), 2 (30-39), 3 (40-49), and 4 (≥50). Cardiac adaptations were quantitatively assessed by CMR indices. RESULTS: After the exclusion of missing or poor-quality CMR datasets or diagnosed disease, 140 of 203 volunteers were part of the final analysis. Women generally had smaller ventricular dimensions and LV mass, but higher biventricular systolic function. There was a significant age-associated decrease in ventricular dimensions as well as a significant increase in LV mass-to-volume ratio (LV-MVR, concentricity) in both sexes (LV-MVR in g/ml: age group 1 vs. 4: females 0.50 vs. 0.57, p=0.016, males 0.56 vs. 0.67, p=0.024). LV stroke volume index decreased significantly with age in both sexes, but stronger for men than for women (in ml/m2: age group 1 vs. 4: females 51.76 vs. 41.94, p<0.001, males 55.31 vs. 40.78, p<0.001). Ventricular proportions (RV-to-LV-volume ratio) were constant between the age groups in both sexes. CONCLUSIONS: In both sexes, healthy aging was associated with an increase in concentricity and a decline in ventricular dimensions. Furthermore, relevant age-related sex differences in systolic LV performance were observed.

Post-hoc standardisation of parametric T1 maps in cardiovascular magnetic resonance imaging: a proof-of-concept.

BACKGROUND: In cardiovascular magnetic resonance imaging parametric T1 mapping lacks universally valid reference values. This limits its extensive use in the clinical routine. The aim of this work was the introduction of our self-developed Magnetic Resonance Imaging Software for Standardization (MARISSA) as a post-hoc standardisation approach. METHODS: Our standardisation approach minimises the bias of confounding parameters (CPs) on the base of regression models. 214 healthy subjects with 814 parametric T1 maps were used for training those models on the CPs: age, gender, scanner and sequence. The training dataset included both sex, eleven different scanners and eight different sequences. The regression model type and four other adjustable standardisation parameters were optimised among 240 tested settings to achieve the lowest coefficient of variation, as measure for the inter-subject variability, in the mean T1 value across the healthy test datasets (HTE, N = 40, 156 T1 maps). The HTE were then compared to 135 patients with left ventricular hypertrophy including hypertrophic cardiomyopathy (HCM, N = 112, 121 T1 maps) and amyloidosis (AMY, N = 24, 24 T1 maps) after applying the best performing standardisation pipeline (BPSP) to evaluate the diagnostic accuracy. FINDINGS: The BPSP reduced the COV of the HTE from 12.47% to 5.81%. Sensitivity and specificity reached 95.83% / 91.67% between HTE and AMY, 71.90% / 72.44% between HTE and HCM, and 87.50% / 98.35% between HCM and AMY. INTERPRETATION: Regarding the BPSP, MARISSA enabled the comparability of T1 maps independently of CPs while keeping the discrimination of healthy and patient groups as found in literature. FUNDING: This study was supported by the BMBF / DZHK.

Adiposity influences on myocardial deformation: a cardiovascular magnetic resonance feature tracking study in people with overweight to obesity without established cardiovascular disease.

The objective of this study was to assess whether dietary-induced weight loss improves myocardial deformation in people with overweight to obesity without established cardiovascular disease applying cardiovascular magnetic resonance (CMR) with feature tracking (FT) based strain analysis. Ninety people with overweight to obesity without established cardiovascular disease (age 44.6 ± 9.3 years, body mass index (BMI) 32.6 ± 4 kg/m2) underwent CMR. We retrospectively quantified FT based strain and LA size and function at baseline and after a 6-month hypocaloric diet, with either low-carbohydrate or low-fat intake. The study cohort was compared to thirty-four healthy normal-weight controls (age 40.8 ± 16.0 years, BMI 22.5 ± 1.4 kg/m2). At baseline, the study cohort with overweight to obesity without established cardiovascular disease displayed significantly increased global circumferential strain (GCS), global radial strain (GRS) and LA size (all p < 0.0001 versus controls) but normal global longitudinal strain (GLS) and normal LA ejection fraction (all p > 0.05 versus controls). Dietary-induced weight loss led to a significant reduction in GCS, GRS and LA size irrespective of macronutrient composition (all p < 0.01). In a population with overweight to obesity without established cardiovascular disease subclinical myocardial changes can be detected applying CMR. After dietary-induced weight loss improvement of myocardial deformation could be shown. A potential clinical impact needs further studies.

Comparison of manual and artificial intelligence based quantification of myocardial strain by feature tracking-a cardiovascular MR study in health and disease.

OBJECTIVES: The analysis of myocardial deformation using feature tracking in cardiovascular MR allows for the assessment of global and segmental strain values. The aim of this study was to compare strain values derived from artificial intelligence (AI)-based contours with manually derived strain values in healthy volunteers and patients with cardiac pathologies. MATERIALS AND METHODS: A cohort of 136 subjects (60 healthy volunteers and 76 patients; of those including 46 cases with left ventricular hypertrophy (LVH) of varying etiology and 30 cases with chronic myocardial infarction) was analyzed. Comparisons were based on quantitative strain analysis and on a geometric level by the Dice similarity coefficient (DSC) of the segmentations. Strain quantification was performed in 3 long-axis slices and short-axis (SAX) stack with epi- and endocardial contours in end-diastole. AI contours were checked for plausibility and potential errors in the tracking algorithm. RESULTS: AI-derived strain values overestimated radial strain (+ 1.8 ± 1.7% (mean difference ± standard deviation); p = 0.03) and underestimated circumferential (- 0.8 ± 0.8%; p = 0.02) and longitudinal strain (- 0.1 ± 0.8%; p = 0.54). Pairwise group comparisons revealed no significant differences for global strain. The DSC showed good agreement for healthy volunteers (85.3 ± 10.3% for SAX) and patients (80.8 ± 9.6% for SAX). In 27 cases (27/76; 35.5%), a tracking error was found, predominantly (24/27; 88.9%) in the LVH group and 22 of those (22/27; 81.5%) at the insertion of the papillary muscle in lateral segments. CONCLUSIONS: Strain analysis based on AI-segmented images shows good results in healthy volunteers and in most of the patient groups. Hypertrophied ventricles remain a challenge for contouring and feature tracking. CLINICAL RELEVANCE STATEMENT: AI-based segmentations can help to streamline and standardize strain analysis by feature tracking. KEY POINTS: • Assessment of strain in cardiovascular magnetic resonance by feature tracking can generate global and segmental strain values. • Commercially available artificial intelligence algorithms provide segmentation for strain analysis comparable to manual segmentation. • Hypertrophied ventricles are challenging in regards of strain analysis by feature tracking.

[Clinical application of large language models : Does ChatGPT replace medical report formulation? An experience report]. / Anwendung von "large language models" in der Klinik : Ersetzt ChatGPT die Arztbrieferstellung? Ein Erfahrungsbericht.

Artificial intelligence (AI)-based language models, such as ChatGPT offer an enormous potential for research and medical care but also for clinical workflow optimization by making medical documentation easier and more efficient in taking over standardized routine tasks. With their ability to guess a text's content using word statistics and thus outputting contextually relevant results in chat dialogues, large language models (LLM) can provide appropriate summaries of medical documentation for different target groups. For instance, text generation in easy to understand language could potentially contribute to an increase in patients' health literacy and, consequently, to increased adherence to treatment. Subsequent, the function of AI-based chatbot models to improve user experiences and enhance competence in the use of AI-based language models will be adressed. Current limitations and chances in creating epicrises are presented as an experience report. In the future, the implementation of local LLMs in medical management systems (hospital information systems, HIS and practice administration systems, PAS) and in conjunction with the electronic patient records (ePA) can fundamentally change clinical and outpatient care.

Multi-site comparison of parametric T1 and T2 mapping: healthy travelling volunteers in the Berlin research network for cardiovascular magnetic resonance (BER-CMR).

BACKGROUND: Parametric mapping sequences in cardiovascular magnetic resonance (CMR) allow for non-invasive myocardial tissue characterization. However quantitative myocardial mapping is still limited by the need for local reference values. Confounders, such as field strength, vendors and sequences, make intersite comparisons challenging. This exploratory study aims to assess whether multi-site studies that control confounding factors provide first insights whether parametric mapping values are within pre-defined tolerance ranges across scanners and sites. METHODS: A cohort of 20 healthy travelling volunteers was prospectively scanned at three sites with a 3 T scanner from the same vendor using the same scanning protocol and acquisition scheme. A Modified Look-Locker inversion recovery sequence (MOLLI) for T1 and a fast low-angle shot sequence (FLASH) for T2 were used. At one site a scan-rescan was performed to assess the intra-scanner reproducibility. All acquired T1- and T2-mappings were analyzed in a core laboratory using the same post-processing approach and software. RESULTS: After exclusion of one volunteer due to an accidentally diagnosed cardiac disease, T1- and T2-maps of 19 volunteers showed no significant differences between the 3 T sites (mean ± SD [95% confidence interval] for global T1 in ms: site I: 1207 ± 32 [1192-1222]; site II: 1207 ± 40 [1184-1225]; site III: 1219 ± 26 [1207-1232]; p = 0.067; for global T2 in ms: site I: 40 ± 2 [39-41]; site II: 40 ± 1 [39-41]; site III 39 ± 2 [39-41]; p = 0.543). CONCLUSION: Parametric mapping results displayed initial hints at a sufficient similarity between sites when confounders, such as field strength, vendor diversity, acquisition schemes and post-processing analysis are harmonized. This finding needs to be confirmed in a powered clinical trial. Trial registration ISRCTN14627679 (retrospectively registered).

Lazy Luna: Extendible software for multilevel reader comparison in cardiovascular magnetic resonance imaging.

BACKGROUND AND OBJECTIVES: Cardiovascular Magnetic Resonance (CMR) imaging is a growing field with increasing diagnostic utility in clinical routine. Quantitative diagnostic parameters are typically calculated based on contours or points provided by readers, e.g. natural intelligences (NI) such as clinicians or researchers, and artificial intelligences (AI). As clinical applications multiply, evaluating the precision and reproducibility of quantitative parameters becomes increasingly important. Although segmentation challenges for AIs and guidelines for clinicians provide quality assessments and regulation, the methods ought to be combined and streamlined for clinical applications. The goal of the developed software, Lazy Luna (LL), is to offer a flexible evaluation tool that is readily extendible to new sequences and scientific endeavours. METHODS: An interface was designed for LL, which allows for comparing annotated CMR images. Geometric objects ensure precise calculations of metric values and clinical results regardless of whether annotations originate from AIs or NIs. A graphical user interface (GUI) is provided to make the software available to non-programmers. The GUI allows for an interactive inspection of image datasets as well as implementing tracing procedures, which follow statistical reader differences in clinical results to their origins in individual image contours. The backend software builds on a set of meta-classes, which can be extended to new imaging sequences and clinical parameters. Following an agile development procedure with clinical feedback allows for a quick implementation of new classes, figures and tables for evaluation. RESULTS: Two application cases present LL's extendibility to clinical evaluation and AI development contexts. The first concerns T1 parametric mapping images segmented by two expert readers. Quantitative result differences are traced to reveal typical segmentation dissimilarities from which these differences originate. The meta-classes are extended to this new application scenario. The second applies to the open source Late Gadolinium Enhancement (LGE) quantification challenge for AI developers "Emidec", which illustrates LL's usability as open source software. CONCLUSION: The presented software Lazy Luna allows for an automated multilevel comparison of readers as well as identifying qualitative reasons for statistical reader differences. The open source software LL can be extended to new application cases in the future.

Multilevel comparison of deep learning models for function quantification in cardiovascular magnetic resonance: On the redundancy of architectural variations.

Background: Cardiac function quantification in cardiovascular magnetic resonance requires precise contouring of the heart chambers. This time-consuming task is increasingly being addressed by a plethora of ever more complex deep learning methods. However, only a small fraction of these have made their way from academia into clinical practice. In the quality assessment and control of medical artificial intelligence, the opaque reasoning and associated distinctive errors of neural networks meet an extraordinarily low tolerance for failure. Aim: The aim of this study is a multilevel analysis and comparison of the performance of three popular convolutional neural network (CNN) models for cardiac function quantification. Methods: U-Net, FCN, and MultiResUNet were trained for the segmentation of the left and right ventricles on short-axis cine images of 119 patients from clinical routine. The training pipeline and hyperparameters were kept constant to isolate the influence of network architecture. CNN performance was evaluated against expert segmentations for 29 test cases on contour level and in terms of quantitative clinical parameters. Multilevel analysis included breakdown of results by slice position, as well as visualization of segmentation deviations and linkage of volume differences to segmentation metrics via correlation plots for qualitative analysis. Results: All models showed strong correlation to the expert with respect to quantitative clinical parameters (rz ' = 0.978, 0.977, 0.978 for U-Net, FCN, MultiResUNet respectively). The MultiResUNet significantly underestimated ventricular volumes and left ventricular myocardial mass. Segmentation difficulties and failures clustered in basal and apical slices for all CNNs, with the largest volume differences in the basal slices (mean absolute error per slice: 4.2 ± 4.5 ml for basal, 0.9 ± 1.3 ml for midventricular, 0.9 ± 0.9 ml for apical slices). Results for the right ventricle had higher variance and more outliers compared to the left ventricle. Intraclass correlation for clinical parameters was excellent (≥0.91) among the CNNs. Conclusion: Modifications to CNN architecture were not critical to the quality of error for our dataset. Despite good overall agreement with the expert, errors accumulated in basal and apical slices for all models.

Different Impacts on the Heart After COVID-19 Infection and Vaccination: Insights From Cardiovascular Magnetic Resonance.

Introduction: Myocarditis-like findings after COVID-19 (coronavirus disease 2019) infection and vaccination were reported by applying cardiovascular magnetic resonance (CMR). These results are very heterogenous and dependent on several factors such as hospital admission or outpatient treatment, timing of CMR, and symptomatic load. This retrospective study aimed to identify differences in myocardial damage in patients with persistent symptoms both after COVID-19 infection and vaccine by applying CMR. Materials and Methods: This study entails a retrospective analysis of consecutive patients referred for CMR between August 2020 and November 2021 with persistent symptoms after COVID-19 infection or vaccination. Patients were compared to healthy controls (HC). All patients underwent a CMR examination in a 1.5-T scanner with a scan protocol including: cine imaging for biventricular function and strain assessment using feature tracking, T2 mapping for the quantification of edema, and T1 mapping for diffuse fibrosis and late gadolinium enhancement (LGE) for the detection and quantification of focal fibrosis. Patients were divided into a subacute COVID-19 (sCov) group with symptoms lasting < 12 weeks, post-COVID-19 (pCov) group with symptoms > 12 weeks, and patients after COVID-19 vaccination (CovVac). Results: A total of 162 patients were recruited of whom 141 were included for analysis. The median age in years (interquartile range (IQR)) of the entire cohort was 45 (37-56) which included 83 women and 58 men. Subgroups were as follows (total patients per subgroup, median age in years (IQR), main gender): 34 sCov, 43 (37-52), 19 women; 63 pCov, 52 (39-58), 43 women; 44 CovVac, 43 (32-56), 23 men; 44 HC (41 (28-52), 24 women). The biventricular function was preserved and revealed no differences between the groups. No active inflammation was detected by T2 mapping. Global T1 values were higher in pCov in comparison with HC (median (IQR) in ms: pCov 1002ms (981-1023) vs. HC 987ms (963-1009; p = 0.005) with other parings revealing no differences. In 49/141 (34.6%) of patients, focal fibrosis was detectable with the majority having a non-ischemic pattern (43/141; 30.4%; patients) with the subgroups after infection having more often a subepicardial pattern compared with CovVac (total (% of group): sCov: 7/34(21%); pCov 13/63(21%); CovVac 2/44(5%); p = 0.04). Conclusion: Patients after COVID-19 infection showed more focal fibrosis in comparison with patients after COVID-19 vaccination without alterations in the biventricular function.

Fast acquisition of left and right ventricular function parameters applying cardiovascular magnetic resonance in clinical routine - validation of a 2-shot compressed sensing cine sequence.

Objectives. To evaluate if cine sequences accelerated by compressed sensing (CS) are feasible in clinical routine and yield equivalent cardiac morphology in less time. Design. We evaluated 155 consecutive patients with various cardiac diseases scanned during our clinical routine. LV and RV short axis (SAX) cine images were acquired by conventional and prototype 2-shot CS sequences on a 1.5 T CMR. The 2-shot prototype captures the entire heart over a period of 3 beats making the acquisition potentially even faster. Both scans were performed with identical slice parameters and positions. We compared LV and RV morphology with Bland-Altmann plots and weighted the results in relation to pre-defined tolerance intervals. Subjective and objective image quality was evaluated using a 4-point score and adapted standardized criteria. Scan times were evaluated for each sequence. Results. In total, no acquisitions were lost due to non-diagnostic image quality in the subjective image score. Objective image quality analysis showed no statistically significant differences. The scan time of the CS cines was significantly shorter (p < .001) with mean scan times of 178 ± 36 s compared to 313 ± 65 s for the conventional cine. All cardiac function parameters showed excellent correlation (r 0.978-0.996). Both sequences were considered equivalent for the assessment of LV and RV morphology. Conclusions. The 2-shot CS SAX cines can be used in clinical routine to acquire cardiac morphology in less time compared to the conventional method, with no total loss of acquisitions due to nondiagnostic quality. TRIAL REGISTRATION: ISRCTN12344380. Registered 20 November 2020, retrospectively registered.

[Role of cardiovascular magnetic resonance in cardiovascular diagnostics]. / Stellenwert der kardiovaskulären Magnetresonanz in der kardiovaskulären Diagnostik.

Cardiovascular magnetic resonance (CMR) has become an accepted method for noninvasive imaging in cardiology. As part of a multimodality concept, this method can contribute valuable diagnostic aspects, often even as a first-choice method in a variety of diseases. Currently the availability is still limited, but the increasing time efficiency, technical stability and the growing competence will lead to more guideline-compliant use. The increase of CMR inclusion into guidelines of various societies is mainly based on the unique selling point of CMR, which is noninvasive myocardial tissue differentiation. In addition to efficient ischemia diagnosis, the ability to differentiate active from chronic inflammatory processes as well as the identification of reversible and irreversible damage are some aspects CMR can offer. New developments are sequences which allow for a parametric assessment of myocardial tissue based on T1- and T2-relaxation times. This is especially useful if the exact pathophysiology is unclear, as it is often the case in left ventricular hypertrophy for example. Next to the noninvasive myocardial tissue characterization CMR allows for quantitative hemodynamic assessment of the heart and the related pathologies. Flows as well as gradients can be quantified based on 2D-flow-sequences. New 4D-sequences are aiming to further characterize blood flow in the heart and the great vessels beyond flow volume and gradients. As with any diagnostic method a qualified application is crucial. In recent years, the technique itself has become much more stable and consensus recommendations of the Society for Cardiovascular Magnetic Resonance are available for the main indications, both for the MRI scan procedure and for the evaluation. Appropriate qualifications and certification opportunities are offered both nationally and internationally.

Introduction of Lazy Luna an automatic software-driven multilevel comparison of ventricular function quantification in cardiovascular magnetic resonance imaging.

Cardiovascular magnetic resonance imaging is the gold standard for cardiac function assessment. Quantification of clinical results (CR) requires precise segmentation. Clinicians statistically compare CRs to ensure reproducibility. Convolutional Neural Network developers compare their results via metrics. Aim: Introducing software capable of automatic multilevel comparison. A multilevel analysis covering segmentations and CRs builds on a generic software backend. Metrics and CRs are calculated with geometric accuracy. Segmentations and CRs are connected to track errors and their effects. An interactive GUI makes the software accessible to different users. The software's multilevel comparison was tested on a use case based on cardiac function assessment. The software shows good reader agreement in CRs and segmentation metrics (Dice > 90%). Decomposing differences by cardiac position revealed excellent agreement in midventricular slices: > 90% but poorer segmentations in apical (> 71%) and basal slices (> 74%). Further decomposition by contour type locates the largest millilitre differences in the basal right cavity (> 3 ml). Visual inspection shows these differences being caused by different basal slice choices. The software illuminated reader differences on several levels. Producing spreadsheets and figures concerning metric values and CR differences was automated. A multilevel reader comparison is feasible and extendable to other cardiac structures in the future.

Progressive myocardial injury in myotonic dystrophy type II and facioscapulohumeral muscular dystrophy 1: a cardiovascular magnetic resonance follow-up study.

AIM: Muscular dystrophy (MD) is a progressive disease with predominantly muscular symptoms. Myotonic dystrophy type II (MD2) and facioscapulohumeral muscular dystrophy type 1 (FSHD1) are gaining an increasing awareness, but data on cardiac involvement are conflicting. The aim of this study was to determine a progression of cardiac remodeling in both entities by applying cardiovascular magnetic resonance (CMR) and evaluate its potential relation to arrhythmias as well as to conduction abnormalities. METHODS AND RESULTS: 83 MD2 and FSHD1 patients were followed. The participation was 87% in MD2 and 80% in FSHD1. 1.5 T CMR was performed to assess functional parameters as well as myocardial tissue characterization applying T1 and T2 mapping, fat/water-separated imaging and late gadolinium enhancement. Focal fibrosis was detected in 23% of MD2) and 33% of FSHD1 subjects and fat infiltration in 32% of MD2 and 28% of FSHD1 subjects, respectively. The incidence of all focal findings was higher at follow-up. T2 decreased, whereas native T1 remained stable. Global extracellular volume fraction (ECV) decreased similarly to the fibrosis volume while the total cell volume remained unchanged. All patients with focal fibrosis showed a significant increase in left ventricular (LV) and right ventricular (RV) volumes. An increase of arrhythmic events was observed. All patients with ventricular arrhythmias had focal myocardial changes and an increased volume of both ventricles (LV end-diastolic volume (EDV) p = 0.003, RVEDV p = 0.031). Patients with supraventricular tachycardias had a significantly higher left atrial volume (p = 0.047). CONCLUSION: We observed a remarkably fast and progressive decline of cardiac morphology and function as well as a progression of rhythm disturbances, even in asymptomatic patients with a potential association between an increase in arrhythmias and progression of myocardial tissue damage, such as focal fibrosis and fat infiltration, exists. These results suggest that MD2 and FSHD1 patients should be carefully followed-up to identify early development of remodeling and potential risks for the development of further cardiac events even in the absence of symptoms. Trial registration ISRCTN, ID ISRCTN16491505. Registered 29 November 2017 - Retrospectively registered, http://www.isrctn.com/ISRCTN16491505.

Role of CMR Imaging in Diagnostics and Evaluation of Cardiac Involvement in Muscle Dystrophies.

PURPOSE OF REVIEW: This review aims to outline the utility of cardiac magnetic resonance (CMR) in patients with different types of muscular dystrophies for the assessment of myocardial involvement, risk stratification and in guiding therapeutic decisions. RECENT FINDINGS: In patients suffering from muscular dystrophies (MD), even mild initial dysfunction may lead to severe heart failure over a time course of years. CMR plays an increasing role in the diagnosis and clinical care of these patients, mostly due to its unique capability to precisely characterize subclinical and progressive changes in cardiac geometry, function in order to differentiate myocardial injury it allows the identification of inflammation, focal and diffuse fibrosis as well as fatty infiltration. CMR may provide additional information in addition to the physical examination, laboratory tests, ECG, and echocardiography. Further trials are needed to investigate the potential impact of CMR on the therapeutic decision-making as well as the assessment of long-term prognosis in different forms of muscular dystrophies. In addition to the basic cardiovascular evaluation, CMR can provide a robust, non-invasive technique for the evaluation of subclinical myocardial tissue injury like fat infiltration and focal and diffuse fibrosis. Furthermore, CMR has a unique capability to detect the progression of myocardial tissue damage in patients with a preserved systolic function.