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. SUMMARY: 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

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

Inflammation and In-Stent Restenosis: The Role of Serum Markers and Stent Characteristics in Carotid Artery Stenting

BACKGROUND: Carotid angioplasty and stenting (CAS) may currently be recommended especially in younger patients with a high-grade carotid artery stenosis. However, evidence is accumulating that in-stent restenosis (ISR) could be an important factor endangering the long-term efficacy of CAS. The aim of this study was to investigate the influence of inflammatory serum markers and procedure-related factors on ISR as diagnosed with duplex sonography. METHODS: We analyzed 210 CAS procedures in 194 patients which were done at a single university hospital between May 2003 and June 2010. Periprocedural C-reactive protein (CRP) and leukocyte count as well as stent design and geometry, and other periprocedural factors were analyzed with respect to the occurrence of an ISR as diagnosed with serial carotid duplex ultrasound investigations during clinical long-term follow-up. RESULTS: Over a median of 33.4 months follow-up (IQR: 14.9-53.7) of 210 procedures (mean age of 67.9±9.7 years, 71.9% male, 71.0% symptomatic) an ISR of ≥70% was detected in 5.7% after a median of 8.6 months (IQR: 3.4-17.3). After multiple regression analysis, leukocyte count after CAS-intervention (odds ratio (OR): 1.31, 95% confidence interval (CI): 1.02-1.69; p = 0.036), as well as stent length and width were associated with the development of an ISR during follow-up (OR: 1.25, 95% CI: 1.05-1.65, p = 0.022 and OR: 0.28, 95% CI: 0.09-0.84, p = 0.010). CONCLUSIONS: The majority of ISR during long-term follow-up after CAS occur within the first year. ISR is associated with periinterventional inflammation markers and influenced by certain stent characteristics such as stent length and width. Our findings support the assumption that stent geometry leading to vessel injury as well as periprocedural inflammation during CAS plays a pivotal role in the development of carotid artery ISR

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

Global impact of COVID-19 on childhood tuberculosis: an analysis of notification data

Background There is concern that the COVID-19 pandemic has damaged global childhood tuberculosis management. Quantifying changes in childhood tuberculosis notifications could support more targeted interventions to restore childhood tuberculosis services. We aimed to use time-series modelling to evaluate the impact of COVID-19 on child tuberculosis notifications. Methods Annual tuberculosis case notification data reported to WHO by 215 countries were used to calculate annual notification counts for the years 2014–20, stratified by age groups (0–4, 5–14, and ≥15 years) and sex. We used time-series modelling to predict notification counts for 2020, and calculated differences between these predictions and observed notifications in 2020 for each of the six WHO regions and at the country level for 30 countries with high tuberculosis burden. We assessed associations between these differences and the COVID-19 stringency index, a measure of COVID-19 social impact. Findings From 2014 to 2019, annual tuberculosis notification counts increased across all age groups and WHO regions. More males than females in the 0–4 years age group and ≥15 years age group had notifications in all years from 2014 to 2020 and in all WHO regions. In the 5–14 years age group, more females than males were notified globally in all years, although some WHO regions had higher notifications from males than females. In 2020, global notifications were 35·4% lower than predicted (95% prediction interval –30·3 to –39·9; 142 525 observed vs 220 794 predicted notifications [95% prediction interval 204 509 to 237 078]) for children aged 0–4 years, 27·7% lower (–23·4 to –31·5; 256 398 vs 354 578 [334 724 to 374 431]) in children aged 5–14 years, and 18·8% lower (–15·4 to –21·9; 5 391 753 vs 6 639 547 [6 375 086 to 6 904 007]) for people aged 15 years or older. Among those aged 5–14 years, the reduction in observed relative to predicted notifications for 2020 was greater in males (–30·9% [–24·8 to –36·1]) than females (–24·5% [–18·1 to –29·9]). Among 28 countries with high tuberculosis burden, no association was observed between the stringency of COVID-19 restrictions and the relative difference in observed versus predicted notifications. Interpretation Our findings suggest that COVID-19 has substantially affected childhood tuberculosis services, with the youngest children most affected. Although children have mostly had fewer severe health consequences from COVID-19 than have adults, they have been disproportionately affected by the effects of the pandemic on tuberculosis care. Observed sex differences suggest that targeted interventions might be required. As countries rebuild health systems following the COVID-19 pandemic, it is crucial that childhood tuberculosis services are placed centrally within national strategic plans

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

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, 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