Sean M. Lang

Background Balanced steady-state free-precession (bSSFP) cine imaging is the clinical standard for ventricular function assessment but requires multiple breath-holds, which can be challenging for pediatric and young adult patients. Deep learning (DL)–accelerated cine imaging offers the potential to reduce breath-hold burden, shorten scan time, and enable free-breathing acquisitions. Objective To clinically evaluate a commercially available DL-accelerated cine bSSFP sequence (SonicDL) across multiple acceleration levels and breath-hold/free-breathing configurations, and to assess diagnostic image quality, ventricular volumetric accuracy, and scan time reductions. Materials and methods This retrospective study included 25 patients with pectus excavatum and 15 with cardiomyopathy who underwent conventional cine imaging with breath-hold duration of nine cardiac-cycle interval (9-RR) and SonicDL acquisitions at 4-RR breath-hold, 1-RR breath-hold, and 1-RR free breathing. Diagnostic image quality was independently scored by three expert readers using a 5-point scale. Automated DL-based segmentation provided biventricular volumetric indices, with phase-contrast flow serving as the physiological reference for stroke volume. Statistical analysis included repeated-measures ANOVA, paired tests, ICCs, and Bland–Altman analysis. Results SonicDL significantly reduced scan time 57% (4-RR breath-hold), 79% (1-RR breath-hold), and 87% (1-RR free breathing) compared with 9-RR breath-hold imaging. Diagnostic image quality was highest for 4-RR breath-hold (median 4.50), significantly exceeding other protocols (P2 for most parameters) with limits of agreement typically within ±6 mL/m². Stroke volumes showed close agreement with phase-contrast flow (biases 1-5 mL/m²) across all protocols. In cardiomyopathy patients, diagnostic image quality was lower overall, but volumetric indices agreement remained within clinically acceptable ranges. Conclusion SonicDL cine bSSFP imaging substantially reduces breath-hold burden and scan time while maintaining diagnostic image quality and close agreement with conventional cine and physiologic flow measurements. The 4-RR breath-hold protocol provided the most favorable balance of acceleration and fidelity, while free-breathing acquisitions offered a practical alternative for patients with limited breath-hold capacity. Graphical abstract The alternative text for this image may have been generated using AI.

Background: Duchenne muscular dystrophy (DMD) has unique characteristics when compared with other forms of non‐DMD dilated cardiomyopathy. We sought to describe epicardial adipose tissue (EAT), the fat layer surrounding the myocardium, and investigate its relationship to longitudinal cardiac changes in DMD. Methods: This single‐center, retrospective, cohort study included all DMD patients with ≥5 cardiac magnetic resonance studies between 2015 and 2022. EAT thickness was measured in the right atrioventricular groove during end diastole. Adiposity data were obtained from dual‐energy x‐ray absorptiometry scans. Linear mixed‐effects regression was used for statistical modeling. Results: A total of 117 patients were included with 590 cardiac magnetic resonance studies, 333 dual‐energy X‐ray absorptiometry, and 255 NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide) values. The median age at first cardiac magnetic resonance was 13.1 years and at any cardiac magnetic resonance study was 15.9 years. The median EAT thickness was 15.5±4 mm. EAT thickness was positively associated with age ( P P P P =0.98) or for left ventricular volume ( P =0.61) or mass ( P =0.52) when indexed for height. Conclusions: DMD is associated with early, appreciable EAT, which increases over the life span and is positively related to measures of general adiposity. Increased EAT is associated with higher levels of NT‐proBNP without a change in systolic function, suggesting it may contribute to diastolic dysfunction in DMD.

BACKGROUND: 2D-speckle tracking echocardiography may help detect subclinical ventricular dysfunction, but data in multisystem inflammatory syndrome in children (MIS-C) are scarce. We investigated left ventricular (LV) strain parameters in MIS-C and their association with outcomes. METHODS: We performed an ambi-directional, 32-center cohort study on hospitalized MIS-C patients (March 2020–November 2021) with at least 1 echocardiogram read by the Core Lab. Generalized estimating equation modeling was used to test associations between LV strain and a composite in-hospital adverse cardiovascular outcome (vasoactive support, arrhythmias, cardiac arrest, extracorporeal support, death, or heart transplant). RESULTS: Of 349 patients (median age, 8.7 years [interquartile range, 5.3–12.9]), 35% had decreased LV ejection fraction during hospitalization, and 45% had depressed LV strain (either 4-chamber LV longitudinal strain [4CH-LVLS] or mid-ventricular LV circumferential strain [mid-LVCS]). The worst 4CH-LVLS and mid-LVCS occurred at ≈5 days of illness; 50% of abnormal LV strain normalized within 1 week, and 95% within 50 days. In-hospital adverse outcomes occurred in 35% of patients; these patients were older ( P =0.003) and, at admission, had more likely abnormal troponin ( P P P P =0.015), worse LV ejection fraction ( P P =0.002; mid-LVCS, P =0.001). Covariate-adjusted individual models for each strain parameter showed that 4CH-LVLS (adjusted odds ratio, 1.09 [95% CI, 1.07–1.12]), mid-LVCS (adjusted odds ratio, 1.06 [95% CI, 1.04–1.09]), worst LV strain Z score between 4CH-LVLS and mid-LVCS (adjusted odds ratio, 1.30 [95% CI, 1.21–1.41]), and early diastolic longitudinal strain rate (EDLSR; adjusted odds ratio, 1.68 [95% CI, 1.26–2.23]) at admission were found to be associated with adverse outcomes. CONCLUSIONS: About half of MIS-C patients had abnormal LV strain during hospitalization. 4CH-LVLS, mid-LVCS, the most abnormal strain Z score, and early diastolic longitudinal strain rate at admission were independently associated with in-hospital adverse cardiovascular outcome. These data may help early characterization and prognostication in MIS-C.

BACKGROUND: Multisystem Inflammatory Syndrome in Children is characterized by high rates of acute cardiovascular involvement with rapid recovery of organ dysfunction. However, information regarding long-term sequelae is lacking. We sought to characterize the systolic function and myocardial tissue properties using cardiac magnetic resonance (CMR) imaging in a multicenter observational cohort of Multisystem Inflammatory Syndrome in Children patients. METHODS: In this observational cohort study, comprising 32 centers in North America, CMR studies were analyzed by a core laboratory to assess ventricular volumetric data, tissue characterization, and coronary involvement. RESULTS: A total of 263 CMRs from 255 Multisystem Inflammatory Syndrome in Children patients were analyzed. The mean patient age was 11.4±4.4 years. Most studies were performed at 3 months (33%) or 6 months (45%) after hospitalization. Left ventricular dysfunction was present in 17 (6.7%) of the first CMRs and was never worse than mild. Dysfunction was observed in 4/7 (57%) patients at admission, 5/87 (6.9%) patients at 3 months, and 6/129 (4.6%) patients imaged either at 6 months or 1 year post-hospitalization. Late gadolinium enhancement was present in 2 (0.8%) patients, 1 at 3 months and another at 6 months following hospitalization. Coronary artery dilation was present in 13 of the 174 (7.5%) patients. Nine patients met the Lake Louise criteria for myocarditis (3.5%) at the time of CMR. CONCLUSIONS: In this largest published multiinstitutional longitudinal CMR evaluation of confirmed Multisystem Inflammatory Syndrome in Children patients, the prevalence of ventricular dysfunction and myocardial tissue characterization abnormalities on medium-term follow-up was low. However, a small number of patients had mild residual abnormalities at 6 months and 1 year following hospitalization. REGISTRATION: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT05287412.

Cardiac dysfunction is a leading cause of morbidity and mortality in Duchenne muscular dystrophy (DMD). Left atrial (LA) function is a poorly understood concept in this patient population, and research suggests underlying structural changes that could affect atrial function. Cardiac magnetic resonance (CMR) imaging may provide an important non-invasive approach to evaluating LA function. This study was a single center retrospective review of consecutive CMR studies over a 1 year period comparing LA phasic function within a cohort of DMD patients, and to those with structurally and functionally normal hearts. LA strain measurements including global reservoir, conduit, boost-pump strain, and LA volumes were obtained retrospectively. Spearman correlation analyses were performed on atrial strain measurements. 107 DMD and 79 normal CMR studies were included. The DMD cohort had worse systolic function (p < 0.001), smaller indexed max LA and left ventricular (LV) volumes (p < 0.001), and greater LA emptying fraction (p < 0.001). In the DMD cohort, emptying fraction decreased with advanced patient age (p < 0.001) and diminishing systolic function (p < 0.001). DMD patients with moderate or severe LV dysfunction demonstrated lower LA emptying fraction (p = 0.002), more impaired 2-chamber LA reservoir (p = 0.003), and LA pump (p = 0.006) and conduit strain (p = 0.018). DMD patients with preserved function have lower indexed LA volumes with higher LA emptying fractions than controls. Progression of disease and age is associated with decreased LA emptying fraction with early manifestations in reservoir and conduit strain. These findings suggest that strain markers of LA compliance and early left ventricular relaxation are associated with worsening cardiomyopathy in the DMD population.

Background: Breath‐holding (BH) for cine balanced steady state free precession (bSSFP) imaging is challenging for patients with impaired BH capacity. Deep learning‐based reconstruction (DLR) of undersampled k‐space promises to shorten BHs while preserving image quality and accuracy of ventricular assessment.Purpose: To perform a systematic evaluation of DLR of cine bSSFP images from undersampled k‐space over a range of acceleration factors.Study Type: Retrospective.Subjects: Fifteen pectus excavatum patients (mean age 16.8 ± 5.4 years, 20% female) with normal cardiac anatomy and function and 12‐second BH capability.Field Strength/Sequence: 1.5‐T, cine bSSFP.Assessment: Retrospective DLR was conducted by applying compressed sensitivity encoding (C‐SENSE) acceleration to systematically undersample fully sampled k‐space cine bSSFP acquisition data over an acceleration/undersampling factor (R) considering a range of 2 to 8. Quality imperceptibility (QI) measures, including structural similarity index measure, were calculated using images reconstructed from fully sampled k‐space as a reference. Image quality, including contrast and edge definition, was evaluated for diagnostic adequacy by three readers with varying levels of experience in cardiac MRI (>4 years, >18 years, and 1 year). Automated DL‐based biventricular segmentation was performed commercially available software by cardiac radiologists with more than 4 years of experience.Statistical Tests: Tukey box plots, linear mixed effects model, analysis of variance (ANOVA), weighted kappa, Kruskal–Wallis test, and Wilcoxon signed‐rank test were employed as appropriate. A P‐value R increased. Diagnostically adequate image quality was observed up to R = 5. The effect of R on all biventricular volumetric indices was non‐significant (P = 0.447).Data Conclusion: The biventricular volumetric indices obtained from the reconstruction of fully sampled cine bSSFP acquisitions and DLR of the same k‐space data undersampled by C‐SENSE up to R = 5 may be comparable.Evidence Level: 3Technical Efficacy: Stage 1

Cardiac magnetic resonance (CMR) incorporates a field of view that has the potential to capture clinically relevant extracardiac findings (ECF); however, there has been minimal investigation of ECF prevalence in children’s hospitals, where the patient population varies in age and diagnosis. We retrospectively reviewed consecutive, clinically indicated, CMR studies performed at a tertiary care children’s hospital during a 1-year period from January 1 to December 31, 2019. ECFs were classified as significant or non-significant based on whether they were described in the final impression of the CMR report. A total of 851 distinct patients had a CMR study during the 1-year period. Mean age was 19.5 (range 0.2; 74.2) years. A total of 254 ECFs were present in 158 of the 851 studies (18.6%) with 9.8% of all studies having significant ECFs. A total of 40.2% of ECFs were previously unknown and 9.1% (23/254) of ECFs included further recommendations (2.1% of all studies). ECFs were most often found in the chest (48%) or abdomen/pelvis (46%). Three patients were incidentally found to have malignancy (renal cell, thyroid, and hepatocellular carcinoma). Comparing studies with significant ECFs to the group without, CMR indications for biventricular CHD (43% vs 31%, p = 0.036), single ventricle CHD (12% vs 3.9%, p = 0.002), and aortopathy/vasculopathy (16% vs 7.6%, p = 0.020) were more common. The odds of significant ECF increased with increasing age (OR 1.82, 95% CI 1.10–3.01) and increased most notably between ages 14 to 33 years old. Recognition of the high percentage of ECFs remains important for timely diagnosis of these incidental findings.