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BackgroundMyotonic dystrophy type 1 (DM1) is a hereditary muscular dystrophy affecting ∼2.1–14.3/100,000 adults. Cardiac manifestations of DM1 include conduction disorders and rarely cardiomyopathies. DM1 increases the risk of obstetric complications, however, little is known about the relationship between pregnancy and cardiomyopathy in DM1 due to disease rarity.CaseA 23-year-old with DM1 developed cardiomyopathy during pregnancy. Despite initial medical stabilization, she subsequently developed multiple spontaneous coronary artery dissections postpartum, worsening cardiomyopathy and multiorgan failure. She died 5 months postpartum.ConclusionThough cardiomyopathy and arterial dissection are both known complications of pregnancy, this case suggests individuals with myotonic dystrophy type 1 may be at heightened risk for cardiac disease during the peripartum period. Physicians caring for women with suspected or proven DM1 should offer counseling and be alerted to the risk of cardiac complications with pregnancy and in the peripartum period. Pregnant and peripartum women with DM1 are likely to benefit from more frequent assessments of cardiac function including echocardiograms and early institution of heart failure management protocols when symptoms of cardiomyopathy present.

Objectives To evaluate deformable registration algorithms (DRA)-based quantification of cine steady-state free-precession (SSFP) for myocardial strain assessment in comparison with feature-tracking (FT) and speckle-tracking echocardiography (STE). Methods Data sets of 28 patients/10 volunteers, undergoing same-day 1.5T cardiac MRI and echocardiography were included. LV global longitudinal (GLS), circumferential (GCS) and radial (GRS) peak systolic strain were assessed on cine SSFP data using commercially available FT algorithms and prototype DRA-based algorithms. STE was applied as standard of reference for accuracy, precision and intra-/interobserver reproducibility testing. Results DRA showed narrower limits of agreement compared to STE for GLS (-4.0 [-0.9,-7.9]) and GCS (-5.1 [1.1,-11.2]) than FT (3.2 [11.2,-4.9]; 3.8 [13.9,-6.3], respectively). While both DRA and FT demonstrated significant differences to STE for GLS and GCS (all p<0.001), only DRA correlated significantly to STE for GLS (r=0.47; p=0.006). However, good correlation was demonstrated between MR techniques (GLS:r=0.74; GCS:r=0.80; GRS:r=0.45, all p<0.05). Comparing DRA with FT, intra-/interobserver coefficient of variance was lower (1.6 %/3.2 % vs. 6.4 %/5.7 %) and intraclass-correlation coefficient was higher. DRA GCS and GRS data presented zero variability for repeated observations. Conclusions DRA is an automated method that allows myocardial deformation assessment with superior reproducibility compared to FT. Key Points • Inverse deformable registration algorithms (DRA) allow myocardial strain analysis on cine MRI. • Inverse DRA demonstrated superior reproducibility compared to feature-tracking (FT) methods. • Cine MR DRA and FT analysis demonstrate differences to speckle-tracking echocardiography • DRA demonstrated better correlation with STE than FT for MR-derived global strain data.

ObjectivesTo validate deformable registration algorithms (DRAs) for cine balanced steady-state free precession (bSSFP) assessment of global longitudinal strain (GLS) and global circumferential strain (GCS) using harmonic phase (HARP) cardiovascular magnetic resonance as standard of reference (SoR).MethodsSeventeen patients and 17 volunteers underwent short axis stack and 2-/4-chamber cine bSSFP imaging with matching slice long-axis and mid-ventricular spatial modulation of magnetization (SPAMM) myocardial tagging. Inverse DRA was applied on bSSFP data for assessment of GLS and GCS while myocardial tagging was processed using HARP. Intra- and inter-observer variability assessment was based on repeated analysis by a single observer and analysis by a second observer, respectively. Standard semi-automated short axis stack segmentation was performed for analysis of left ventricular (LV) volumes and ejection fraction (EF).ResultsDRA demonstrated strong relationships to HARP for myocardial GLS (R2 = 0.75; p < 0.0001) and endocardial GLS (R2 = 0.61; p < 0.0001). GCS result comparison also demonstrated significant relationships between DRA and HARP for myocardial strain (R2 = 0.61; p < 0.0001) and endocardial strain (R2 = 0.51; p < 0.0001). Both methods demonstrated small systematic errors for intra- and inter-observer variability but DRA demonstrated consistently lower CV. Global LVEF was significantly lower (p = 0.0099) in patients (53.7%; IQR 43.9/64.0%) than in healthy volunteers (62.6%; IQR 61.1/66.2%). DRA and HARP strain data demonstrated significant relationships to LVEF.ConclusionsNon-rigid deformation method–based DRA provides a reliable measure of peak systolic GCS and GLS based on cine bSSFP with superior intra- and inter-observer reproducibility compared to HARP.Key Point• Myocardial strain can be reliably analyzed using inverse deformable registration algorithms (DRAs) on cine CMR.• Inverse DRA-derived strain shows higher reproducibility than tagged CMR.• DRA and tagged CMR-based myocardial strain demonstrate strong relationships to global left ventricular function.

Test-retest reproducibility is of utmost importance in follow-up of right ventricular (RV) volumes and function; optimal slice orientation though is not yet known. We compared test-retest reproducibility and intra-/inter-observer variability of right ventricular (RV) volumes and function assessed with short-axis and transverse cardiovascular magnetic resonance (CMR). Eighteen volunteers underwent cine CMR for RV assessment obtaining ventricular coverage in short-axis and transverse slice orientation. Additional 2D phase contrast flow imaging of the main pulmonary artery (MPA) was performed. After complete repositioning repeat acquisitions were performed. Data sets were contoured by two blinded observers. Statistical analysis included Student's t-test, Bland-Altman plots, intra-class correlation coefficient (ICC) and 2-way ANOVA, SEM and minimal detectable difference calculations. Heart rates (65.0 ± 7.4 vs. 67.6 ± 9.9 bpm; P = 0.1) and MPA flow (89.8 ± 16.6 vs. 87.2 ± 14.9 mL; P = 0.1) did not differ between imaging sessions. EDV and ESV demonstrated an inter-study bias of 0.4 %[−9.5 %,10.3 %] and 2.1 %[−12.3 %,16.4 %] for short-axis and 1.1 %[−7.3 %,9.4 %] and 0.8 %[−16.0 %,17.6 %] for transverse orientation, respectively. There was no significant interaction between imaging orientation and interstudy reproducibility (p = 0.395–0.824), intra-observer variability (p = 0.726–0.862) or inter-observer variability (p = 0.447–0.706) by 2-way ANOVA. Inter-observer agreement by ICC was greater for short axis versus transverse orientation for all parameters (0.769–0.986 vs. 0.625–0.983, respectively). Minimal detectable differences for short axis and transverse orientations were 10.1 mL/11.5 mL for EDV, 8.3 mL/8.4 mL for ESV and 4.1 % vs. 4.7 % for EF, respectively. Short-axis and transverse orientation both provide reliable and reproducible measures for follow-up of RV volumes and global function. Therefore, additional transverse SSFP cine CMR may not necessarily be required if performed for the sole purpose of quantitative volumetric RV assessment.