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This study aimed to investigate the acute changes in proximal aortic distensibility, a measure of aortic stiffness, induced by acute exercise in participants with and without heart failure (HF). Participants with HF (n = 24) and without HF (n = 26) underwent cardiovascular magnetic resonance (CMR) (1.5 T) imaging at rest and after submaximal supine bicycle ergometry. The participants were further categorized into HF with reduced ejection fraction (HFrEF) (n = 14) and HF with preserved ejection fraction (n = 10) based on the left ventricular ejection fraction. At rest and immediately after exercise, cine CMR images of the cross-sectional ascending and descending aorta at the pulmonary artery bifurcation level were obtained to determine aortic distensibility (AoD), with lower AoD indicating greater aortic stiffness. Differences in means of values at rest and before and after exercise were compared using the nonparametric Wilcoxon sign test. There was no significant difference in AoD at rest between subjects with HF and controls. However, immediately after exercise, participants with HF but not controls exhibited a significant reduction in AoD, indicating higher aortic stiffness related to exercise (median [interquartile range] for the ascending aorta: 3.16 (1.26) × 10 -3 mm Hg -1 to 2.39 (1.57) × 10 -3 mm Hg -1 and the descending aorta: 4.19 (2.58) × 10 -3 mm Hg -1 to 2.96 (1.79) × 10 -3 mm Hg -1 ) (both p = 0.023). This decrease was particularly observed in participants with HFrEF but not in those with HF with preserved ejection fraction. Exercise-induced aortic stiffness, detectable by noninvasive CMR, may contribute to unfavorable ventricular-vascular interactions during exercise in participants with HF, especially HFrEF., Competing Interests: Declaration of competing interest The authors have no competing interest to declare., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Transesophageal echocardiography (TEE) plays an important role for real-time procedural guidance during surgical smyectomy (SM) for hypertrophic obstructive cardiomyopathy (HOCM). We aimed to compare (1) interventricular septum (IVS) thickness using 2- (2D) and 3-dimensional (3D) intraoperative TEE and preoperative cardiac magnetic resonance (CMR) and (2) mitral valve (MV) leaflet length using 2D, 3D TEE, automatic quantification of mitral valve (AMVQ) and preoperative CMR. We prospectively studied 50 patients with HOCM (age 59 ± 12 years, 44% men) who underwent SM during 2018 to 2019. The maximal basal, mid, and distal anteroseptum (AS) and inferoseptum (IS) were measured by multiplanar 3D reconstruction on TEE and by short-axis imaging on preoperative CMR and classified as mild (≤18 mm), moderate (18 to 25 mm), or severe (≥25 mm) groups based on AS and IS thickness on CMR. MV leaflet lengths were evaluated by preoperative CMR and intraprocedural 2D TEE, zoom 3D TEE, and AMVQ (EchoPAC, General Electric, Wisconsin). There was a moderate correlation between AS and IS thickness on 3D TEE and CMR (R 2 = 0.46, p <0.01 and R 2 = 0.41, p <0.01, respectively), with 3D TEE showing an average overestimation of 3.8 and 4.7 mm versus CMR. The 3D TEE overestimated 14 patients (56%) with mild thickness as moderate and 5 patients (22%) with moderate thickness as severe. Assuming 3D TEE as the gold standard, the closest correlation for anterior mitral leaflet length was with CMR (average overestimation by CMR of 0.5 mm [root mean square deviation (RMSE%) 17]), intermediate correlation with 2D TEE (average deviation of 0.6 mm [RMSE% 21]) and no correlation with AMVQ (average deviation of 0.7 mm [RMSE% 24]). In conclusion, 3D TEE overestimates IVS thickness versus CMR in patients with HOCM who underwent SM, with greater discrepancy in those with thinner IVS. There are significant differences in MV lengths measured using different imaging techniques., Competing Interests: Declaration of competing interest Dr. Desai is a consultant and has research agreements with Bristol Myers Squibb, cytokinetics, Tenaya, Viz-AI, and Edgewise. Dr. Smedira is a consultant for Bristol Myers Squibb. No industry support was utilized in the conduct of this study. The authors have no competing interests to declare., (Copyright © 2024 Elsevier Inc. All rights reserved.)

In patients with cardiac amyloidosis, pericardial involvement is common, with up to half of patients presenting with pericardial effusions. The pathophysiological mechanisms of pericardial pathology in cardiac amyloidosis include chronic elevations in right-sided filling pressures, myocardial and pericardial inflammation due to cytotoxic effects of amyloid deposits, and renal involvement with subsequent uremia and hypoalbuminemia. The pericardial effusions are typically small; however, several cases of life-threatening cardiac tamponade with hemorrhagic effusions have been described as a presenting clinical scenario. Constrictive pericarditis can also occur due to amyloidosis and its identification presents a clinical challenge in patients with cardiac amyloidosis who concurrently manifest signs of restrictive cardiomyopathy. Multimodality imaging, including echocardiography, cardiac computed tomography, and cardiac magnetic resonance imaging, is useful in the evaluation and management of this patient population. The recognition of pericardial effusion is important in the risk stratification of patients with cardiac amyloidosis as its presence confers a poor prognosis. However, specific treatment aimed at the effusions themselves is seldom indicated. Cardiac tamponade and constrictive pericarditis may necessitate pericardiocentesis and pericardiectomy, respectively., Competing Interests: Declaration of competing interest Dr. Klein reports serving on the advisory boards for Sobi and Pfizer. The other authors have no competing interests to declare., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Sudden cardiac death (SCD) represents the most severe complication of hypertrophic cardiomyopathy (HCM). However, the relation between strain, strain rate (SR), and risk factors in SCD risk stratification remains elusive. The study aimed to assess the attenuation of strain and SR in HCM by feature tracking cardiac magnetic resonance. All strain and SRs were obtained automatically by feature tracking, with manual adjustment of endocardial and epicardial borders. Strain indicators included left ventricular global longitudinal, circumferential, global radial strain (GRS), peak diastolic-longitudinal, circumferential, and radial SR. Patients were categorized into high-risk and low-risk groups for SCD based on the 2020 American Heart Association/American College HCM risk-SCD model. The correlation between strain/SR and SCD risk factors was assessed through Spearman correlation analysis. Furthermore, a multivariate logistic regression analysis was conducted to explore the factors that influence SCD risk in HCM patients. A total of 105 HCM patients were analyzed in this study, including 38 patients in the high-risk group, and 67 patients in the low-risk group. Compared with the low-risk group, the high-risk group exhibited significantly worse strain and SR (p <0.001). Furthermore, both circumferential and GRS and SR exhibited meaningful associations with risk factors for SCD. Additionally, GRS emerged as an independent risk factor for predicting heightened SCD risk in HCM patients (p <0.001). In conclusion, left ventricular strain and SR based on feature tracking-cardiac magnetic resonance can be evaluated for SCD risk and are strongly associated with SCD risk factors., Competing Interests: Declaration of competing interest The authors have no competing interests to declare., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Competing Interests: Declaration of competing interest Dr. Gupta: CVRx: honorarium for educational presentations. Dr. Sheikh: Abbott: institutional research support and honorarium for educational presentations. Alnylam: consultant, institutional research support. Akcea: institutional research support. Dr. Weissman: institutional research grants through MedStar Health Research Institute Corelab from Abbott, Boston Scientific, Medtronic. The remaining authors have no conflicts of interest to declare.

Cardiac sarcoidosis (CS) is a relapsing-remitting disease, and immune suppression (IS) is the mainstay of therapy. Predictors of relapse for patients with CS in remission are not well characterized. We assessed incidence of relapse in consecutive patients with CS treated with high-dose steroids and/or steroid-sparing agents (SSA) in our center from 2000 to 2020. Remission was defined as reaching maintenance therapy (no IS, SSA, and/or prednisone ≤5 mg/d) for ≥1 month. Relapse was defined as recurrence of CS syndrome requiring IS intensification: heart failure, ventricular arrhythmia, decrease in left ventricular ejection fraction, or increased disease burden on imaging. Among 68 patients, the mean age was 50.7±9.0 years; 25 (37%) were women, and 32 (47%) were Black. In total, 59 patients (87%) reached remission. Over a median follow-up of 39.5 months (interquartile range 17.6, 92.5), 28 (48%) relapsed. Greater percentage of late gadolinium enhancement (LGE) on pretreatment magnetic resonance imaging corresponded with increased likelihood of relapse (odds ratio 1.396 per 5% increase [95% confidence interval (CI) 1.04 to 1.88]; p = 0.028). LGE ≥11% predicted elevated risk of relapse (adjusted odds ratio 4.998 [1.34 to 18.64]; p = 0.017). Shorter time to relapse was observed with isolated CS (adjusted hazard ratio 4.084 [1.44,11.56]; p = 0.008) and LGE ≥11% (adjusted hazard ratio 3.007 [1.01, 8.98]; p = 0.049). Approximately 1 in 2 patients with CS in remission experienced relapse. Greater burden of LGE on cardiac magnetic resonance imaging and isolated CS are associated with greater risk of relapse. Future work is needed to refine risk stratification for relapse and to optimize surveillance strategies on the basis of the burden of disease., Competing Interests: Declaration of Competing Interest Kelly Arps has received research support from Medtronic and honoraria from MedIQ. Jonathan P. Piccini is supported by R01AG074185 from the National Institutes of Aging; receives grants for clinical research from Abbott, American Heart Association, the Association for the Advancement of Medical Instrumentation, Bayer, Boston Scientific, iRhythm, and Philips; and serves as a consultant to Abbott, Abbvie, Ablacon, Altathera, ARCA Biopharma, Biotronik, Boston Scientific, Bristol Myers Squibb, LivaNova, Medtronic, Mile-stone, ElectroPhysiology Frontiers, Pfizer, Sanofi, Philips, and UpToDate. Sean D. Pokorney reports grants from Janssen Pharmaceuticals and the US Food and Drug Administration; grants and personal fees from Bristol-Myers Squibb, Pfizer, Boston Scientific, and Janssen Pharmaceuticals; and personal fees from Medtronic and Philips Albert Y. Suna reports research support from Medtronic and Merit Medical and advisory board/consulting support from Biosense Webster, Medtronic, and Merit Medical. The remaining authors have no competing interests to declare., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Septal midwall late gadolinium enhancement (LGE) is a characteristic finding on cardiac magnetic resonance imaging (CMR) in nonischemic dilated cardiomyopathy (DCM) and is associated with adverse events. Its significance in ischemic cardiomyopathy (ICM) is unknown. With this multicenter observational study, we aimed to study the characteristics of septal midwall LGE and evaluate its prognostic value in ICM. A total of 1,084 patients with an impaired left ventricular (LV) ejection fraction (<50%) on LGE-CMR, either because of ICM (53%) or DCM, were included retrospectively. Septal midwall LGE was defined as midmyocardial stripe-like or patchy LGE in septal segments and was present in 10% of patients with ICM compared with 34% of patients with DCM (p <0.001). It was significantly associated with larger LV volumes and lower LV ejection fraction, irrespective of etiology. The primary endpoint was all-cause mortality and secondary endpoint was ventricular arrhythmias (VAs), including resuscitated cardiac arrest, sustained VA, and appropriate implantable cardioverter-defibrillator (ICD) therapy. During a median follow-up of 2.7 years, we found a significant association between septal midwall LGE and mortality in patients with DCM (hazard ratio [HR] 1.92, p = 0.03), but not in patients with ICM (HR 1.35, p = 0.39). Risk of VAs was significantly higher in patients with septal midwall LGE on CMR, both in DCM (HR 2.80, p <0.01) and in ICM (HR 2.70, p <0.01). In conclusion, septal midwall LGE, typically seen in DCM, was also present in 10% of patients with ICM and was associated with increased LV dilation and worse function, irrespective of etiology. When present, septal midwall LGE was associated with adverse outcome., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to declare., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Mounting evidence shows that right ventricle (RV) function carries independent prognostic influence in various disease states. This study aimed to investigate the incidence and impact of permanent RV infarction in patients with inferior ST-segment elevation myocardial infarction (STEMI) and culprit lesion in the right coronary artery (RCA). In this substudy of the DANAMI-3 (DANish Study of Optimal Acute Treatment of Patients with ST-segment Elevation Myocardial Infarction) trial, cardiac magnetic resonance was performed in 291 patients at day 1 and follow-up 3 months after primary percutaneous coronary intervention of 674 patients with STEMI with the culprit lesion in the RCA. Final infarct was assessed using late gadolinium enhancement on cardiac magnetic resonance at 3 months. Patients with permanent RV infarction (20%) had lower ventricular function at follow-up; RV ejection fraction (EF) 47% ±6 versus 50% ± 5 (p <0.005) and left ventricular (LV) EF 56% ± 8 versus 60% ± 9 (p <0.006). Furthermore, patients with permanent RV infarction had a higher incidence of microvascular obstruction 39 (67%) versus 81 (39%) (p <0.001), larger final LV infarct size 16% ±8 versus 10% ± 8 (p <0.001) and larger LV area at risk 33% ± 10 versus 29% ± 9 (p <0.001). Permanent RV infarction was an independent predictor of final LV infarct size (p <0.001) but was not associated with LVEF (β = -0.0; p = 0.13) in multivariable analyses. In conclusion, permanent RV infarction was seen in 20% of patients with inferior STEMI and culprit lesion in RCA and independently predicted final LV infarct size. However, permanent RV infarction did not predict overall LV function. LGE was used to detect infarct location and quantify infarct size. 17 LGE in RV free wall on follow-up CMR was considered as permanent infarction. LGE images were obtained 10 minutes after intravenous injection of 0.1-mmol/kg body weight of gadolinium-based contrast (Gadovist; Bayer Schering, Berlin, Germany) using an electrocardiogram (ECG)-triggered inversion-recovery sequence. The inversion time was adjusted to null the signal from the normal myocardium. Short-axis images were acquired from the atrioventricular plane to the apex with adjacent 8-mm slices. The remaining protocol has been described previously. 16 ., (Copyright © 2022 The Author(s). Published by Elsevier Inc. All rights reserved.)

Late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) imaging can help detect myocardial damage. 123 I-betamethyl-p-iodophenyl-pentadecanoic acid single-photon emission computed tomography (BMIPP-SPECT) was developed to evaluate fatty acid metabolism and has been reported to help detect myocardial damage in cardiac sarcoidosis (CS). We analyzed data from CMR-LGE and performed BMIPP-SPECT in patients with CS taking prednisolone and investigated the association of BMIPP-SPECT with LGE as a prognostic factor in CS. Patients with CS who underwent BMIPP-SPECT and CMR-LGE at the time of diagnosis within 2 months were classified into those with and without a major adverse cardiac event (MACE). Total BMIPP-SPECT defect score (BDS) and LGE extent score (LES) were used to estimate myocardial damage. The relation between BDS and LES was explored using Pearson's correlation coefficient. Their ability to predict MACEs was analyzed using Kaplan-Meier analysis. Medical data of 45 patients were analyzed retrospectively (mean follow-up, 4.5 years). BDS and LES were significantly correlated (p <0.0001). BDS was significantly greater for the group with MACE than that without MACE (p = 0.0008). LES of patients with MACE was significantly greater than those without MACE (p = 0.0045). Patients with BDS ≥16 had a significantly higher MACE rate than those with BDS <16 (p = 0.0029). The group with LES ≥9 was significantly associated with MACE (p = 0.0098). In conclusion, BDS reflected myocardial damage similar to that detected by CMR-LGE and was a predictive marker of MACE in patients with CS. BMIPP-SPECT may help predict the prognosis of patients with CS who cannot undergo CMR-LGE., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Hypertrophic cardiomyopathy (HC) is the most common cardiovascular inherited disease, and it is associated with arrhythmic events, heart failure, and death. Strain analysis by tissue tracking (TT) techniques on cardiac magnetic resonance (CMR) is a novel noninvasive diagnostic tool. However, the usefulness of CMR-TT to identify patients with HC at risk of adverse outcomes remains unknown. CMR strain parameters by CMR-TT were prospectively measured in a cohort of 136 consecutive patients with HC. Clinical (death or readmission for heart failure) and arrhythmic (any ventricular tachycardia) events during follow-up were prospectively recorded. Global radial systolic strain rate and global radial diastolic strain rate showed the best area under the receiver operating characteristic curve (ROC curve) to predict adverse clinical events. On Cox multivariate regression models, a global radial systolic strain rate value <1.4/s and a global radial diastolic strain rate value ≥ -1.38/s were independently associated with clinical events at follow-up (adjusted hazard ratio 6.57, 95% confidence interval [CI] 2.01 to 21.49, p = 0.002; adjusted hazard ratio 5.96, 95% CI 1.79 to 19.89, p = 0.004, respectively). Regarding arrhythmic events, global radial peak strain <27% showed the best area under the ROC curve and remained independently associated with ventricular tachycardia after adjustment for confounders (odds ratio 7.33, 95% CI 1.07 to 50.41, p = 0.043). CMR strain parameters by TT predict clinical and arrhythmic events in patients with HC., Competing Interests: Disclosures The authors have no conflicts of interest to declare., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Quantification of myocardial perfusion reserve (MPR) using vasodilator stress cardiac magnetic resonance is increasingly used to detect coronary artery disease. However, MPR can also be altered because of changes in microvascular function. We aimed to determine whether MPR can distinguish between ischemic cardiomyopathy (IC) secondary to coronary artery disease and non-IC (NIC) with microvascular dysfunction and no underlying epicardial coronary disease. A total of 60 patients (mean age 65 ± 14 years, 30% women), including 31 with IC and 29 with NIC, were identified from a pre-existing vasodilator stress cardiac magnetic resonance registry. Short-axis cine slices were used to measure left ventricular ejection fraction (LVEF) using the Simpson method of disks. MPR index (MPRi) was determined from first-pass myocardial perfusion images during stress and rest using the upslope ratio, normalized for the arterial input and corrected for rate pressure product. Patients in both groups were divided into subgroups of LVEF ≤35% and LVEF >35%. Differences in MPRi between the subgroups were examined. MPRi was moderately correlated with LVEF in patients with NIC (r = 0.53, p = 0.03), whereas the correlation in patients with IC was lower (r = 0.32, p = 0.22). Average LVEF in NIC and IC was 34% ± 8% and 35% ± 8%, respectively (p = 0.63). MPRi was not significantly different in IC compared with NIC (1.17 [0.88 to 1.61] vs 1.23 [1.07 to 1.66], p = 0.41), including the subgroups of LVEF (IC: 1.20 ± 0.56 vs NIC: 1.15 ± 0.24, p = 0.75 for LVEF ≤35% and IC: 1.35 ± 0.44 vs NIC: 1.58 ± 0.50, p = 0.19 for LVEF >35%). However, MPRi was significantly lower in patients with LVEF ≤35% compared with those with LVEF>35% (1.17 ± 0.40 vs 1.47 ± 0.47, p = 0.01). Similar difference between LVEF groups was noted in the patients with NIC (1.15 ± 0.24 vs 1.58 ± 0.50, p = 0.006) but not in the patients with IC (1.20 ± 0.56 vs 1.35 ± 0.44, p = 0.42). MPRi can be abnormal in the presence of left ventricular dysfunction with nonischemic etiology. This is a potential pitfall to consider when using this approach to detect ischemia because of epicardial coronary disease using myocardial perfusion imaging., Competing Interests: Disclosures Dr. A. Patel has received research support from Philips, Arterys, CircleCVI, and Neosoft. The remaining authors have no conflicts of interest to declare., (Copyright © 2022. Published by Elsevier Inc.)

We compared speckle tracking echocardiography (STE) and feature tracking cardiovascular magnetic resonance (FT-CMR) in patients with hypertrophic cardiomyopathy (HC) with a varying extent of fibrosis as defined by late gadolinium enhancement to look at the level of agreement between methods and their ability to relate those to myocardial fibrosis. At 2 reference centers, 79 patients with HC and 16 volunteers (the control group) underwent STE and CMR with late gadolinium enhancement and FT-CMR. Patients were classified into 3 categories: no detectable, limited, and extensive fibrosis. Global longitudinal strain (GLS) and global radial strain (GRS) were derived using FT-CMR and STE. STE-derived GRS was decreased in all HC categories compared with the control group (p <0.001), whereas FT-CMR GRS was reduced only in patients with HC with fibrosis (p <0.05). Reduced STE-derived GLS was associated with extensive fibrosis (p <0.05) and a value less than -15.2% identified those with extensive fibrosis (sensitivity 79%, specificity 92%, area under the curve 0.863, 95% confidence interval [CI] 0.76 to 0.97, p <0.001). Inter-modality agreement was moderate for STE versus CMR-GLS (overall population intra-class correlation coefficient = 0.615, 95% CI 0.42 to 0.75, p <0.001; patients with HC 0.63, 0.42 to 0.76, p <0.001) and GRS (overall population intra-class correlation coefficient = 0.601, 95% CI 0.397 to 0.735, p <0.001). A low level of agreement for GRS was seen between methods in patients with HC. In conclusion, strain indexes measured using echocardiography and CMR are reduced in patients with HC compared with the control group and correlate well with the burden of myocardial fibrosis. Reduced STE-GLS can identify patients with extensive fibrosis, but whether there is an added value for risk stratification for sudden cardiac death remains to be determined., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Our aim was to assess changes of right ventricular end-diastolic volumes (RVEDVi) and right ventricular ejection fraction (RVEF) in asymptomatic adults with repaired tetralogy of Fallot, with native right ventricular outflow tract and severe pulmonary regurgitation by serial cardiac magnetic resonance imaging (CMR). The study included 23 asymptomatic adults who underwent ≥3 CMR studies (total of 88 CMR studies). We compared changes in RVEDVi and RVEF between first and last study (median follow-up: 8.8 years, interquartile range: 6.3 to 13.1 years) and between all study pairs. Variability of measurements between study pairs (65 consecutive and 139 nonconsecutive CMR study pairs) were assessed using Bland-Altman analysis and intraclass correlation coefficients. On average, there were no significant changes of RVEDVi or RVEF over the study period (change in RVEDVi: +0.4 ± 17.8 ml/m 2 , change in RVEF: -1.0 ± 5.5%). Assessment of variability of measurements between study pairs demonstrated no systematic change in RVEDVi and RVEF between study pairs with limits of agreement within the range of previously published studies (RVEDVi -29.1 to +27.2 ml/m 2 ; RVEF -11.5% to 10.2%). High intraclass correlation coefficients for RVEDVi (0.943, 95% CI 0.906 to 0.965, p <0.001) and RVEF (0.815, 95% CI 0.697 to 0.887, p <0.0001) indicate high reliability of reported measurements. In conclusion, in asymptomatic adults with repaired tetralogy of Fallot with native right ventricular outflow tracts and severe pulmonary regurgitation, CMR measurements of RV volumes and RVEF remain stable during follow-up with variability between CMR studies in individual patients, as expected for interobserver and interstudy variability. Measurements derived from a single CMR study or changes occurring between 2 CMR studies should be used with caution for clinical decision-making., Competing Interests: Disclosures The authors have no conflicting interests to declare., (Copyright © 2021 The Author(s). Published by Elsevier Inc. All rights reserved.)

This study aims to characterize flow, volume, and strain of the left atrium in hypertrophic cardiomyopathy (HC) with atrial fibrillation (AF) using cine cardiovascular magnetic resonance (CMR) imaging. Cine CMR data for 144 patients with HC, including 29 patients with episodes of paroxysmal AF and 13 patients with persistent AF, were retrospectively analyzed. The vortex flow of the left atrial (LA, %) was measured using a vortex flow map and was used as an estimate of flow. The LA end-systolic volume index (ml/m 2 ), LA ejection fraction (%) and global peak longitudinal LA strain (%) derived from a feature-tracking method were used as estimates of volume and strain. Vortex flow of the LA in patients with paroxysmal AF was significantly smaller than in patients without AF (vertical long-axis view; 26.7 ± 10.8% vs 33.2 ± 12.2%, p <0.005). The patients with paroxysmal AF had greater LA end-systolic volume index and global peak longitudinal LA strain and lower LA ejection fraction compared with those without AF. In conclusion, patients with HC with paroxysmal AF are characterized by small vortex flow, large volume, and decreased strain of LA on cine CMR., Competing Interests: Disclosures The authors have no conflicts of interest to declare., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Early repolarization pattern (ERP) is associated with increased mortality in case-control studies, but the mechanism and role of left ventricular mass (LVM) remain unclear. Our objectives were to understand (1) whether ERP associates with adverse outcomes in a multiethnic population and (2) to explore the role of LVM in these associations. Participants from the Dallas Heart Study with an electrocardiogram interpretable for ERP, defined as J point elevation ≥1 mm in 2 contiguous leads, were included. Combined all-cause mortality and nonfatal cardiovascular disease (CVD) events and individual components were assessed using Cox proportional hazards modeling after adjustment for demographics, traditional CVD risk factors, electrocardiogram intervals, and cardiac magnetic resonance imaging-derived factors. Cardiac magnetic resonance imaging-defined LVM was then added to the most fully adjusted model. Of the 2,686 participants, 240 (8.9%) demonstrated ERP. Participants with ERP were more likely to be male and Black, with lower body mass index, greater left ventricular end-diastolic volumes, and LVM. Over a median follow-up of 11 years, the combined end point occurred in 326 patients. Multivariable modeling demonstrated ERP was associated with the combined end point (HR [95% CI] 1.61 [1.14 to 2.26]), all-cause mortality (1.67 [1.00 to 2.80]). However, further adjusting for LVM attenuated the associations of ERP with the primary end point (HR [95% CI] 1.22 [0.85 to 1.77]) and secondary end points of mortality (1.39 [0.80 to 2.41]) and nonfatal CVD (1.05 [0.68 to 1.64]). ERP was associated with increased mortality and nonfatal CVD events, which was attenuated after adjusting for LVM, a previously under-recognized clinical phenotype. Previous associations of ERP with adverse cardiovascular outcomes may be partially explained by greater LVM in those with ERP., Competing Interests: Disclosures The authors have no conflicts of interest to disclose., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Mitral valve prolapse (MVP) is characterized by excessive leaflet tissue leading to a wide spectrum of mitral regurgitation (MR) ranging from trivial to severe. The prolapse volume (PV) below the prolapsing leaflets in end-systole was suspected to impact both chamber remodeling and MR grading in MVP. Based on 157 consecutive patients (45 women; mean age 62±15) referred for CMR assessment of MR, either from MVP (n = 91; 58%) or fibroelastic disease (FED) (n = 66; 42%), we sought to study (i) the interaction between PV and cardiac chamber geometry (ii) to study the impact of PV on MR quantification in MVP. Despite similar left ventricular (LV) size, PV was larger in MVP (11±9ml) than in FED (2±2ml). PV progressively increased with the severity of MR in MVP but not in FED. Despite a low regurgitant volume (32±18ml), some MVP patients with less than moderate MR exhibit significant cardiac chambers remodeling compared to 52 age and sex-matched controls. PV correlated significantly (r = 0.52) with the LV dilatation in severe MR but also in less than moderate MR. In MVP, PV>14ml was associated with a significant underestimation (Bias=-26±32ml) of regurgitant volume by PISA compared to CMR. In conclusion, in MVP, PV may play a role in left cardiac chambers remodeling, even in patients without severe MR, and in discordant grading of MR between echocardiography and CMR., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Women with Turner syndrome (TS) have high prevalence of cardiovascular anomalies. Literature suggests pregnancy is associated with a higher dissection risk, presumably preceded by aortic dilatation. Whether the aortic diameter truly changes during pregnancy in TS is not well investigated. This study aims to evaluate ascending aortic diameter change during pregnancy and reports on cardiac events during and directly after pregnancy. This tertiary hospital retrospective study investigated all TS women pregnancies (2009 to 2018). Outcome parameters included aortic diameter growth and aortic complications, specifically dissection. Thirty-five pregnancies in 30 TS women, 57% assisted by oocyte donation. Mean age at delivery 32 ± 5 years. In 27 pregnancies of 22 women imaging was available. From over 350 childless TS women a comparison group of 27 was individually matched. The median ascending aortic diameter growth between pre- and postpregnancy imaging was 1.0 mm (IQR -1.0; 2.0), no significant change (p = 0.077). Whether the patient had a bicuspid aortic valve (p = 0.571), monosomy X or mosaic karyotype (p = 0.071) or spontaneous pregnancy or resulting from oocyte donation (p = 0.686) had no significant influence on diameter change. Aortic growth between pregnancy and matched childless group (0.23 vs 0.32 mm/year, p = 0.788) was not significant over 3.3 ± 2 versus 4.4 ± 1 years. During pregnancy or the first 6 months after delivery no aortic complications were observed. In conclusion, this study suggests pregnancy in TS women does not induce faster ascending aortic diameter increase. Also not in presence of a bicuspid aortic valve, monosomy X karyotype, and oocyte donation. No aortic complications occurred. Based on current study pregnancy in TS women seems safe., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Fabry cardiomyopathy can cause symptomatic left ventricular outflow tract obstruction. We review a case of Fabry cardiomyopathy mimicking hypertrophic cardiomyopathy on echocardiography with severe left ventricular outflow tract obstruction treated with ventricular septal myectomy., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Hypertrophic cardiomyopathy (HC) is the most common inherited cardiomyopathy, with varied timing of phenotypic and clinical presentation. Literature describing cardiac magnetic resonance (CMR) imaging and late gadolinium enhancement (LGE) in young patients with HC is limited. This study included patients diagnosed with HC at young age (<21 years) between January 1990 and January 2015 who underwent transthoracic echocardiography and CMR with assessment of LGE at a single tertiary referral center. LGE was quantified via a method of 6 standard deviations and patients were grouped based upon presence or absence of LGE (≤1% and >1% LGE, respectively). Sudden cardiac death (SCD) risk was assessed in patients >16 years of age using the European SCD risk score. A composite outcome of New York Heart Association class III-IV symptoms, aborted SCD, heart transplantation, and all-cause mortality was assessed via Kaplan-Meier curves with log-rank analysis. Overall, 126 patients were included (78 male; 62%). Median age of diagnosis was 15 (12 to 18) years. LGE was present in 81 (64%) patients, although only 4 (3%) patients had LGE >15%. Median age at CMR imaging was 19 (15 to 23) years. Patients with LGE had greater wall thickness (25 ± 8 mm vs 22 ± 7 mm, p = 0.01). Median European SCD risk score was 4.7 (2.9 to 6.5). Median follow-up was 6.5 (2.5 to 13) years with 26 patients (21%) meeting the composite outcome. There were no significant differences in composite outcome since age of diagnosis when stratified by presence/absence of LGE (p = 1.0). The presence of LGE in young HC patients was not an independent risk factor for cardiovascular morbidity and mortality. Wall thickness was greater in patients with LGE. There remains a need for further evaluation of this unique HC cohort., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Cardiac magnetic resonance (CMR) offers the capability to objectively detect pericarditis by identifying pericardial thickening, edema/inflammation by Short-TI Inversion Recovery-T2 weighted (STIR-T2w) imaging, edema/inflammation or fibrosis by late gadolinium enhancement (LGE), and presence of pericardial effusion. This is especially helpful for the diagnosis of recurrent pericarditis. Aim of the present paper is to assess the diagnostic accuracy of CMR findings as well as their potential prognostic value for the diagnosis of recurrent pericarditis. Multicenter cohort study of consecutive patients with recurrent pericarditis evaluated by CMR. We included 128 consecutive cases (60 males, 47%; mean age 48 ± 14 years). CMR was performed at a mean time of 12 days (95% confidence interval 15 to 21) after the clinical diagnosis. We evaluated the diagnostic accuracy and areas under the receiver operating characteristic (ROC) curve for CMR diagnostic criteria and complications (additional recurrences, cardiac tamponade, and constrictive pericarditis). Areas under the ROC curve were respectively 64% for pericardial thickening, 84% for pericardial edema, 82% for pericardial LGE, and 71% for pericardial effusion. After a mean follow-up of 34 months, recurrences occurred in 52% of patients, tamponade in 6%, and constrictive pericarditis in 11%. Using a multivariable Cox model, elevation of CRP and presence of CMR pericardial thickening were predictors of adverse events, whereas the presence of CMR LGE was associated with a lower risk. The prognostic model for adverse events using gender, age, CRP level, and all CMR variables showed a C-index of 0.84. In conclusion, CMR findings show high diagnostic accuracy and may help identifying patients at higher risk of complications., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Atrial fibrillation (AF) is prevalent and strongly associated with higher cardiovascular disease (CVD) risk. Machine learning is increasingly used to identify novel predictors of CVD risk, but prediction improvements beyond established risk scores are uncertain. We evaluated improvements in predicting 5-year AF risk when adding novel candidate variables identified by machine learning to the CHARGE-AF Enriched score, which includes age, race/ethnicity, height, weight, systolic and diastolic blood pressure, current smoking, use of antihypertensive medication, diabetes, and NT-proBNP. We included 3,534 participants (mean age, 61.3 years; 52.0% female) with complete data from the prospective Multi-Ethnic Study of Atherosclerosis. Incident AF was defined based on study electrocardiograms and hospital discharge diagnosis ICD-9 codes, supplemented by Medicare claims. Prediction performance was evaluated using Cox regression and a parsimonious model was selected using LASSO. Within 5 years of baseline, 124 participants had incident AF. Compared with the CHARGE-AF Enriched model (c-statistic, 0.804), variables identified by machine learning, including biomarkers, cardiac magnetic resonance imaging variables, electrocardiogram variables, and subclinical CVD variables, did not significantly improve prediction. A 23-item score derived by machine learning achieved a c-statistic of 0.806, whereas a parsimonious model including the clinical risk factors age, weight, current smoking, NT-proBNP, coronary artery calcium score, and cardiac troponin-T achieved a c-statistic of 0.802. This analysis confirms that the CHARGE-AF Enriched model and a parsimonious 6-item model performed similarly to a more extensive model derived by machine learning. In conclusion, these simple models remain the gold standard for risk prediction of AF, although addition of the coronary artery calcium score should be considered., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Fulminant myocarditis (FM) is a rare, distinct form of myocarditis that has been difficult to classify. Since 1991, the definition of FM has evolved, and it is currently considered an acute illness with hemodynamic derangement and arrhythmias due to a severe inflammatory process requiring support of cardiac pump function and/or urgent management of serious arrhythmias. Diagnosis is aided through use of biomarkers and cardiac imaging, but endocardial biopsy remains the gold standard. Recent evidence has revealed that patients with FM are significantly more likely to die or require heart transplantation than those with the nonfulminant form, refuting previous studies proposing a paradoxically low mortality in patients with FM. Acute hemodynamic derangement is managed by intensive contemporary pharmacologic and interventional approaches, whereas the role of immunosuppressive therapy has not been clarified. Early recognition and aggressive management are essential for favorable outcomes. In conclusion, FM is an inflammatory process requiring intensive support, and it causes a higher morbidity and mortality than acute nonfulminant myocarditis., (Copyright © 2019. Published by Elsevier Inc.)

Mitral annulus disjunction (MAD) is characterized by a separation between the atrial wall mitral junction and the left ventricular (LV) free wall. Little is known regarding cardiac magnetic resonance (CMR) performance to detect MAD and its prevalence in mitral valve prolapse (MVP). Based on 89 MVP patients (63 women; mean age 64 ± 13) referred for CMR assessment of MR, either from myxomatous mitral valve disease (MMVP) (n = 40; 45%) or fibroelastic disease (n = 49; 55%), we sought to assess the frequency of MAD and its consequences on LV morphology. Patients were classified in 2 groups according to MAD presence (MAD+) or absence (MAD-). MAD (measuring 8 ± 4 mm) was diagnosed in 35% (31 of 89) of MVP patients, more frequently in MMVP than fibroelastic disease (60% vs 14%). MAD+ was associated with MMVP; bileaflet MVP and nonsustain ventricular tachycardia but not with the severity of MR. Diagnostic accuracy of transthoracic echocardiography for the detection of MAD was fair (65% sensitivity, 96% specificity) with CMR as reference. MAD+ showed significantly enlarged basal and mid LV diameters and enlarged mitral-annulus diameter. In patients with late gadolinium enhancement, presence of LV fibrosis at level of papillary muscle was more frequent in MAD+. After adjustment on age and MR severity, MMVP, and enlarged end-systolic mitral annulus diameter were independently associated with MAD+. In conclusion, MAD was present in about 1/3 of MVP patients, mostly in MMVP and independent of MR severity. Enlarged mitral-annulus and basal LV diameters, nonsustain ventricular tachycardia and papillary muscle fibrosis were associated with MAD presence., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Aortic regurgitation (AR) continues to be an important cause of morbidity and mortality in pediatric patients. Although echocardiographic parameters are well established for the adults, there are no clear cut-off values for AR severity in children. Cardiac magnetic resonance (CMR) imaging is considered a "gold standard" for a quantitative evaluation of the AR, but it is not widely available. This study assesses which echo parameter can accurately define AR severity as assessed by CMR in pediatric patients. A total of 27 pediatric patients (12 ± 3 years, range 6 to 18 years) with different degree of AR underwent echo assessment within an average of 35 days from CMR. CMR included phase-contrast velocity-encoded imaging for the measurement of regurgitant fraction (RF). Severe AR was defined as RF >33%. Echo evaluation included vena contracta, pressure half time, the ratio between the AR jet and the left ventricular outflow tract diameter (jet/left ventricular outflow tract), presence of holodiastolic reversal flow in abdominal aorta, the ratio between the velocity-time integral of the reversal flow over the forward flow in descending aorta (echoRF). Among the studied parameters, the strongest predictor of severe AR, as assessed by CMR, was echoRF. Receiver-operating characteristic curve showed, for a cutoff >0.38, an area under the curve of 0.886 (p <0.0001), a sensitivity of 71%, and a specificity of 100%. Correlation coefficient between echoRF and RF was R = 0.929 (p <0.0001). In conclusion, echoRF is a strong echo-Doppler marker of severe AR in the pediatric population. This parameter should be routinely added in the standard echo evaluation of pediatric patients with AR., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Atrial flutter (AFL) is a common form of arrhythmia recurrence after atrial fibrillation (AF) ablation. We aimed to define (1) the incidence of AFL and (2) the clinical factors associated with cavo-tricuspid isthmus dependent (typical) and atypical AFL, after AF ablation. The retrospective cohort consisted of 1,029 patients that underwent initial radiofrequency AF ablation from May 2005 to December 2013 at a single academic center. Patients with missing follow-up data, history of AFL ablation, and those with undocumented AFL were excluded. Atrial volumes were measured using three-dimensional cardiac computed tomography or magnetic resonance imaging. A total of 607 patients were included in the final cohort (age 59.2 ± 10.6 years, 76.0% men, 58.7% paroxysmal AF). During a median follow-up of 845 days (interquartile range 389 to 1,597 days), 122 (20.1%) patients developed AFL. Of these, 17 had typical AFL, 98 had atypical AFL, and 7 patients had both circuits. In the multivariable Cox regression analysis, only right atrial volume index (hazard ratio [HR] 1.25 per 10 ml/m 2 , confidence interval [CI] 95% 1.10 to 1.42) was associated with incident typical AFL; whereas persistent AF (HR 1.59, CI 95% 1.06 to 2.40), linear lesions (HR 1.58, CI 95% 1.02 to 2.46) and left atrial volume index (HR 1.17 per 10 ml/m 2 , CI 95% 1.07 to 1.27) were associated with incident atypical AFL. In conclusion, noninvasive measures of right and left atrial remodeling are strongly associated with incident AFL after AF ablation. Strategies to prevent incident AFL using these measures after index ablation warrant further investigation., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Heart failure with preserved ejection fraction (HFpEF) is frequently accompanied by co-morbidities and a systemic proinflammatory state, resulting in coronary microvascular dysfunction (CMD), as well as myocardial fibrosis. The purpose of this study is to examine the relation between myocardial perfusion reserve (MPR) and diffuse myocardial fibrosis in patients with HFpEF using cardiovascular magnetic resonance. A single center study was performed in 19 patients with clinical HFpEF and 15 healthy control subjects who underwent quantitative first-pass perfusion imaging to calculate global MPR. T1 mapping was used to assess fibrosis and to calculate extracellular volume. Spiral cine displacement encoded stimulated echo was used to calculate myocardial strain. Comprehensive 2D echocardiograms with speckle tracking, cardiopulmonary exercise testing, and brain natriuretic peptide levels were also obtained. In patients with HFpEF, mean left ventricular EF was 61% ± 9% and left ventricular mass index 45 ± 12 g/m 2 . Compared with controls, HFpEF patients had reduced global MPR (2.29 ± 0.64 vs 3.38 ± 0.76, p = 0.002) and VO 2 max (16.5 ± 6.8 vs 30.9 ± 7.7 ml/kg min, p <0.001) whereas extracellular volume (0.29 ± 0.04 vs 0.25 ± 0.04, p = 0.02), pulmonary artery systolic pressure (35.4 ± 13.7 vs 22.3 ± 5.4 mm Hg, p = 0.004), and average E/e' (15.0 ± 7.6 vs 8.6 ± 2.0, p = 0.005) were increased. Displacement encoded stimulated echo peak systolic circumferential strain (p = 0.60) as well as echocardiographic derived global longitudinal strain (p = 0.07) were similar between both groups. The prevalence of CMD, defined as global MPR <2.5, in the HFpEF group was 69%. In conclusion, HFpEF patients have a high prevalence of CMD and diffuse fibrosis. These parameters may be useful clinical end points for future therapeutic trials., (Copyright © 2019 Elsevier Inc. All rights reserved.)

The cardiovascular magnetic resonance imaging (CMR) features of adults with repaired truncus arteriosus (rTA) are largely undefined. We sought to explore CMR characteristics in rTA and to identify associations between imaging findings and cardiovascular outcomes. Adults with rTA and CMR were identified and anatomic subtypes (1-4) were assigned (Collett and Edwards classification). CMR characteristics, clinical data at last follow-up and adverse cardiovascular outcome were recorded. Twenty-seven adults (19% male) were studied (median age at cardiovascular magnetic resonance 26 years [interquartile range 18 to 40]) over 5.2-year duration [interquartile range 2.5 to 7.5]. With the exception of mildly increased RV mass (30 ± 12 g/m 2 ), cardiac chamber measurements were within the normal range. In CMR measurements, only pulmonary artery peak velocity differed in subtypes (highest in subtype 3, 318 ± 26 cm/s, p = 0.029). Number of cardiovascular interventions in adulthood was moderately correlated with left ventricular end-diastolic volume (r = 0.463, p = 0.015), left ventricular ejection fraction (r = 0.425, p = 0.027) and neoaortic root size (r = 0.398, p = 0.039). Cardiovascular events (nonmutually exclusive) in 5 of 27 patients (19%) included death (n = 1), heart failure (n = 1), ventricular tachycardia (n = 1), and atrial tachycardia (n = 3). Increased cardiovascular risk was associated with decreased right ventricular ejection fraction (odds ratio 1.153, confidence interval 1.003 to 1.326, p = 0.046) and smaller ascending aorta diameter (odds ratio 1.758, confidence interval 1.037 to 2.976, p = 0.036). In conclusion, decreased right ventricular ejection fraction and smaller ascending aorta on cardiovascular magnetic resonance were associated with adverse events in rTA., (Copyright © 2019. Published by Elsevier Inc.)

Sudden cardiac death (SCD) is the most life-threating complication of hypertrophic cardiomyopathy. Guidelines of the European Society of Cardiology (ESC) suggest the implantation of an implantable cardioverter defibrillator in primary prevention according to a 5-year risk SCD score ≥6%. The aim of the study is to evaluate the prognostic role of late gadolinium enhancement (LGE) in patients with a 5-year risk SCD score <6%. In this multicenter study, we performed cardiac magnetic resonance in 354 consecutive hypertrophic cardiomyopathy patients (257 males, range of age 54 ± 17) with a risk SCD score <6% (302 with <4% and 52 with ≥4 and <6% risk). Hard cardiac events, including SCD, resuscitated cardiac arrest, appropriate implantable cardioverter defibrillator interventions, sustained ventricular tachycardia, occurred in 22 patients. LGE was detected in a high proportion (92%) of patients with hard cardiac events (p = 0.002). At receiver-operating characteristic curve analysis, LGE extent ≥10% was the best threshold to predict major arrhythmic events (area under the curve: 0.74). Kaplan-Meier curves showed that patients with LGE ≥10% had a worse prognosis than those with lower extent (p < 0.0001). LGE extent was the best independent predictor of hard cardiac events (hazard ratio 1.05; 95% confidence interval [CI] 1.03 to 107; p < 0.0001). The estimates 5-year risk of hard cardiac event was 2.5% (95% CI 0.8 to 4.2) in patients with LGE extent <10% and 23.4% (95% CI 10.2 to 36.5) for those with LGE extent ≥10%. In conclusion, this study demonstrates as the extent of LGE ≥10% is able to recognize additional patients at increased risk for malignant arrhythmic episodes in a population with low-to-intermediate ESC SCD risk score., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Residual aortic regurgitation (AR) is a major complication after transcatheter aortic valve implantation (TAVI). Although the echocardiographic assessment of post-TAVI AR remains challenging, cardiac magnetic resonance (CMR) allows direct quantification of AR. The aim of this study was to review the level of agreement between 2-dimensional transthoracic echocardiography (2D TTE) and CMR on grading the severity of AR after TAVI, and determine the accuracy of TTE in detecting moderate or severe AR. Electronic databases were searched in order to identify studies comparing 2D TTE to CMR for post-TAVI AR assessment. Kappa coefficient was used to determine the level of agreement between the 2 imaging modalities. CMR was used as the reference standard in order to assess the diagnostic accuracy of 2D TTE. Seven studies were included in this systematic review. Six studies reported a low correlation between 2D TTE and CMR (kappa coefficient ranging from -0.02 to 0.41), whereas one study showed good agreement with a kappa coefficient of 0.72. Given the heterogeneity in the included studies the diagnostic accuracy of TTE was evaluated by estimating the hierarchical summary receiver operator characteristic curve. The area under the curve for detection of moderate or severe AR with TTE was 0.83 (95% confidence interval 0.79 to 0.86). In conclusion, despite the reported significant disconcordance between TTE and CMR grading of AR, TTE has sufficient ability to discriminate moderate or severe AR from mild or none AR after TAVI in the clinical setting. CMR should be considered when TTE results are equivocal., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Tricuspid annular (TA) dilation is a key process in functional tricuspid regurgitation, but normal TA dimensions using cardiovascular magnetic resonance have not been established. We measured TA diameters in 66 healthy volunteers, aged 38 ± 11 years, during 3 different phases of the cardiac cycle (end-systole, early diastole, and end-diastole) and in 2 routinely acquired cardiovascular magnetic resonance imaging planes (4-chamber [4C] and right ventricular inflow-outflow [RVIO]). Three readers independently measured each value and 1 reader repeated measurements 1 month apart. The upper limit of normal (ULN) was calculated as 1.96 standard deviations above the mean. We assessed inter- and intraobserver reliability using the intraclass correlation coefficient (ICC) and Bland-Altman analysis. We found the TA diameter largest during early diastole in the 4C view with an ULN of 43 mm (22 mm/m 2 ). Men had larger absolute TA diameters (36 mm, 95% CI 27 to 44 mm) than women (30 mm, 95% CI 23 to 37 mm) but not after indexing for body surface area (both 18 mm/m 2 ). In the RVIO view, the largest TA diameter occurred during early diastole with a ULN value of 46 mm (27 mm/m 2 ). In this view, females had a larger indexed TA than men (21 mm/m 2 vs 17 mm/m 2 ). Reproducibility of measurements was excellent in all cardiac phases with an inter-rater ICC between 0.90 to 0.96 and an intrarater ICC 0.89 to 0.96. In conclusion, we have provided normative data regarding TA dimensions in routinely acquired 4C and RVIO views, and these values are larger than the current thresholds of annular dilation measured by echocardiography. Gender differences with the TA diameter in the RVIO view may be an important finding with consideration of future tricuspid devices., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Clinical risk stratification for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HC) employs rules derived from American College of Cardiology Foundation/American Heart Association (ACCF/AHA) guidelines or the HCM Risk-SCD model (C-index ∼0.69), which utilize a few clinical variables. We assessed whether data-driven machine learning methods that consider a wider range of variables can effectively identify HC patients with ventricular arrhythmias (VAr) that lead to SCD. We scanned the electronic health records of 711 HC patients for sustained ventricular tachycardia or ventricular fibrillation. Patients with ventricular tachycardia or ventricular fibrillation (n = 61) were tagged as VAr cases and the remaining (n = 650) as non-VAr. The 2-sample ttest and information gain criterion were used to identify the most informative clinical variables that distinguish VAr from non-VAr; patient records were reduced to include only these variables. Data imbalance stemming from low number of VAr cases was addressed by applying a combination of over- and undersampling strategies. We trained and tested multiple classifiers under this sampling approach, showing effective classification. We evaluated 93 clinical variables, of which 22 proved predictive of VAr. The ensemble of logistic regression and naïve Bayes classifiers, trained based on these 22 variables and corrected for data imbalance, was most effective in separating VAr from non-VAr cases (sensitivity = 0.73, specificity = 0.76, C-index = 0.83). Our method (HCM-VAr-Risk Model) identified 12 new predictors of VAr, in addition to 10 established SCD predictors. In conclusion, this is the first application of machine learning for identifying HC patients with VAr, using clinical attributes. Our model demonstrates good performance (C-index) compared with currently employed SCD prediction algorithms, while addressing imbalance inherent in clinical data., (Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.)

The 3-vessel and trachea view is now integrated into obstetrical screening and facilitates prenatal detection of vascular rings. We examined trends in prenatal detection, associated cardiac and extracardiac anomalies, and surgical management in this population. We reviewed a population-based cohort of pediatric vascular ring patients diagnosed prenatally and postnatally between 2002 and 2017 in Alberta, Canada. Of 106 cases, 28 (26%) had a prenatal diagnosis. Prenatal detection increased over time: 0/29 from 2002 to 2009, 4/28 (14%) from 2009 to 2011, 7/23 (30%) from 2012 to 2014, and 17/26 (65%) from 2015 to 2017 (p <0.01). The prenatal group more commonly had right aortic arch/left ductus/aberrant left subclavian artery (24/28vs 53/78, p = 0.04) and associated cardiac pathology (18/28vs 33/78, p = 0.05). The rate of genetic anomalies was overall higher than previously reported (34%) and did not differ between groups (11/28vs 25/78, p = 0.48). Those with a prenatal diagnosis were less likely to require cross-sectional imaging (9/28vs 48/78, p <0.01), modifying the vascular ring subtype diagnosis in 2 patients. Surgical intervention was common and did not differ between groups (24/28vs 66/78, p = 0.89). In conclusion, prenatal detection of vascular rings has increased. Despite differences in vascular ring subtype and associated cardiac pathology, the incidence of genetic anomalies and need for surgical intervention is not associated with timing of diagnosis. Genetic counseling should be universally offered. The diagnostic accuracy of echocardiography suggests additional imaging may not be routinely required., (Copyright © 2019 Elsevier Inc. All rights reserved.)

In obstructive hypertrophic cardiomyopathy (HC), extreme heterogeneity of septal morphology makes septal myectomy particularly challenging. Although cardiovascular magnetic resonance (CMR) reconstructs ventricular anatomy with high spatial resolution, CMR is not used systematically to plan preoperatively septal myectomy. In this study, we report our results with using CMR to plan the extent of septal excision in 112 consecutive HC patients who subsequently underwent myectomy. Depth and length of the myectomy planned at CMR were compared with those of the septal muscle excised in a single piece in all patients. Anterior septum maximal thickness at CMR was 22 ± 5 mm and excised muscle thickness 9 ± 3 mm. Planned myectomy length was 35 ± 11 mm (range 17 to 65) and excised muscle length 38 ± 10 mm (range 10 to 70), indicating extension of septal resection to mid-cavity. Thickness and length of the planned myectomy showed a significant correlation with the excised muscle (R 2  = 0.345; p <0.001; and R 2  = 0.358; p <0.001, respectively). Deep septal crypts were identified at CMR in 12(11%) patients, preventing muscle excision from areas at increased risk of iatrogenic septal defect. Large aberrant muscle bundles that could decrease mid-cavity dimension were identified at CMR and excised in 26(23%) patients. In the 55 patients with postoperative CMR, qualitative comparison of pre and postoperative ventricular morphology showed a smooth and apically extended myectomy. In conclusion, CMR planning of septal myectomy provided high resolution images of septal morphology and allowed us to perform a standardized and apically extended septal excision that was associated with favorable outcome. Our novel approach could make myectomy more accessible to cardiovascular surgeons., (Copyright © 2019 Elsevier Inc. All rights reserved.)

There is controversy regarding the utility of left ventricular (LV) mechanics assessed by feature-tracking steady-state free-precession (FT-SSFP), a readily implementable technique in clinical practice. In particular, whether LV mechanics assessed by FT-SSFP predicts outcomes in subjects with heart failure (HF) with reduced ejection fraction (HFrEF), with preserved ejection fraction (HFpEF), or without HF is unknown. We aimed to assess whether LV mechanics measured with FT-SSFP cine magnetic resonance imaging (MRI) predicts adverse outcomes. We prospectively enrolled 612 adults without HF (n = 402), with HF with reduced ejection fraction (HFrEF; n = 113), or HFpEF (n = 97) and assessed LV strain using FT-SSFP cine MRI. Over a median follow-up of 39.5 months, 75 participants had an HF admission, and 85 died. In Cox proportional hazards models, lower global longitudinal (Standardized hazard ratio 1.56, 95% confidence interval [CI] 1.22 to 2.00, p = 0.0004), circumferential (Standardized HR 1.46, 95% CI 1.08 to 1.95, p = 0.0123), and radial strain (Standardized HR 0.59, 95% CI 0.43 to 0.83, p = 0.0019) were independently associated with the composite endpoint, after adjustment for HF status, LV ejection fraction (LVEF), age, sex, ethnicity, body mass index, systolic and diastolic blood pressure, hypertension, diabetes, coronary artery disease, and glomerular filtration rate. Furthermore, global longitudinal strain stratified the risk of adverse outcomes across tertiles better than LVEF. In analyses that included only participants with a preserved LVEF, systolic radial, circumferential and longitudinal strain were independently predictive of adverse outcomes. We conclude that LV longitudinal, circumferential and radial strain measured using FT-SSFP cine MRI (a readily implementable technique in clinical practice) predict the risk of adverse events, independently of LVEF., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Implantable cardioverter defibrillators (ICDs) have been a relative contraindication to cardiovascular magnetic resonance imaging. Although cardiovascular magnetic resonance provides valuable information regarding scar in patients with ventricular arrhythmias or cardiomyopathy, ICDs in these patients frequently cause artifacts hindering accurate interpretation of both cine and late gadolinium enhancement (LGE) images. We sought to quantify the frequency and severity of artifact on LGE images and assess whether a modified wideband LGE protocol could improve the diagnostic yield of scar identification in agreement with invasive electroanatomic mapping (EAM). Forty-nine patients with ICDs and ventricular tachycardia (VT) or cardiomyopathy underwent CMR (Philips 1.5T), including standard and wideband LGE imaging. A safety algorithm was followed throughout the protocol. Standard and wideband LGE short-axis images were graded using an artifact score on a per-slice basis. LGE on wideband images was compared with EAM in 27 of 49 patients who underwent VT ablation. There were no adverse patient- or device-related events. With standard LGE imaging, 84% of patients demonstrated some degree of hyperenhancement artifact, which persisted in 22% on wideband LGE but with much less extent. Wideband LGE imaging resulted in an increase from 48% to 94% diagnostic-quality slices, with a significant reduction in artifact score, and correlated with EAM in 21 of 27 patients (78%). In conclusion, assessment of standard LGE is markedly limited by artifact in patients with ICD. The use of wideband LGE significantly improves image quality and can accurately localize myocardial scar before VT ablation., (Copyright © 2019 Elsevier Inc. All rights reserved.)

International variability in infarct size following acute anterior ST-elevation myocardial infarction without shock treated with primary percutaneous coronary intervention (PCI) has been little studied. Patients enrolled in the Counterpulsation to Reduce Infarct Size pre-PCI for Acute Myocardial Infarction international randomized trial were analyzed according to their region of enrollment: United States (US) (n = 60), Europe/Australia (EU/AU) (n = 104), or India (n = 123). Cardiac magnetic resonance imaging was performed 3-5 days after PCI to assess infarct size, expressed as percentage of left ventricular mass, and analyzed by an imaging core laboratory. The relation between infarct size and region was modelled using multivariable linear regression adjusting for time from symptom onset to first hospital contact, myocardial infarction severity, and baseline characteristics. Infarct size was 36.4% of left ventricular mass in US patients (95% confidence interval [CI] 31.5 to 41.4), 36.5% (95% CI 32.6 to 40.4) in EU/AU patients, and 44.7% (95% CI 41.1 to 48.2) in patients from India (p = 0.01). In multiplicity-adjusted regression analysis, mean infarct size in patients from India was higher than in US patients (mean difference of 8.3%; 95% CI 0.7 to 15.8; p = 0.03), and EU/AU patients (mean difference of 8.2%; 95% CI 1.6 to 14.8; p = 0.01). There was no significance difference in infarct size between patients from the EU/AU and the US (mean difference of 0.1%; 95% CI -7.5 to 7.4; p = 0.99). ln conclusion, in patients with anterior ST-elevation myocardial infarction without cardiogenic shock treated with primary PCI, infarct size was larger in India compared to the United States and EU/AU, even after adjustment for performance metrics, including time to treatment, and other potential confounders. Future studies are needed to better elucidate this discrepancy., (Copyright © 2019 Elsevier Inc. All rights reserved.)

`To analyze the impact of additional coronary artery disease, quantified by the SYNTAX (SYNergy between PCI with TAXus and cardiac surgery) score, on left ventricular ejection fraction (LVEF) and long-term outcomes in a cohort of ST-elevated myocardial infarction (STEMI) patients with a concomitant chronic total coronary occlusion (CTO). A total of 302 STEMI patients were randomized to percutaneous coronary intervention of a CTO (CTO PCI) (n = 148) or conservative CTO treatment (n = 154). SYNTAX scores were calculated by an independent corelab (Cardialysis BV, Rotterdam) at two time-points: (1) at baseline, and (2) after primary PCI in the conservative CTO arm and after CTO PCI in the invasive arm (named 'discharge SYNTAX score'). The population was divided in two groups (below or equal to the median SYNTAX score preprimary PCI, or above the median). At 4-month follow-up, the LVEF was significantly lower in patients in the group with a SYNTAX score above the group median (42.8% vs 48.5%, p = 0.001), and the SYNTAX score was an independent predictor for LVEF at 4 months (β-0.151 (SE 0.068), p = 0.028). In the group with a SYNTAX score above the group median the mortality rate was higher (10.1% vs 3.9%, p = 0.025), and there was a trend towards a higher MACE rate (15.4% vs 8.5%, p = 0.063). In conclusion, in this sub-analysis of the EXPLORE trial we observed a worse LVEF and a higher mortality rate for patients with a SYNTAX score above the median. We found that the SYNTAX score is an independent negative predictor for LVEF and an independent positive predictor for LVEDV at 4-month follow-up., (Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.)

Our aim was to evaluate the temporal changes in left ventricular (LV) diastolic filling in relation to other LV parameters using cardiac MRI (CMR) in patients with HER2 positive breast cancer receiving trastuzumab therapy. Fourty-one women with early stage HER2+ breast cancer underwent serial CMR (baseline, 6, 12, and 18 months) after initiation of trastuzumab therapy. A single, blinded observer measured LV parameters on de-identified CMRs in random order. Linear mixed models were used to investigate temporal changes. Compared to baseline, there were significant decreases in systolic function as measured by both left ventricular ejection fraction (LVEF) (p <0.001 at 6 and 12 months) and peak ejection rate corrected for end-diastolic volume (PER/LVEDV) (p = 0.008 at 6 months, p = 0.01 at 12 months). However, these differences were no longer significant at 18 months. In contrast, significant reductions in diastolic function as measured by LV peak filling rate corrected for end-diastolic volume (PFR/LVEDV) were observed at 6 months (p = 0.012), 12 months (p = 0.031), and up to 18 months (p = 0.034). There were no significant temporal changes in the time to peak filling rate corrected for cardiac cycle (TPF/RR). The reduction in PFR/LVEDV at 18 months was no longer significant when corrected for heart rate. In conclusion, there were significant subclinical deleterious effects on both LV systolic and diastolic function among patients receiving trastuzumab. While there was recovery in LV systolic function after therapy cessation at 18 months, reduction in PFR/LVEDV appeared to persist. Thus, diastolic dysfunction may serve as a marker of trastuzumab-induced cardiotoxicity that needs to be confirmed in a larger study., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Incidence of Yellow Fever (YF) has increased in Brazil, and cardiac findings such as bradyarrhythmias and conduction abnormalities have been described. We aimed to perform a comprehensive cardiac evaluation of patients with YF, and to assess the association between cardiac involvement and disease severity. Patients hospitalized with YF from February to March 2018 underwent clinical and laboratory evaluation, focused bedside echocardiography (GE Vivid IQ), electrocardiogram and, in case of alterations, 24-hours Holter. Patients were divided into 2 groups according to YF severity. Five patients underwent magnetic resonance imaging and 3 had necropsy. Seventy patients had confirmed YF, 69% with severe form. Mean age was 48 ± 14 years, 63 (90%) were males and 5 (7%) died. Significant electrocardiogram abnormalities were present in 52% of patients with mild/moderate form of YF (G1) and 77% of those with severe form (G2), p = 0.046. Sinus bradycardia was observed in 24% (N = 17): G1 23% versus G2 25%, p = 0.67. Among 32 patients who underwent Holter, 14 (44%) had mean HR <60 beats per minute, being 8 from G2. Echocardiogram revealed left ventricular dysfunction in 4 (6%) patients, from G2. Left ventricular wall thickening with a hyper-refringent myocardial texture suggesting infiltration was observed in 17 patients (G1 18% vs G2 27%, p = 0.55). One magnetic resonance (G2) was suggestive of myocarditis, and one necropsy revealed areas of myocardial necrosis and acute myocarditis. In conclusion, cardiac involvement was observed in patients with YF, most commonly bradycardia and myocardial hyper-refringent texture suggestive of infiltration., (Copyright © 2018. Published by Elsevier Inc.)

The use of cardiac magnetic resonance (cMR) to assess remodeling and tissue characterization in primitive and secondary cardiomyopathies has progressively increased, and it carries important prognostic informations. The aim of this study was to assess the overall clinical value of cMR before implantable cardioverter defibrillator (ICD). All patients referred to our center for an ICD implantation and submitted to cMR (n = 134) were analyzed. All the cMR diagnostic findings and following clinical events were reviewed to assess clinical relevance in patients care. The use of cMR before ICD implantation has progressively increased during the decade studied (13% to 53%, p <0.001). Subjects who underwent cMR were younger, more often female, with lower NYHA class and higher ejection fraction (p <0.05 for all). Unexpected diagnostic findings were observed in 34 patients (25%), resulting in an immediate therapeutic strategy modification in 13%. A pattern of fibrosis leading to a change in the disease's etiology and thrombus detection were the most frequent cMR findings, followed by anatomical incidental findings. Any grade of fibrosis carried a higher annual incidence of combined death or ventricular arrhythmias (9.92% vs 1.83%, p = 0.02). Annual event rate was related to the extent of scarring. In conclusion, we observed a progressively increase of cMR utilization before ICD implantation during the last decade. This practice has yielded a significant increase of new diagnostic findings, carrying unique prognostic information linked to tissue characterization., (Copyright © 2018. Published by Elsevier Inc.)

Recent data suggest that myocardial septal late gadolinium enhancement (LGE) may have an independent prognostic value in patients with acute myocarditis undergoing cardiac magnetic resonance (CMR). Aim of the present study is to evaluate its prevalence and prognostic implications in these patients with or without preserved LV function. Retrospective cohort study including all cases of clinically suspected acute myocarditis referred for CMR. A diagnosis of acute myocarditis was confirmed by CMR according to Lake Louise Criteria. Cardiovascular mortality, heart failure, heart transplantation, and sustained ventricular arrhythmias were considered adverse events at follow-up. Seventy-one patients were included in the present study (mean age 47 years 95% confidence intervals 42 to 51, 53 males; 75%). Left Ventricular Ejection Fraction (LVEF) was preserved in 45 cases (63%) and pericardial effusion was detected in 26 cases (38%). CMR was performed at a mean time of 11 days (95% confidence intervals 7.5 to 14.4) from symptoms onset. Myocardial hyperemia and edema were detected in 53 cases (75%), myocardial LGE in 66 cases (93%). Septal LGE was reported in 21 cases (30%). After a mean follow-up of 60.8 months, the mean LVEF increased from 51.6 ± 14.0% to 56.6 ± 10.9% (p = 0.021) and combined adverse events were only recorded in 4 patients (6%) with reduced basal LVEF. These patients had more commonly septal LGE (respectively 58% vs13%, p <0.0001). However, on multivariable analysis septal LGE had no additional predictive value over reduced basal LVEF. In conclusion, our study suggests that septal LGE is not uncommon in patients with acute myocarditis but has no added prognostic value over reduced LVEF at presentation., (Copyright © 2018 Elsevier Inc. All rights reserved.)

We sought to evaluate the prognostic role of cardiac magnetic resonance (CMR) in patients with definite, borderline and possible diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) as defined by the International Task Force (TF) in 2010. CMR was performed in 175 patients: 52 with definite, 50 with borderline and 73 possible ARVC. Abnormal-CMR was defined as the presence of ≥1 CMR abnormalities (including abnormalities of right ventricular and left ventricular wall motion, fat infiltration, late gadolinium enhancement, dilation and dysfunction of either ventricles). During the follow-up time 35 patients had hard cardiac events (sudden cardiac death, appropriate implantable cardioverter defibrillator shock and resuscitated cardiac arrest), and 34 of them occurred in patients with abnormal-CMR (negative predictive value = 96.9%). At the multivariate Cox-regression analysis LV involvement at CMR (fat infiltration and/or late gadolinium enhancement), and episode of nonsustained ventricular tachycardia (NSVT) were independent predictors of cardiac events in both the whole population (LV involvement: HR 3.69, 95% CI 1.57-8.65, p = 0.0002; NSVT: HR 5.8, 95% CI 2.82-11.9, p < 0.0001), and in the group of patients with definite ARVC (LV involvement: HR 3.03, 95% CI 1.15 to 8.02, p = 0.02; NSVT: HR 12.1, 95% CI 4.02-36.5, p < 0.0001). In conclusion, CMR evidence of LV involvement is a strong independent predictor of cardiac events in patients with definite, borderline or possible ARVC diagnosis. Abnormal CMR has very high negative predictive value for hard cardiac events., (Copyright © 2018. Published by Elsevier Inc.)

Cardiac magnetic resonance (CMR) has significant diagnostic and prognostic utility in myocardial infarction evaluation. However, its application in spontaneous coronary artery dissection (SCAD) is not described. Patients with confirmed SCAD who had CMR performed within 8 days of the index SCAD were reviewed. Clinical variables including demographics and medical history were recorded. CMR indexes, including myocardial delayed enhancement (MDE), microvascular obstruction, perfusion defects, left ventricular ejection fraction, and wall motion score index were measured. A total of 18 patients (all women, mean age 47.1 years) were included. Overall burden of atherosclerotic risk factors was low; 3 had a previous SCAD. Two patients underwent CMR before coronary angiography, whereas the others received CMR thereafter. Mean time between SCAD diagnosis and CMR completion was 2.7 days (range 0 to 8). Mean left ventricular ejection fraction and wall motion score index were 56.1% and 1.27, respectively. A total of 15 patients had MDE consistent with myocardial infarction in the SCAD distribution(s) identified on coronary angiography, with 8 patients having concomitant microvascular obstruction. Ten patients had transmural MDE. At follow-up (mean 386 days), all patients were alive; extracoronary vascular abnormalities were identified in 14; 6 had recurrent chest pain; and 2 had recurrent SCAD. Both patients with recurrent SCAD had no unique features on CMR to predict a future event. In conclusion, CMR provided significant value in clarifying the diagnosis and assessing for adverse sequelae after acute SCAD. Further studies are needed to determine its role in SCAD prognostication., (Copyright © 2018. Published by Elsevier Inc.)

In nonhigh risk patients with hypertrophic cardiomyopathy (HC), the presence of extensive late gadolinium enhancement (LGE ext ) at cardiovascular magnetic resonance (CMR) imaging has been proposed as a risk modifier in the decision process for implantable cardioverter defibrillator implantation. With a pretest risk of about 10%, a strategy that alters the likelihood of LGE ext could markedly affect efficacious CMR imaging. Our aim was to study the potential of clinical variables and biomarkers to predict LGE ext . In 98 HC patients without any clear indication for implantable cardioverter defibrillator implantation, we determined the discriminative values of a set of clinical variables and a panel of biomarkers (hs-cTnT, NTproBNP, GDF-15, and Gal-3, CICP) for LGE ext , that is, LGE ≥15% of the left ventricular mass. LGE ext was present in 10% (10/98) of patients. The clinical prediction model contained a history of nonsustained ventricular tachycardia, maximal wall thickness and reduced systolic function (c-statistic: 0.868, p <0.001). Of all biomarkers, only hs-cTnT was associated with LGE ext , in addition to the improved clinical model of diagnostic accuracy (p = 0.04). A biomarker-only strategy allowed the exclusion of LGE ext in half of the cohort, in case of a hs-cTnT concentration less than the optimal cutoff (Youden index; 8 ng/L-sensitivity 100%, specificity 54%). In conclusion, in this nonhigh risk HC cohort, the pretest likelihood of LGE ext can be altered using clinical variables and the addition of hs-cTnT. The promising findings with the use of hs-cTnT only call for new initiatives to study its impact on efficacious CMR imaging in a larger HC population, either with or without additional use of clinical variables., (Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.)

Defining short-term prognosis in nonischemic cardiomyopathy (NICM) is challenging in clinical practice. Although left ventricular reverse remodeling (LVRR) is a key prognostic marker in NICM there are few parameters able to predict it. We investigated whether a complete structural and functional cardiac magnetic resonance imaging (cMRI) evaluation was incremental to the classic clinical-echocardiographic approach in predicting LVRR in a large cohort of NICM patients receiving evidence-based treatment. Patients with a recent diagnosis of NICM (<3 months) who underwent complete clinical, echocardiographic and cMRI assessment were consecutively enrolled from 2008 to 2016. LVRR was defined as an increase in ≥10 points or normalization of left ventricular ejection fraction, associated with a ≥10% reduction or normalization of left ventricular end-diastolic diameter at midterm (median time 20 months) echocardiographic follow-up. Among 80 NICM patients included in the study, LVRR was observed in 43 (54%). At multivariate analysis, the clinical-echocardiographic evaluation failed to identify independent predictors of LVRR. However, absence of late gadolinium enhancement (odds ratio [OR] 9.07; confidence interval [CI] 2.7 to 13.1; p value 0.0003), left ventricular mass (OR 1.018; CI 1.001 to 1.036; p value 0.045) and peak circumferential strain (OR 1.213; CI 1.011 to 1.470; p value 0.049) assessed by cMRI were independently associated with LVRR. A model for LVRR prediction based on cMRI and clinical-echocardiographic parameters performed significantly better than the clinical-echocardiographic model alone (area under curve 0.84 vs 0.72; p value 0.023). In conclusion, an integrated imaging approach with the addition of a structural and functional cMRI study to the standard-of-care evaluation improves the prediction of LVRR in a large cohort of patients with recently diagnosed NICM receiving evidence-based treatment., (Copyright © 2018 Elsevier Inc. All rights reserved.)

The left atrial appendage (LAA) is a nonpulmonary vein trigger site in atrial fibrillation (AF). The association of LAA structural remodeling (SRM) identified by late gadolinium enhancement magnetic resonance imaging (LGE-MRI) and AF ablation outcome has never been described. This study sought to investigate the clinical significance of LAA-SRM in AF patients who undergo ablation therapy. Consecutive patients with AF who underwent catheter ablation therapy within 14 days following MRI scan were included in this study. LAA-SRM was assessed using LGE-MRI images to quantify the extent of LAA-LGE. Patients were followed for arrhythmia recurrence after the ablation procedure. A total of 74 patients were included in the study, 68% were male, with a mean age of 72 years. Mean LAA-LGE extent was 9%. There were 37 arrhythmia recurrences (50%) observed over a mean follow-up period of 18 months. The recurrence rate was significantly higher (73.3% vs 37.5%; p = 0.045) in patients with LAA-LGE extent in the highest tier (T4) compared with the lowest tier (T1). LAA-LGE extent was independently associated with arrhythmia recurrence (adjusted hazard ratio [HR] 1.054; 95% confidence interval [CI] 1.008 to 1.103). In addition, there was an approximately fourfold increased risk of arrhythmia recurrence (adjusted HR 4.117, 95% CI 1.260 to 13.459) in patients with advanced LAA-SRM (T4 vs T1). In conclusion, the extent of LAA-SRM identified by LGE-MRI is associated with arrhythmia recurrence after AF ablation., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Cardiovascular magnetic resonance (CMR) perfusion imaging and fractional flow reserve (FFR) assess myocardial ischemia. FFR measures the pressure loss across a stenosis determining hemodynamic significance but does not assess the area subtended by the stenotic vessel. CMR perfusion imaging measures the extent of myocardial blood flow reduction (=ischemic burden). Both techniques allow for continuous rather than categorical evaluation, but their relationship is poorly understood. This study investigates the relationship between the FFR value and the extent of myocardial ischemia. Forty-nine patients with angina underwent CMR perfusion imaging. FFR was measured in vessels with a visual diameter stenosis >40%. The extent of ischemia for each coronary artery was measured by delineating the perfusion defect on the CMR images and expressing as a percentage of the left ventricular myocardium. The correlation between the extent of ischemia measured by CMR and FFR was good (r = -0.85, p < 0.0005). The mean FFR value was 0.67 ± 0.17, and the mean perfusion defect was 8.9 ± 9.3%. An FFR value of ≥0.75 was not associated with ischemia on CMR. The maximum amount of ischemia (23.0 ± 1.5%) was found at FFR values 0.4 to 0.5. In patients with 1 vessel disease (49%), the mean ischemic burden was 15.3 ± 8.3%. In patients with 2 vessel diseases (18%), the mean ischemic burden was 26.0 ± 12%. Reproducibility for the measurement of ischemic burden was very good with a Kappa coefficient (k = 0.826, p = 0.048). In conclusion, there is good correlation between the FFR value and the amount of myocardial ischemia in the subtended myocardium., (Copyright © 2017. Published by Elsevier Inc.)

Aortic stiffness measured by cardiac magnetic resonance (CMR) in connective tissue disorder (CTD) patients has been previously shown to be abnormal and to be associated with adverse aortic outcomes. The rate of increase in aortic stiffness with normal aging has been previously described. However, longitudinal changes in aortic stiffness have not been characterized in CTD patients. We examined longitudinal changes in CMR-derived aortic stiffness in children and young adults with CTDs. A retrospective analysis of 50 children and young adults (median age, 20 years; range, 0.2 to 49; 40% < 18 years old) with a CTD, and with at least 2 CMR examinations (total 152 examinations) over a median duration of 3.9 (1 to 13.2) years was performed. Aortic stiffness measures (strain, distensibility, and β stiffness index) were calculated on each examination at the aortic root (AoR), ascending aorta, and descending aorta. Longitudinal changes in parameters were analyzed using linear mixed-effects models. Aortic strain and distensibility decreased with age, whereas the β stiffness index increased at all aortic segments. The average rates of decline in distensibility (x10 -3  mm Hg -1 per 10-year increase in age) were 0.7, 1.3, and 1 at the AoR, ascending aorta, and descending aorta, respectively. The rates of decline in distensibility were not associated with the rates of AoR dilation or surgical AoR replacement. In conclusion, on serial CMR measurements in children and young adults with CTDs, aortic stiffness progressively increased with age, with rates of change only slightly higher than those previously reported in healthy adults., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Postinfarction mechanical dispersion (MD), that is, the regional heterogeneity of myocardial contraction throughout the cardiac cycle, has detrimental effects on left ventricular (LV) function and is related to the occurrence of heart failure and ventricular arrhythmias. However, its prevalence, pathophysiological determinants, and clinical utility are still unknown. The aim of the present study is to clarify these issues. In total, 130 consecutive patients (mean age 60 ± 12 years, 75% male) with a first ST-segment elevation myocardial infarction (STEMI) were included. Cardiac magnetic resonance (CMR) with late gadolinium enhancement imaging was performed to assess LV function, infarct size, and microvascular obstruction. Feature-tracking analysis was applied to cine-CMR short-axis images to assess MD, defined as the SD of the time-to-peak circumferential strain of the LV segments expressed as percent cardiac cycle. For comparison purpose, 40 control subjects similar in age and gender to the STEMI group were also included. Patients were followed-up for a median of 95 months; the outcome event was defined as a composite of cardiovascular death, aborted sudden cardiac death, and hospitalization for heart failure. STEMI patients had significantly higher MD compared with controls (12.0 ± 5.35% vs 3.85 ± 0.99%, p <0.001). At multivariate analysis, heart rate (β = 0.20, p = 0.008), LV end-systolic volume index (β = 0.37, p <0.001), and infarct size (β = 0.23, p = 0.017) were significantly and independently related to MD. The outcome event occurred in 26 (20%) patients. At multivariate Cox proportional hazards analysis, MD was significantly and independently related to the outcome event (p <0.001). MD provided significant incremental value over the other clinical and CMR variables in predicting the outcome event (p <0.001 for the chi-square change). In conclusion, MD after STEMI is a marker of the extent of myocardial damage; its assessment by feature-tracking CMR provides significant, independent, and incremental long-term prognostic information., (Copyright © 2017 Elsevier Inc. All rights reserved.)