Comprehensive Cardiovascular Magnetic Resonance Research Articles

P5262Subclinical myocardial abnormalities in systemic sclerosis-associated versus non-connective tissue disease pulmonary hypertension by CMR multiparametric mapping

Abstract Introduction Scleroderma (SSc)-associated pulmonary arterial hypertension (PAH) has the worst prognosis of all PAH subtypes despite having relatively more favourable haemodynamic and cardiac functional profiles. Myocardial abnormalities in SSc have been demonstrated by cardiovascular magnetic resonance (CMR) multiparametric tissue mapping. However, myocardial tissue characterisation studies across distinct PAH subtypes including SSc are limited. Purpose We compared indices of tissue characterisation by CMR multiparametric mapping between patients with SSc with and without PAH, non-connective tissue disease pulmonary hypertension (non-CTD PH) and healthy volunteers. Methods One-hundred and thirty-six patients underwent a CMR study over a 30-month period: 104 patients with systemic sclerosis, of whom 39 had SSc-PAH and 65 had no PH; 32 patients with idiopathic PAH, chronic thromboembolic PH or portopulmonary PH (non-CTD PH group). Patients underwent comprehensive CMR tissue characterisation including quantification of native myocardial T1 (MOLLI), myocardial T2 and ECV from automatically generated tissue maps along with conventional late gadolinium enhancement (LGE) imaging. Twenty age-matched controls underwent the same CMR study protocol. Patients were assessed for PH by right heart catheterisation. Results Native myocardial T1 and myocardial T2 and myocardial ECV are significantly elevated in SSc-PAH versus non-CTD PH (all p<0.05, Figure 1) despite no differences in LV systolic function between these patient cohorts. Patients with SSc have similar degrees of elevated T1, T2 and ECV irrespective of the presence or absence of PAH, suggesting a diffuse myocardial process due to SSc itself. Both SSc sub-groups have significantly higher T1, T2 and ECV compared with controls (all p<0.05). All patients with SSc were subdivided by the presence or absence of ventricular insertion point LGE. Even in the absence of LGE, T1, T2 and ECV were significantly higher in SSc patients versus controls (all p<0.001). However, the presence of focal insertional LGE in SSc was not associated with different burdens of interstitial disease, as defined by median ECV. This highlights the unique role of multiparametric tissue maps in assessing diffuse myocardial involvement beyond the identification of focal LGE. Conclusion Subclinical abnormalities of the myocardium can be detected by CMR multiparametric tissue mapping in patients with SSc. The higher native myocardial T1 and T2 along with the elevated ECV in SSc-PAH are likely to be accounted for by SSc involvement itself. Abnormalities of the myocardial architecture could be a potential contributory reason for the poorer outcomes in SSc-PAH versus non-CTD PH despite the more favourable haemodynamics and right heart function observed in the former patient sub-group. Further work should be directed at determining the prognostic capacity of these metrics in SSc-PAH. Acknowledgement/Funding British Heart Foundation, Action Pharmaceuticals Ltd

Quantitative detection of changes in regional wall motion using real time strain-encoded cardiovascular magnetic resonance

A comprehensive cardiovascular magnetic resonance (CMR) exam includes assessment of regional wall motion, which typically involves qualitative interpretation by an experienced reader, although time-consuming quantitative measurements of myocardial wall thickening can be performed. Myocardial strain imaging offers the potential to more accurately quantify the extent and severity of regional wall motion abnormalities and has even been proposed as a mechanism to identify changes in regional contractility before they are visually evident1. Strain imaging may be particularly valuable to assess the impact of coronary artery disease on the myocardium because ischemia is known to first alter the longitudinal deformation of the subendocardial myocardial fibers prior to impacting the regional thickening in the radial direction that is required to create a visually appreciable wall motion abnormality1. In patients with chest pain, alterations in strain following the infusion of an inotrope such as dobutamine can be used to improve the detection of underlying coronary artery disease2. Myocardial strain can be measured using several different CMR-based tagging techniques, such as complementary spatial modulation of magnetization (C-SPAMM) 3 with harmonic phase encoding (HARP) post-processing 4,5, displacement encoding with stimulating echoes (DENSE) 6, cine phase contrast imaging, and strain-encoded CMR (SENC) 7–9. Additionally, it is also possible to use feature tracking algorithms to derive strain measurements from standard cine-CMR images 10,11. Each of these approaches has its advantages and limitations. Recent improvements in SENC-CMR allow for image acquisition to occur during a single heart beat in real time without sacrificing temporal resolution. Unlike the other strain imaging approaches, with SENC imaging being performed during a single heart beat, image quality is not significantly impacted by respiratory motion and arrhythmia. Previous single center studies have suggested that SENC-CMR can be used to improve the detection of coronary artery disease when compared to cine-image wall motion interpretation 12. In this multi-center study, we hypothesized that in the presence of coronary artery disease, changes in regional longitudinal and circumferential strain detected using SENC under intermediate-dose dobutamine stress CMR would precede changes in wall thickening visible on cine-CMR images.

Altered ascending aortic wall shear stress in patients with corrected atrioventricular septal defect: a comprehensive cardiovascular magnetic resonance and 4D flow MRI evaluation.

In patients after atrioventricular septal defect correction, altered geometry leads to a changed position and subsequent flow over the left ventricular outflow tract. We hypothesised that this altered flow may influence haemodynamics in the ascending aorta. In total, 30 patients after atrioventricular septal defect correction (age 27.6 ± 12.8 years) and 28 healthy volunteers (age 24.8 ± 13.7 years) underwent 4D flow cardiovascular magnetic resonance. Left ventricular ejection fraction and mean and peak wall shear stress calculated at ascending aortic peak systole were obtained from cardiovascular magnetic resonance. Left ventricular outflow tract data including velocity and diameter were obtained from echocardiography. Patients showed a higher mean (911 ± 173 versus 703 ± 154 mPa, p = 0.001) and peak ascending aortic wall shear stress (1264 ± 302 versus 1009 ± 240 mPa, p = 0.001) compared to healthy volunteers. Increased blood flow velocities over the left ventricular outflow tract (1.49 ± 0.30 m/s versus 1.22 ± 0.20 m/s, p < 0.001) correlated well with mean and peak ascending aortic wall shear stress (r = 0.67, p < 0.001 and r = 0.77, p < 0.001). After atrioventricular septal defect correction, increased wall shear stress was observed, which correlated to velocities over the left ventricular outflow tract. These findings imply that altered outflow tract geometry contributes to changed aortic haemodynamics.

Validation of aortic valve 4D flow analysis and myocardial deformation by cardiovascular magnetic resonance in patients after the arterial switch operation

BackgroundAortic regurgitation (AR) and subclinical left ventricular (LV) dysfunction expressed by myocardial deformation imaging are common in patients with transposition of the great arteries after the arterial switch operation (ASO). Echocardiographic evaluation is often hampered by reduced acoustic window settings. Cardiovascular magnetic resonance (CMR) imaging provides a robust alternative as it allows for comprehensive assessment of degree of AR and LV function. The purpose of this study is to validate CMR based 4-dimensional flow quantification (4D flow) for degree of AR and feature tracking strain measurements for LV deformation assessment in ASO patients.MethodsA total of 81 ASO patients (median 20.6 years, IQR 13.5–28.4) underwent CMR for 4D and 2D flow analysis. CMR global longitudinal strain (GLS) feature tracking was compared to echocardiographic (echo) speckle tracking. Agreements between and within tests were expressed as intra-class correlation coefficients (ICC).ResultsEleven ASO patients (13.6%) showed AR > 5% by 4D flow, with good correlation to 2D flow assessment (ICC = 0.85). 4D flow stroke volume of the aortic valve demonstrated good agreement to 2D stroke volume over the mitral valve (internal validation, ICC = 0.85) and multi-slice planimetric LV stroke volume (external validation, ICC = 0.95). 2D flow stroke volume showed slightly less, though still good agreement with 4D flow (ICC = 0.78) and planimetric LV stroke volume (ICC = 0.87). GLS by CMR was normal (− 18.8 ± 4.4%) and demonstrated good agreement with GLS and segmental analysis by echocardiographic speckle tracking (GLS = − 17.3 ± 3.1%, ICC of 0.80).ConclusionsAortic 4D flow and CMR feature tracking GLS analysis demonstrate good to excellent agreement with 2D flow assessment and echocardiographic speckle tracking, respectively, and can therefore reliably be used for an integrated and comprehensive CMR analysis of aortic valve competence and LV deformation analysis in ASO patients.

Identification of Cardiomyopathy-Associated Circulating miRNA Biomarkers in Muscular Dystrophy Female Carriers Using a Complementary Cardiac Imaging and Plasma Profiling Approach.

Background: Different from males with Duchenne/Becker muscular dystrophy (DMD/BMD) in whom overt myopathy is the rule, muscular dystrophy (MD) female carriers are mostly free of skeletal muscle symptoms. However, similar to MD males, these females are also prone to cardiomyopathy. Since circulating microRNAs (miRNAs) have been proposed as diagnostic biomarkers for various cardiovascular diseases, the aim of the current study was to identify specific circulating miRNAs in the plasma of female DMD/BMD carriers that may allow an early and accurate diagnosis of cardiac involvement in these cases.Methods: Twenty-nine female MD carriers and 24 age-matched healthy female controls were prospectively enrolled. All MD carriers and controls underwent comprehensive cardiovascular magnetic resonance (CMR) studies as well as venous blood sampling on the same day.Results: An impaired left ventricular (LV) systolic function was detected in 4 (14%) MD carriers while late gadolinium enhancement (LGE) indicative of myocardial fibrosis was present in 13 female patients (45%)—with an exclusively non-ischemic pattern. Among the circulating miRNAs examined, six were significantly up-regulated in MD carriers compared to female controls: miR-206 (103-fold increase, p < 0.0001), miR-222 (41-fold, p < 0.0001), miR-26a (fourfold, p = 0.029), miR-342 (27-fold, p < 0.0001), miR-378a-3p (minimum 3,600-fold; almost undetectable in controls, p = 0.013), miR-378a-5p (64-fold, p < 0.0001); only two miRNAs were substantially down-regulated in MD carriers: miR-144 (p < 0.0001) and miR-29a (p = 0.002) (both undetectable in carriers). A significant down-regulation of the miR-29c (<0.001-fold, p = 0.006) was observed in MD carriers with abnormal CMR findings (comprising functional and/or structural abnormalities) compared to those with normal CMR examinations. Univariable analyses regarding the presence of abnormal CMR findings resulted in four significant variables: LV end-diastolic volume index (EDVi), LV end-systolic volume index (ESVi), an elevated plasma creatine kinase (CK), and decreased serum miR-29c levels. In subsequent multivariable analysis, the only independent predictor for an abnormal CMR among MD carriers was circulating miR-29c (OR 0.99, 95% CI 0.98–0.99, p = 0.037). Moreover, an elevated CK and/or a downregulated miR-29c level (<0.05 × 10-3) resulted in an improved AUC value of 0.79 (0.62–0.97, p = 0.007) (79, 80 and 80%, sensitivity, specificity and overall accuracy) for the CMR-based diagnosis of cardiomyopathy in MD carriers when compared to using the two parameters individually.Conclusion: In female MD carriers, down-regulation of circulating miR-29c relates to the presence of functional and/or structural cardiac abnormalities (as detected by CMR) and appears to be a promising novel biomarker—in addition to conventional CK plasma levels—for an early diagnosis of cardiomyopathy.

Diffuse myocardial fibrosis in adolescents operated with arterial switch for transposition of the great arteries - A CMR study

Diffuse myocardial fibrosis in adolescents operated with arterial switch for transposition of the great arteries - A CMR study

The heart in systemic lupus erythematosus - A comprehensive approach by cardiovascular magnetic resonance tomography.

BackgroundIn systemic lupus erythematosus (SLE), cardiac manifestations, e.g. coronary artery disease (CAD) and myocarditis are leading causes of morbidity and mortality. The prevalence of subclinical heart disease in SLE is unknown. We studied whether a comprehensive cardiovascular magnetic resonance (CMR) protocol may be useful for early diagnosis of heart disease in SLE patients without known CAD.MethodsIn this prospective, observational, cross-sectional study CMR including cine, late gadolinium enhancement (LGE) and stress perfusion sequences, ECG, and blood sampling were performed in 30 consecutive SLE patients without known CAD. All patients fulfilled at least 4/11 American College of Rheumatology (ACR) Criteria for the classification of SLE.Results30 patients (83% female) were enrolled, mean age was 45±14 years and mean SLE disease duration was 10±8 years. 80% had low to moderate disease activity. All had a low SLE damage index. CMR was abnormal in 13/30 (43%), showing LGE in 9/13, stress perfusion deficits in 5/13 and pericardial effusion (PE) in 7/13. Patients with non-ischemic LGE had more often microalbuminuria while patients with stress perfusion deficits a history of hypertension, renal disorder as ACR criterion, repolarisation abnormalities on ECG and larger LV enddiastolic volume index. There was no correlation between clinical symptoms and CMR results.ConclusionOur study shows that cardiac involvement as observed by CMR is frequent in SLE and not necessarily associated with typical symptoms. CMR may thus help to detect subclinical cardiac involvement, which could lead to earlier treatment. Additionally we identify possible risk factors associated with cardiac involvement.

CMR-derived extracellular volume fraction (ECV) in asymptomatic heart transplant recipients: correlations with clinical features and myocardial edema

Myocardial interstitial expansion seems to be fundamental to the process of adverse left ventricular remodeling. Recent evidence has shown that the extracellular volume fraction (ECV) derived from cardiovascular magnetic resonance (CMR) can be used as a noninvasive method to quantify myocardial interstitial volume in a range of heart diseases. Our aim was to determine whether ECV is increased in asymptomatic orthotopic heart transplant (HTx) patients and its associations with clinical features and T2 values, the elevation of which usually suggests myocardial edema. A group of asymptomatic cardiac transplant recipients and some healthy volunteers were invited to undergo a comprehensive CMR scan, including cine imaging, late gadolinium enhancement, T1 mapping and T2 mapping, from March to June in 2017. All quantitative measurements were averaged from the basal and mid short-axis slices. Fifty-eight recipients (mean age, 42.7 ± 11.5years; 13 females), at a median of 1.8years (0.3-6.3years) after HTx, and 20 healthy volunteers (mean age, 39.5 ± 11.3 years; 5 females) underwent the CMR scan. We found that both the ECV and T2 values were higher in the post-HTx group (ECV: 26.7 ± 3.3 vs. 24.6 ± 2.5%, p = 0.008; T2: 47.7 ± 2.8 vs. 44.5 ± 1.6ms, p < 0.001) than in the control group. ECV was moderately associated with organ ischemia time at the time of transplantation but not with the hemodynamics parameter or the time since transplantation at CMR. Additionally, a relatively strong correlation was observed between ECV and T2 (r = 0.7, p < 0.001). So, our conclusion is thatCMR-derived ECV is increased and associated with peri-transplant ischemia time in asymptomatic HTx patients. Andthe strong correlation of ECV with elevated T2 indicates that myocardial edema may be an important part of the extracellular volume expansion after heart transplantation.

Cardiac Magnetic Resonance in Stable Coronary Artery Disease: Added Prognostic Value to Conventional Risk Profiling.

Aims Cardiovascular magnetic resonance (CMR) permits a comprehensive evaluation of stable coronary artery disease (CAD). We sought to assess whether, in a large contemporaneous population receiving optimal medical therapy, CMR independently predicts prognosis beyond conventional cardiovascular risk factors (RF). Methods We performed a single centre, observational prospective study that enrolled 465 CAD patients (80% males; 63±11 years), optimally treated with ACE-inhibitors/ARB, aspirin, and statins (76-85%). Assessments included conventional evaluation (clinical history, atherosclerosis RF, electrocardiography, and echocardiography) and a comprehensive CMR with LV dimensions/function, late gadolinium enhancement (LGE), and stress perfusion CMR (SPCMR). Results During a median follow-up of 62 months (IQR 23-74) there were 50 deaths and 92 major adverse cardiovascular events (MACE). CMR variables improved multivariate model prediction power of mortality and MACE over traditional RF alone (F-test p<0.05 and p<0.001, respectively). LGE was an independent prognostic factor of mortality (hazard ratio [95% CI]: 3.4 [1.3−8.8]); moreover, LGE (3.3 [1.7−6.3]) and SPCMR (2.1 [1.4−3.2]) were the best predictors of MACE. Conclusion LGE is an independent noninvasive marker of mortality in the long term in patients with stable CAD and optimized medical therapy. Furthermore, LGE and SPCMR independently predict MACE beyond conventional risk stratification.

Is MRI equivalent to CT in the guidance of TAVR? A pilot study.

To compare a comprehensive cardiovascular magnetic resonance imaging (MRI) protocol with contrast-enhanced computed tomography angiography (CTA) for guidance in transcatheter aortic valve replacement (TAVR) evaluation. Non-contrast three-dimensional (3D) 'whole heart' MRI imaging for aortic annulus sizing and measurements of coronary ostia heights, contrast-enhanced MRI angiography (MRA) for evaluation of transfemoral routes as well as aortoiliofemoral-CTA were performed in 16 patients referred for evaluation of TAVR. Aortic annulus measurements by MRI and CTA showed a very strong correlation (r=0.956, p<0.0001; effective annulus area for MRI 430±74 vs. 428±78 mm2 for CTA, p=0.629). Regarding decision for valve size there was complete consistency between MRI and CTA. Moreover, vessel luminal diameters and angulations of aortoiliofemoral access as measured by MRA and CTA showed overall very strong correlations (r= 0.819 to 0.996, all p<0.001), the agreement of minimal vessel diameter between the two modalities revealed a bias of 0.02 mm (upper and lower limit of agreement: 1.02 mm and -0.98 mm). In patients referred for TAVR, MRI measurements of aortic annulus and minimal aortoiliofemoral diameters showed good to excellent agreement. Decisions based on MRI measurements regrading prosthesis sizing and transfemoral access would not have modified TAVR-strategy as compared to a CTA-based choice. • 'Whole heart' MRI and CTA measurements of aortic annulus correlate very strongly. • MRI- and CTA-based prostheses sizing are in excellent agreement. • MRA and CTA equally guide TAVR access strategy.

Additive value of 3T cardiovascular magnetic resonance coronary angiography for detecting coronary artery disease

BackgroundThe purpose of the work was to evaluate the incremental diagnostic value of free-breathing, contrast-enhanced, whole-heart, 3 T cardiovascular magnetic resonance coronary angiography (CE-MRCA) to stress/rest myocardial perfusion imaging (MPI) and late gadolinium enhancement (LGE) imaging for detecting coronary artery disease (CAD).MethodsFifty-one patients with suspected CAD underwent a comprehensive cardiovascular magnetic resonance (CMR) examination (CE-MRCA, MPI, and LGE). The additive diagnostic value of MRCA to MPI and LGE was evaluated using invasive x-ray coronary angiography (XA) as the standard for defining functionally significant CAD (≥ 50% stenosis in vessels > 2 mm in diameter).Results90.2% (46/51) patients (54.0 ± 11.5 years; 71.7% men) completed CE-MRCA successfully. On per-patient basis, compared to MPI/LGE alone or MPI alone, the addition of MRCA resulted in higher sensitivity (100% vs. 76.5%, p < 0.01), no change in specificity (58.3% vs. 66.7%, p = 0.6), and higher accuracy (89.1% vs 73.9%, p < 0.01) for CAD detection (prevalence = 73.9%). Compared to LGE alone, the addition of CE-MRCA resulted in higher sensitivity (97.1% vs. 41.2%, p < 0.01), inferior specificity (83.3% vs. 91.7%, p = 0.02), and higher diagnostic accuracy (93.5% vs. 54.3%, p < 0.01).ConclusionThe inclusion of successful free-breathing, whole-heart, 3 T CE-MRCA significantly improved the sensitivity and diagnostic accuracy as compared to MPI and LGE alone for CAD detection.

Cardiovascular magnetic resonance evidence of myocardial fibrosis and its clinical significance in adolescent and adult patients with Ebstein\u2019s anomaly

BackgroundMyocardial fibrosis is a common pathophysiological process that is related to ventricular remodeling in congenital heart disease. However, the presence, characteristics, and clinical significance of myocardial fibrosis in Ebstein’s anomaly have not been fully investigated. This study aimed to evaluate myocardial fibrosis using cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) and T1 mapping techniques, and to explore the significance of myocardial fibrosis in adolescent and adult patients with Ebstein’s anomaly.MethodsForty-four consecutive patients with unrepaired Ebstein’s anomaly (34.0 ± 16.2 years; 18 males), and an equal number of age- and gender-matched controls, were included. A comprehensive CMR protocol consisted of cine, LGE, and T1 mapping by modified Look-Locker inversion recovery (MOLLI) sequences were performed. Ventricular functional parameters, native T1, extracellular volume (ECV), and LGE were analyzed. Associations between myocardial fibrosis and disease severity, ventricular function, and NYHA classification were analyzed.ResultsLGE was found in 10 (22.7%) patients. Typical LGE in Ebstein’s anomaly was located in the endocardium of the septum within the right ventricle (RV). The LV ECV of Ebstein’s anomaly were significantly higher than those of the controls (30.0 ± 3.8% vs. 25.3 ± 2.3%, P < 0.001). An increased ECV was found to be independent of the existence of LGE. Positive LGE or higher ECV (≥30%) was associated with larger fRV volume, aRV volume, increased disease severity, and worse NYHA functional class. In addition, ECV was significantly correlated with the LV ejection fraction (P < 0.001).ConclusionsBoth focal and diffuse myocardial fibrosis were observed in adolescent and adult patients with Ebstein’s anomaly. Increased diffuse fibrosis is associated with worse LV function, increased Ebstein’s severity, and worse clinical status.

020 Comprehensive cardiovascular magnetic resonance assessment of anderson-fabry cardiomyopathy- natural history and assessment of treatment effect

Background The Anderson Fabry Cardiomyopathy (AFC) is heterogeneous, complex, and its pathophysiology remains incompletely understood. There is uncertainty regarding the optimal timing and impact of enzyme replacement therapy (ERT) in cardiac involvement, with a pressing need for biomarkers able to identify early disease, monitor and guide treatment. Objectives The aims of this study were two-fold: 1) to perform comprehensive cardiac phenotyping in a large cohort of Anderson-Fabry disease (AFD), and 2) characterise their short-term natural history and assess the impact of ERT using multi-parametric Cardiac Magnetic resonance imaging (MPCMRi), echocardiography and ECG analysis. Methods 113 prospectively enrolled, genetically-confirmed, AFD patients were compared to 20 age-matched healthy volunteers (HVs). 37 of these patients re-attended for a one-year follow-up scan, and were sub-grouped according to treatment status (newly started on ERT, ERT naive, and established on ERT). Results The AFC is characterised by increased LV mass of several different hypertrophic patterns, impaired LV global longitudinal strain and left atrial function, low myocardial native T1, high T2, and increased extracellular volume fraction. Significantly higher T1s were seen in the inferolateral wall, even before the development of increased wall thickness or LV mass, or LGE. A newly proposed marker of disease severity, the ‘T1 ratio’ , reflects the relationship between inferolateral and remote myocardial T1, and strongly correlated with LV mass, percentage LGE and ECV fraction, whilst avoiding potential uncertainty caused by pseudo-normalisation of T1s in severe AFD. ERT initiation reduced LVM. T1 values (excluding the inferolateral wall) fell significantly in untreated patients (974±36 ms v 955±44 ms, p=0.04) associated with a significant increase in voxel based spread (288 v 350, p=0.02), whereas both treatment groups showed no change in T1 values. The T1 ratio showed a trend towards improvement (0.94±0.08 v 0.98±0.1, p=0.05) in the established ERT group. Electrocardiography and echocardiography did not detect disease progression or treatment effects in any group. Conclusions AFC is a complex pathology with intracellular and extracelluar disease components. MPCMRi elucidates these disease processes and allows early disease detection, and possibly disease and treatment monitoring.

Quantification of both the area-at-risk and acute myocardial infarct size in ST-segment elevation myocardial infarction using T1-mapping

BackgroundA comprehensive cardiovascular magnetic resonance (CMR) in reperfused ST-segment myocardial infarction (STEMI) patients can be challenging to perform and can be time-consuming. We aimed to investigate whether native T1-mapping can accurately delineate the edema-based area-at-risk (AAR) and post-contrast T1-mapping and synthetic late gadolinium (LGE) images can quantify MI size at 1.5 T. Conventional LGE imaging and T2-mapping could then be omitted, thereby shortening the scan duration.MethodsTwenty-eight STEMI patients underwent a CMR scan at 1.5 T, 3 ± 1 days following primary percutaneous coronary intervention. The AAR was quantified using both native T1 and T2-mapping. MI size was quantified using conventional LGE, post-contrast T1-mapping and synthetic magnitude-reconstructed inversion recovery (MagIR) LGE and synthetic phase-sensitive inversion recovery (PSIR) LGE, derived from the post-contrast T1 maps.ResultsNative T1-mapping performed as well as T2-mapping in delineating the AAR (41.6 ± 11.9% of the left ventricle [% LV] versus 41.7 ± 12.2% LV, P = 0.72; R2 0.97; ICC 0.986 (0.969–0.993); bias −0.1 ± 4.2% LV). There were excellent correlation and inter-method agreement with no bias, between MI size by conventional LGE, synthetic MagIR LGE (bias 0.2 ± 2.2%LV, P = 0.35), synthetic PSIR LGE (bias 0.4 ± 2.2% LV, P = 0.060) and post-contrast T1-mapping (bias 0.3 ± 1.8% LV, P = 0.10). The mean scan duration was 58 ± 4 min. Not performing T2 mapping (6 ± 1 min) and conventional LGE (10 ± 1 min) would shorten the CMR study by 15–20 min.ConclusionsT1-mapping can accurately quantify both the edema-based AAR (using native T1 maps) and acute MI size (using post-contrast T1 maps) in STEMI patients without major cardiovascular risk factors. This approach would shorten the duration of a comprehensive CMR study without significantly compromising on data acquisition and would obviate the need to perform T2 maps and LGE imaging.

Hemorrhage promotes inflammation and myocardial damage following acute myocardial infarction: insights from a novel preclinical model and cardiovascular magnetic resonance

BackgroundMyocardial hemorrhage is a frequent complication following reperfusion in acute myocardial infarction and is predictive of adverse outcomes. However, it remains unsettled whether hemorrhage is simply a marker of a severe initial ischemic insult or directly contributes to downstream myocardial damage. Our objective was to evaluate the contribution of hemorrhage towards inflammation, microvascular obstruction and infarct size in a novel porcine model of hemorrhagic myocardial infarction using cardiovascular magnetic resonance (CMR).MethodsMyocardial hemorrhage was induced via direct intracoronary injection of collagenase in a novel porcine model of ischemic injury. Animals (N = 27) were subjected to coronary balloon occlusion followed by reperfusion and divided into three groups (N = 9/group): 8 min ischemia with collagenase (+HEM); 45 min infarction with saline (I-HEM); and 45 min infarction with collagenase (I+HEM). Comprehensive CMR was performed on a 3 T scanner at baseline and 24 h post-intervention. Cardiac function was quantified by cine imaging, edema/inflammation by T2 mapping, hemorrhage by T2* mapping and infarct/microvascular obstruction size by gadolinium enhancement. Animals were subsequently sacrificed and explanted hearts underwent histopathological assessment for ischemic damage and inflammation.ResultsAt 24 h, the +HEM group induced only hemorrhage, the I-HEM group resulted in a non-hemorrhagic infarction, and the I+HEM group resulted in infarction and hemorrhage. Notably, the I+HEM group demonstrated greater hemorrhage and edema, larger infarct size and higher incidence of microvascular obstruction. Interestingly, hemorrhage alone (+HEM) also resulted in an observable inflammatory response, similar to that arising from a mild ischemic insult (I-HEM). CMR findings were in good agreement with histological staining patterns.ConclusionsHemorrhage is not simply a bystander, but an active modulator of tissue response, including inflammation and microvascular and myocardial damage beyond the initial ischemic insult. A mechanistic understanding of the pathophysiology of reperfusion hemorrhage will potentially aid better management of high-risk patients who are prone to adverse long-term outcomes.

39 The Impact of New Left Bundle Branch Block Following Trans-Catheter Aortic Valve Impantation. Is There a TAVI LBBB-induced Cardiomyopathy? Insights from Cardiovascular Magnetic Resonance Imaging

Introduction Left bundle branch block (LBBB) is common following trans-catheter aortic valve implantation (TAVI) and has been linked to increased mortality, although whether this is due to the development of a TAVI-induced LBBB cardiomyopathy is unclear. Methods 48 patients undergoing TAVI for severe aortic stenosis were evaluated. 24 patients with TAVI-induced LBBB (LBBB-T) were matched with 24 patients with a narrow post-procedure QRS (nQRS). Patients underwent comprehensive Cardiovascular Magnetic Resonance (CMR) imaging prior to and 6m post-TAVI. Measured cardiac reverse remodelling parameters included left ventricular ejection fraction (LVEF), global longitudinal strain (GLS) and left sided chamber size. Inter and intraventricular dyssynchrony was determined using time to peak radial strain derived from CMR Feature Tracking. Results In the nQRS group there was no change in QRSd (93 ± 17 to 96 ± 11 ms, p = 0.098). In the LBBB-T group, QRSd increased by a mean of 55 ms from 96 ± 14 to 151 ± 12 (p= 18.6 ± 5.8 mm, p = 0.773). Post-procedure aortic regurgitant fraction was similar between groups (nQRS 5.4 ± 5.7 vs LBBB-T 5.5 ± 3.3%, p = 0.948). There was an inverse correlation between QRS duration and change in LVEF (r=-0.46, p = 0.001) and QRS duration and change in LV GLS (r=-0.37, p = 0.010). Conclusion LBBB-T is associated with less favourable cardiac reverse remodelling at medium term follow up. In view of this, every effort should be made to prevent TAVI-induced LBBB, especially as TAVI is extended to a younger, lower risk population.

Athletic Cardiac Adaptation in Males Is a Consequence of Elevated Myocyte Mass.

Background—Cardiac remodeling occurs in response to regular athletic training, and the degree of remodeling is associated with fitness. Understanding the myocardial structural changes in athlete’s heart is important to develop tools that differentiate athletic from cardiomyopathic change. We hypothesized that athletic left ventricular hypertrophy is a consequence of increased myocardial cellular rather than extracellular mass as measured by cardiovascular magnetic resonance.Methods and Results—Forty-five males (30 athletes and 15 sedentary age-matched healthy controls) underwent comprehensive cardiovascular magnetic resonance studies, including native and postcontrast T1 mapping for extracellular volume calculation. In addition, the 30 athletes performed a maximal exercise test to assess aerobic capacity and anaerobic threshold. Participants were grouped by athleticism: untrained, low performance, and high performance (O2max <60 or>60 mL/kg per min, respectively). In athletes, indexed cellular mass was greater in high- than low-performance athletes 60.7±7.5 versus 48.6±6.3 g/m2; P<0.001), whereas extracellular mass was constant (16.3±2.2 versus 15.3±2.2 g/m2; P=0.20). Indexed left ventricular end-diastolic volume and mass correlated with O2max (r=0.45, P=0.01; r=0.55, P=0.002) and differed significantly by group (P=0.01; P<0.001, respectively). Extracellular volume had an inverse correlation with O2max (r=−0.53, P=0.003 and left ventricular mass index (r=-0.44, P=0.02).Conclusions—Increasing left ventricular mass in athlete’s heart occurs because of an expansion of the cellular compartment while the extracellular volume becomes relatively smaller: a difference which becomes more marked as left ventricular mass increases. Athletic remodeling, both on a macroscopic and cellular level, is associated with the degree of an individual’s fitness. Cardiovascular magnetic resonance ECV quantification may have a future role in differentiating athlete’s heart from change secondary to cardiomyopathy.

Identification of cardiomyopathy associated circulating miRNA biomarkers in patients with muscular dystrophy using a complementary cardiovascular magnetic resonance and plasma profiling approach.

BackgroundDuchenne and Becker muscular dystrophy (DMD and BMD) are X-chromosomal recessive neuromuscular disorders that are caused by mutations in the dystrophin gene and characterized by cardiac involvement. Circulating microRNAs (miRNAs) have been proposed as diagnostic biomarkers for various cardiovascular diseases. However, circulating miRNAs reflecting the presence and/or disease severity of cardiac involvement in DMD/BMD patients have not been described so far.MethodsSixty-three male patients with known MD and 26 age-matched healthy male controls were prospectively enrolled. All MD patients and controls underwent comprehensive cardiovascular magnetic resonance (CMR) studies as well as venous blood sampling on the same day.ResultsAn impaired left ventricular (LV) systolic function (defined as LV-EF <55 %) was detected in 29 (46 %) and presence of late gadolinium enhancement (LGE) indicative of myocardial fibrosis in 48 (76 %) MD patients with an exclusively non-ischemic pattern. Whereas no significant differences were observed for the 27 selected circulating miRNAs in MD patients with abnormal CMR findings (comprising structural and/or functional impairments) compared to those with completely normal CMR studies, a significant up-regulation of three miRNAs was observed in LGE-positive MD patients compared to LGE-negative ones: miR-222 (1.8-fold, p = 0.035), miR-26a (2.1-fold, p = 0.03) and miR-378a-5p (2.4-fold, p = 0.026). A signature of these three miRNAs (miR-26a, miR-222 and miR-378a-5p) resulted in an area under the curve (AUC) value of 0.74 for the diagnosis of LGE-positive MD patients. In a multivariable model, three independent predictors for LGE presence were identified comprising not only clinical and laboratory markers (LV-EF: OR 0.47, 95 % CI 0.24-0.89, p = 0.021 and elevated hs-Trop: OR 2559, 95 % CI 2.97-22.04*105, p = 0.023) but also the circulating miR-222 (OR 938, 95 % CI 938.46, 3.56-24.73*104, p = 0.016).ConclusionsUp-regulation of circulating miRNAs miR-222, miR-26a and miR-378a-5p indicates the presence of myocardial scars in MD patients. Plasma miR-222 appears to be a promising novel biomarker reflecting structural – but not functional – cardiac alterations in MD patients.Electronic supplementary materialThe online version of this article (doi:10.1186/s12968-016-0244-3) contains supplementary material, which is available to authorized users.

Pattern and prognostic value of cardiac involvement in patients with late-onset pompe disease: a comprehensive cardiovascular magnetic resonance approach

BackgroundPompe disease is an autosomal recessive disorder caused by deficiency of the lysosomal α–1,4-glucosidase leading to accumulation of glycogen in target tissues with progressive organ failure. While the early infantile-onset form is characterized by early severe hypertrophic cardiomyopathy with cardiac and respiratory failure, clinically relevant cardiomyopathy seems to be uncommon in patients with late-onset Pompe disease, and the prevalence and nature of myocardial abnormalities are still to be clarified.MethodsSeventeen patients with genetically proven late-onset Pompe disease (50 ± 18 years, 11 male) and 18 age- and gender-matched healthy controls (44 ± 10 year, 12 male) underwent comprehensive cardiovascular magnetic resonance (CMR) including conventional and advanced techniques: cine and feature tracking-based strain imaging for depiction of (even subtle) systolic LV dysfunction as well as late gadolinium enhancement (LGE) and myocardial extracellular volume fraction (ECV) quantification for focal and diffuse fibrosis detection.ResultsAll patients had normal left ventricular (LV) and right ventricular (RV) volumes and normal LV and RV ejection fraction. In comparison to healthy controls, neither conventional cine nor advanced feature-tracking based-strain imaging could depict any (subclinical) myocardial systolic dysfunction. Three (18%) of the patients had non-ischemic LGE in the basal inferolateral wall and 21% demonstrated elevated global ECV values suggestive of interstitial myocardial fibrosis. Non-specific abnormalities such as left atrial (LA) dilatation were present in two patients, while LV hypertrophy was seen only in one. Two of the three LGE-positive patients were also hypertensive and demonstrated high global ECV values (>30%) in addition to dilated LA. After a median follow-up of 25 (11–29) months, only one cardiovascular event occurred: one of the LGE-positive patients with a high cardiovascular risk profile suffered an acute coronary syndrome.ConclusionIn contrast to the early infantile-onset form of Pompe disease, mild and rather non-specific cardiac abnormalities can be detected by CMR only in a small proportion of patients with late-onset Pompe disease. The observed structural abnormalities seem to result from an interplay between the storage disease and other comorbidities and they did not affect short-term to mid-term prognosis in adult Pompe patients.

Ascending aortic wall shear stress and distensibility are different in patients with corrected atrioventricular septal defect compared to healthy controls: a comprehensive CMR and 4D flow MRI evaluation

Background In patients with an atrioventricular septal defect (AVSD), the left ventricular outflow tract (LVOT) and the ascending aorta (AAo) are located more anteriorly due to the position of the common AV valve. This may alter proximal aortic flow, and we hypothesize that this can result in impaired LV systolic function and AAo wall degeneration. We aimed to quantify differences in AAo wall shear stress (WSS), distensibility (Dist), aortic arch pulse wave velocity (PWV) and LV ejection fraction (EF) in these patients versus healthy controls using cardiovascular magnetic resonance (CMR) and four dimensional (4D) flow MRI.