Search

The Society for Cardiovascular Magnetic Resonance (SCMR) is an international society focused on the research, education, and clinical application of cardiovascular magnetic resonance (CMR). The SCMR web site ( https://www.scmr.org ) hosts a case series designed to present case reports demonstrating the unique attributes of CMR in the diagnosis or management of cardiovascular disease. Each clinical presentation is followed by a brief discussion of the disease and unique role of CMR in disease diagnosis or management guidance. By nature, some of these are somewhat esoteric, but all are instructive. In this publication, we provide a digital archive of the 2019 Case of the Week series as a means of further enhancing the education of those interested in CMR and as a means of more readily identifying these cases using a PubMed or similar search engine.

Background: Gadolinium-based contrast agents were not approved in the United States for detecting coronary artery disease (CAD) prior to the current studies., Objectives: The purpose of this study was to determine the sensitivity and specificity of gadobutrol for detection of CAD by assessing myocardial perfusion and late gadolinium enhancement (LGE) imaging., Methods: Two international, single-vendor, phase 3 clinical trials of near identical design, "GadaCAD1" and "GadaCAD2," were performed. Cardiovascular magnetic resonance (CMR) included gadobutrol-enhanced first-pass vasodilator stress and rest perfusion followed by LGE imaging. CAD was defined by quantitative coronary angiography (QCA) but computed tomography coronary angiography could exclude significant CAD., Results: Because the design and results for GadaCAD1 (n = 376) and GadaCAD2 (n = 388) were very similar, results were summarized as a fixed-effect meta-analysis (n = 764). The prevalence of CAD was 27.8% defined by a ≥70% QCA stenosis. For detection of a ≥70% QCA stenosis, the sensitivity of CMR was 78.9%, specificity was 86.8%, and area under the curve was 0.871. The sensitivity and specificity for multivessel CAD was 87.4% and 73.0%. For detection of a 50% QCA stenosis, sensitivity was 64.6% and specificity was 86.6%. The optimal threshold for detecting CAD was a ≥67% QCA stenosis in GadaCAD1 and ≥63% QCA stenosis in GadaCAD2., Conclusions: Vasodilator stress and rest myocardial perfusion CMR and LGE imaging had high diagnostic accuracy for CAD in 2 phase 3 clinical trials. These findings supported the U.S. Food and Drug Administration approval of gadobutrol-enhanced CMR (0.1 mmol/kg) to assess myocardial perfusion and LGE in adult patients with known or suspected CAD., (Published by Elsevier Inc.)

Background: The T 1 Mapping and Extracellular volume (ECV) Standardization (T1MES) program explored T 1 mapping quality assurance using a purpose-developed phantom with Food and Drug Administration (FDA) and Conformité Européenne (CE) regulatory clearance. We report T 1 measurement repeatability across centers describing sequence, magnet, and vendor performance., Methods: Phantoms batch-manufactured in August 2015 underwent 2 years of structural imaging, B 0 and B 1 , and "reference" slow T 1 testing. Temperature dependency was evaluated by the United States National Institute of Standards and Technology and by the German Physikalisch-Technische Bundesanstalt. Center-specific T 1 mapping repeatability (maximum one scan per week to minimum one per quarter year) was assessed over mean 358 (maximum 1161) days on 34 1.5 T and 22 3 T magnets using multiple T 1 mapping sequences. Image and temperature data were analyzed semi-automatically. Repeatability of serial T 1 was evaluated in terms of coefficient of variation (CoV), and linear mixed models were constructed to study the interplay of some of the known sources of T 1 variation., Results: Over 2 years, phantom gel integrity remained intact (no rips/tears), B 0 and B 1 homogenous, and "reference" T 1 stable compared to baseline (% change at 1.5 T, 1.95 ± 1.39%; 3 T, 2.22 ± 1.44%). Per degrees Celsius, 1.5 T, T 1 (MOLLI 5s(3s)3s) increased by 11.4 ms in long native blood tubes and decreased by 1.2 ms in short post-contrast myocardium tubes. Agreement of estimated T 1 times with "reference" T 1 was similar across Siemens and Philips CMR systems at both field strengths (adjusted R 2 ranges for both field strengths, 0.99-1.00). Over 1 year, many 1.5 T and 3 T sequences/magnets were repeatable with mean CoVs < 1 and 2% respectively. Repeatability was narrower for 1.5 T over 3 T. Within T1MES repeatability for native T 1 was narrow for several sequences, for example, at 1.5 T, Siemens MOLLI 5s(3s)3s prototype number 448B (mean CoV = 0.27%) and Philips modified Look-Locker inversion recovery (MOLLI) 3s(3s)5s (CoV 0.54%), and at 3 T, Philips MOLLI 3b(3s)5b (CoV 0.33%) and Siemens shortened MOLLI (ShMOLLI) prototype 780C (CoV 0.69%). After adjusting for temperature and field strength, it was found that the T 1 mapping sequence and scanner software version (both P < 0.001 at 1.5 T and 3 T), and to a lesser extent the scanner model (P = 0.011, 1.5 T only), had the greatest influence on T 1 across multiple centers., Conclusion: The T1MES CE/FDA approved phantom is a robust quality assurance device. In a multi-center setting, T 1 mapping had performance differences between field strengths, sequences, scanner software versions, and manufacturers. However, several specific combinations of field strength, sequence, and scanner are highly repeatable, and thus, have potential to provide standardized assessment of T 1 times for clinical use, although temperature correction is required for native T 1 tubes at least.

Background: Right ventricular (RV) failure is the main cause of mortality in pulmonary arterial hypertension (PAH). Exercise testing helps identify early RV maladaptation and systolic dysfunction and facilitates therapy. Myocardial strain has been shown to be more sensitive than ejection fraction (EF) in detecting subclinical ventricular contractile dysfunction. Chronic pressure overload in PAH had been associated with changes in left ventricular (LV) filling., Purpose: To compare biventricular strains and ventriculo-ventricular interaction in PAH and controls using cardiac magnetic resonance feature tracking (cMRI-FT) and to determine the reproducibility of strain analysis., Study Type: Prospective., Population: Nine PAH and nine control subjects., Field Strength/sequence: 1.5T MRI balanced steady state free precession., Assessment: RV and LV longitudinal strain (Ell RV and Ell LV ) were derived using the mid-axial images. Radial (Err LV ) and circumferential strain (Ecc LV ) were derived using the mid-ventricular short-axis images. Relationships between strain and volumetric parameters were assessed at rest and during submaximal in-magnet exercise., Statistical Tests: Comparison of rest-to-exercise data between PAH and controls was analyzed using two-way repeated measures analysis of variance. The relationship between volumetric parameters and cMRI-FT were assessed using Pearson's correlation. Reproducibility was assessed by using Bland-Altman plots., Results: PAH had significantly lower Ell RV at rest (-16.6 ± 2.7 vs. -20.1 ± 3.6, P = 0.03) despite normal RVEF. During exercise, RV systolic contractile reserve measured by Ell RV was significantly reduced in PAH (P Interaction  = 0.02). In PAH, indexed RV end-systolic volume (ESVi) significantly correlated with Ecc LV and Err LV at rest (r = -0.65 and r = -0.70, P < 0.05) and with Err LV during exercise (r = -0.43, P < 0.05). High observer agreement was demonstrated., Data Conclusion: Despite normal resting RVEF, RV systolic function and contractile reserve as measured by Ell RV was significantly reduced in PAH. The close relation between RVESVi with Ecc LV and Err LV provides evidence of systolic ventriculo-ventricular interaction in PAH. Exercise cMRI-FT may provide a quantitative metric for detection of subclinical RV dysfunction in PAH., Level of Evidence: 1 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2019;49:1427-1436., (© 2018 International Society for Magnetic Resonance in Medicine.)

Background: The clinical use of cardiac magnetic resonance (CMR) in Australian heart failure (HF) patients has limited evidence., Aim: To examine how CMR, compared with routine echocardiography, affects the diagnosis and management of patients with HF., Methods: Single-centre retrospective study of HF patients newly referred to the Advanced Heart Failure and Transplant unit at The Prince Charles Hospital, Brisbane. Between January 2010 and December 2014, all patients clinically referred for both CMR and echocardiography in the workup of HF were analysed. Imaging results, electronic records and medical charts were reviewed for final diagnosis and changes in clinical management., Results: A total of 114 new HF referrals was included. Evaluation of HF of uncertain aetiology (70%) was the most common indication for CMR. In 20% of cases, CMR led to a completely new diagnosis or diagnostic confirmation of suspicions raised by echocardiography. Clinical decision-making was altered in 48%, with the greatest impact on decisions regarding revascularisation. Overall, CMR had a significant impact on 50% of patients. In a multivariable model, the only independent variable significantly associated with clinical impact was the presence/absence of late gadolinium enhancement (P < 0.001). Importantly, body mass index, echocardiography image quality and the presence of sinus rhythm did not show statistical significance in the multivariate analysis., Conclusion: CMR makes a substantial contribution to both the diagnosis and management of HF patients over and above standard echocardiography. This study provides evidence of its clinical utility in the HF population in Australia and supports the role of CMR in the routine assessment of this cohort., (© 2018 Royal Australasian College of Physicians.)

Introduction: The purpose of this study was to obtain a range of normal measurements of the adult thoracic aorta and main pulmonary artery using cardiac MRI, and to assess agreement between measurements made on ECG-gated two-dimensional (2D) breath held steady-state-free precession (SSFP), and three-dimensional (3D) breath held SSFP image acquisitions., Methods: Forty-nine normal volunteers underwent cardiac MRI using a 1.5T system. Two independent examiners measured the ascending aorta, aortic arch, descending thoracic aorta and main pulmonary artery in pre-defined locations., Results: Overall, inter-observer agreement for all measurements was excellent. Close agreement was observed in aortic diameters obtained from the 2D and 3D SSFP methods in six of the nine aortic measurement sites. There was a tendency for the 3D measurements to be smaller than the 2D measurements but this was only significant at two sites, the aortic annulus, and the ascending aorta. There was a significance difference in aortic measurements between the left carotid artery (LC) and the left subclavian artery (LSC)., Conclusion: Normal values for transverse diameters of the thoracic aorta and main pulmonary artery were established using 2D and 3D non-contrast MR sequences in healthy adults. Overall both inter-observer agreement, and agreement between 2D and 3D techniques was good. Mean diameter differences demonstrated at the aortic annulus, ascending aorta and aortic arch between LC and LSC although significant were less than one millimetre and unlikely to be important in clinical practice., (© 2017 The Royal Australian and New Zealand College of Radiologists.)

In subjects with aortic regurgitation (AR) or mitral regurgitation (MR), transthoracic echocardiography (TTE) is recommended for surveillance. Few prospective studies have directly compared the ability of TTE and cardiac magnetic resonance (CMR) to predict clinical outcomes in AR and MR. We hypothesized that, given its higher reproducibility, CMR would predict the need for valve surgery or heart failure (HF) hospitalization better than TTE. Quantitative TTE and CMR were performed on the same day for 51 subjects: 29 with chronic AR and 22 with chronic, primary MR for quantification of valve regurgitation. Baseline measurements of valve regurgitation were compared to the combined primary end point of new HF and valve surgery using receiver operating characteristics, simple logistic regression, and Kaplan-Meier survival analyses. The primary end point occurred in 5 AR subjects (all surgery) and 8 MR subjects (7 surgery, 1 HF) after a mean follow-up of 4.4 ± 1.5 years. For AR, CMR-derived regurgitant volume >50 ml identified those at high risk with 50% undergoing valve surgery versus 0% for those with regurgitant volume ≤50 ml and was more strongly associated with outcomes than regurgitant volume by TTE (p <0.05). For MR, 6.8% of those with regurgitant volume by TTE ≤30 ml developed the primary end point versus 70% in those with regurgitant volume >30 ml. Regurgitant volume by CMR showed no significant separation of survival curves for MR. In conclusion, regurgitant volume by CMR was more predictive of outcomes than by TTE in subjects with AR. In MR, the 2 methods performed similarly., (Copyright © 2017 Elsevier Inc. All rights reserved.)

In this review discuss the application of cardiac magnetic resonance (CMR) to the evaluation and quantification of mitral regurgitation and provide a systematic literature review for comparisons with echocardiography. Using the 2015 Preferred Reporting Items for Systematic Reviews and Meta-Analyses methodology, we searched Medline and PubMed for original research articles published since 2000 that provided data on the quantification of mitral regurgitation by CMR. We identified 220 articles of which 33 were included. Four main techniques of mitral regurgitation quantification were identified. Reproducibility varied substantially between papers but was high overall for all techniques. However, quantification differed between the techniques studied. When compared with two-dimensional echocardiography, mitral regurgitation fraction and regurgitant volume measured by CMR were comparable but typically lower. CMR has high reproducibility for the quantification of mitral regurgitation in experienced centres, but further technological refinement is needed. An integrated and standardised approach that combines multiple techniques is recommended for optimal reproducibility and precise mitral regurgitation quantification. Definitive outcome studies using CMR as a basis for treatment are lacking but needed., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Exclusion of ischemia is important in patients with newly diagnosed systolic heart failure (HF). We prospectively compared standard-of-care invasive catheter angiography (iCA) and echocardiography to a novel non-invasive strategy of both Coronary Computed Tomographic Angiography (CCTA) and Cardiovascular MRI (CMR) to determine the etiology of myocardial dysfunction Prospective data were collected from consecutive patients referred for iCA to investigate echocardiographically-confirmed new onset HF. CMR (1.5T GE) and dual source CCTA were performed within 2-7 days of iCA. Results were blinded and separately analyzed by expert readers. 426 coronary segments from 28 prospectively enrolled patients were analyzed by CCTA and quantitative iCA. The per-patient sensitivity and specificity of CCTA was 100% and 90%, respectively, negative predictive value (NPV) 100%, positive predictive value (PPV) 78%. Mean ejection fraction by CMR was 24%. Presence of ischemic-type LGE on CMR conferred a 67% sensitivity, 100% specificity, 90% NPV and 100% PPV. Combining CCTA with CMR conferred 100% specificity, 100% sensitivity, 100% PPV and 100% NPV for detection or exclusion of coronary disease. In patients with negative CCTA all invasive angiograms could have been avoided. In addition, two patients with no ischemic LGE by CMR had severe coronary disease on both CCTA and iCA, indicating global hibernation. This is a noteworthy finding in contrast to previous reports which suggested that absence of LGE rules out significant CAD. CCTA with CMR in newly-diagnosed HF enables non-invasive assessment of coronary artery disease, the severity and etiology of myocardial dysfunction and defines suitability for revascularization. Absence of ischemic-type LGE at CMR does not exclude CAD as a cause of LV dysfunction. A first-line strategy of functional and anatomic imaging with CMR and CCTA appears appropriate in newly diagnosed HF.

Introduction: Assessment of late gadolinium enhancement (LGE) at cardiac magnetic resonance is often used to detect scar in patients with arrhythmias of right ventricular (RV) origin. Recently, electroanatomic mapping (EAM) has been shown to reliably detect scars corresponding to different cardiomyopathic substrates. We compared LGE with EAM for the detection of scar in patients with arrhythmias of RV origin., Methods and Results: Thirty-one patients with RV arrhythmias and biopsy-proven structural heart disease (18 ARVC and 13 myocarditis), and 5 with idiopathic RV outflow tract arrhythmias underwent LGE analysis and EAM with scar validation through EAM-guided endomyocardial biopsy. EAM scars were present in 23 (64%) patients (all with structural heart disease), whereas LGE was present only in 12 (33%). In 2 cases, EAM provided a false-positive diagnosis of a small scar in the basal perivalvular area. LGE correctly diagnosed EAM scar in 48% of patients, resulting in high positive (92%) but low negative (50%) predictive values. The distribution of LGE was significantly associated with the distribution of EAM scars (P < 0.001 in the free wall, P = 0.003 in the outflow tract, and P = 0.023 in the posterior/inferior wall). Presence of LGE reflected a higher extension of EAM scars (34.4 ± 16.5% vs 7.9 ± 10.1% of the RV area, P < 0.001). At receiver operating characteristic (ROC) analysis, an extension of scar ≥20% of the RV area was the best cut-off value to detect LGE (sensitivity 83%, specificity 92%). Of note, LGE missed 10 of 11 (91%) patients with EAM scars <20% of RV area., Conclusions: LGE is significantly less sensitive than EAM in identifying RV cardiomyopathic substrates. Absence of LGE does not rule out the presence of small scars, and EAM with biopsy should be considered to increase the diagnostic yield., (© 2011 Wiley Periodicals, Inc.)

Background: In ST-elevation myocardial infarction (STEMI) patients, the main stimuli involved in endothelial progenitor cells (EPCs) mobilization are not fully understood. We aimed to assess by cardiac magnetic resonance (CMR) whether the extent of ischemic myocardium (area at risk (AAR)) or of necrotic myocardium (infarct size (IS)) can be correlated to levels of circulating EPCs., Methods: Peripheral EPCs were measured in fifteen STEMI patients at 24h after successful primary percutaneous coronary intervention (pPCI). Between two and four days after pPCI all patients underwent CMR assessment of myocardial AAR, IS, myocardial salvage (MS) and microvascular obstruction at late gadolinium enhancement CMR (LG-MVO)., Results: CD34+/KDR+, CD34+/KDR+/CD45dim, CD34+/KDR+/CD45-, EPCs were related to extent of AAR (rho=0.51, p=0.05; rho=0.55, p=0.03; rho=0.72, p=0.002, respectively), while no relationships were detected with IS, MS or LG-MVO., Conclusions: Our data show that EPCs were strongly correlated to extent of myocardial AAR, thus suggesting that progenitor cells mobilization in STEMI develops in response to myocardial ischemia and not to myocardial necrosis., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Background: Gated Blood Pool Imaging (GBPI) has low inter-test variability compared to echocardiography, and is often used when accurate repeated measurements of left ventricular ejection fraction are required such as in the surveillance of post-transplant patients. The aim of this study was to determine if cardiac MRI (CMR) could replace Gated Blood Pool Imaging for annual assessment of left ventricular (LV) function in the surveillance of patients post-cardiac transplantation., Methods: Forty-nine patients at various stages of post-cardiac transplantation presenting for annual review underwent LV functional assessment with GBPI and CMR. LV ejection fractions (EF) obtained from the two methods were compared and limits of agreement determined., Results: The mean EF (plus and minus two standard deviations) was 58.3+/-18% for GBPI and 57.6+/-18% for CMR. The two methods were found to provide clinically equivalent results., Conclusions: Assessment of LVEF with CMR offers comparable values to GBPI in post-heart transplant patients. However, CMR also allows quantitative volumetric analysis of ventricular volumes and assessment of wall motion and valvular function. As is now accepted in native heart assessment, CMR should be considered the "gold standard" for post-transplant volumetric and functional evaluation. CMR offers information over-and-above traditional imaging modalities., (Crown Copyright (c) 2010. Published by Elsevier B.V. All rights reserved.)

This vignette illustrates a classic case of congenital mitral stenosis (Ruckman-VanPraagh type 1) presenting in adulthood and demonstrates the benefits of multimodality cardiac imaging prior to successful surgical intervention.

Stress cardiovascular magnetic resonance (CMR) is an emerging non-invasive imaging technique for the assessment of known or suspected ischaemic heart disease (IHD). Stress CMR provides information on myocardial perfusion, wall motion, ventricular dimensions and volumes, as well as late gadolinium enhancement (LGE) scar imaging in a single test without ionising radiation. Data from numerous multicentre randomised studies show high diagnostic and prognostic utility, its efficacy as a gatekeeper to invasive coronary angiography and use for guiding coronary revascularisation decisions. Stress CMR is cost-effective across multiple healthcare settings, yet its uptake and usage varies worldwide and is an underutilised technology. New developments include rapid acquisition protocols, automated quantification of perfusion and myocardial blood flow, and artificial intelligence-aided automated analysis and reporting. Stress CMR is becoming more accessible and standardised around the globe and is ready for 'prime time' use in the non-invasive assessment of patients with suspected IHD. [ABSTRACT FROM AUTHOR]

Objective: Ultra-high-field B0 ≥ 7 tesla (7T) cardiovascular magnetic resonance (CMR) offers increased resolution. However, electrocardiogram (ECG) gating is impacted by the magnetohydrodynamic effect distorting the ECG trace. We explored the technical feasibility of a 7T magnetic resonance scanner using an ECG trigger learning algorithm to quantitatively assess cardiac volumes and vascular flow. Methods: 7T scans were performed on 10 healthy volunteers on a whole-body research MRI MR scanner (Siemens Healthineers, Erlangen, Germany) with 8 channel Tx/32 channels Rx cardiac coils (MRI Tools GmbH, Berlin, Germany). Vectorcardiogram ECG was performed using a learning phase outside of the magnetic field, with a trigger algorithm overcoming severe ECG signal distortions. Vectorcardiograms were quantitatively analyzed for false negative and false positive events. Cine CMR was performed after 3rd-order B0 shimming using a high-resolution breath-held ECG-retro-gated segmented spoiled gradient echo, and 2D phase contrast flow imaging. Artefacts were assessed using a semi-quantitative scale. Results: 7T CMR scans were acquired in all patients (100%) using the vectorcardiogram learning method. 3,142 R-waves were quantitatively analyzed, yielding sensitivity of 97.6% and specificity of 98.7%. Mean image quality score was 0.9, sufficient to quantitate both cardiac volumes, ejection fraction, and aortic and pulmonary blood flow. Mean left ventricular ejection fraction was 56.4%, right ventricular ejection fraction was 51.4%. Conclusion: Reliable cardiac ECG triggering is feasible in healthy volunteers at 7T utilizing a state-of-the-art three-lead trigger device despite signal distortion from the magnetohydrodynamic effect. This provides sufficient image quality for quantitative analysis. Other ultra-high-field imaging applications such as human brain functional MRI with physiologic noise correction may benefit from this method of ECG triggering. [ABSTRACT FROM AUTHOR]

This review summaries the utility, application and data supporting use of cardiac magnetic resonance imaging (CMR) to evaluate and quantitate aortic regurgitation. We systematically searched Medline and PubMed for original research articles published since 2000 that provided data on the quantitation of aortic regurgitation by CMR and identified 11 articles for review. Direct aortic measurements using phase contrast allow quantitation of volumetric flow across the aortic valve and are highly reproducible and accurate compared with echocardiography. However, this technique requires diligence in prescribing the correct imaging planes in the aorta. Volumetric analytic techniques using differences in ventricular volumes are also highly accurate but less than phase contrast techniques and only accurate when concomitant valvular disease is absent. Comparison of both aortic and ventricular data for internal data verification ensures fidelity of aortic regurgitant data. CMR data can be applied to many types of aortic valve regurgitation including combined aortic stenosis with regurgitation, congenital valve diseases and post-transcatheter valve placement. CMR also predicts those patients who progress to surgery with high overall sensitivity and specificity. Future studies of CMR in patients with aortic regurgitation to quantify the incremental benefit over echocardiography as well as prediction of cardiovascular events are warranted. [ABSTRACT FROM AUTHOR]

Cardiac imaging is a rapidly evolving field, with improvements in the diagnostic capabilities of non-invasive cardiac assessment. We review the two main emerging technologies in cardiac imaging: computed tomography coronary angiography (CTCA) to evaluate chest symptoms and to exclude coronary artery disease; and cardiovascular magnetic resonance (CMR) for evaluating cardiac morphology, function and presence of scar. CTCA is an excellent "rule out" test, with a negative predictive value approaching 100%. Radiation exposure is no longer a concern for CTCA, with doses routinely < 5 mSv, and as low as < 1 mSv in selected patients. CTCA is useful for excluding coronary artery disease, investigating the anatomy of coronary anomalies or fistulae, and for the patency of coronary bypass grafts. CMR is the reference test for the accurate quantitation of left ventricular and right ventricular size and function. CMR has no ionising radiation, making it particularly suitable for patients with heart failure or congenital heart disease who require longitudinal follow-up. Evaluation of cardiomyopathies (hypertrophic, ischaemic, infiltrative, myocarditis, iron overload or idiopathic) is a unique strength of CMR. Stress perfusion CMR has a strong evidence base and improved spatial and temporal resolution compared with nuclear single-photon emission computed tomography. [ABSTRACT FROM AUTHOR]

Objective: Cardiac magnetic resonance (CMR) has the potential to provide early detection of cardiac involvement in Fabry disease. We aimed to gain further insight into this by assessing a cohort of Fabry patients using CMR.Methods/results: Fifty genotype-positive Fabry subjects (age 45±2 years; 50% male) referred for CMR and 39 matched controls (age 40±2 years; 59% male) were recruited. Patients had a mean Mainz severity score index of 15±2 (range 0-46), reflecting an overall mild degree of disease severity. Compared with controls, Fabry subjects had a 34% greater left ventricular mass (LVM) index (82±5 vs 61±2 g/m(2), p=0.001) and had a significantly greater papillary muscle contribution to total LVM (13±1 vs 6±0.5%, p<0.001), even in the absence of left ventricular hypertrophy (LVH). Late gadolinium enhancement (LGE) was present in 15 Fabry subjects (9/21 males and 6/23 females). The most common site for LGE was the basal inferolateral wall (93%, 14/15). There was a positive association between LVM index and LGE. Despite this, there were two males and three females with no LVH that displayed LGE. Of Fabry subjects who were not on enzyme replacement therapy at enrolment (n=28), six were reclassified as having cardiac involvement (four LVH-negative/LGE-positive, one LVH-positive/LGE-positive and one LVH-positive/LGE-negative).Conclusions: CMR was able to detect cardiac involvement in 48% of this Fabry cohort, despite the overall mild disease phenotype of the cohort. Of those not on ERT, 21% were reclassified as having cardiac involvement allowing improved risk stratification and targeting of therapy. [ABSTRACT FROM AUTHOR]

Background: Sphingolipid deposition in Fabry disease causes left ventricular (LV) hypertrophy, of which the accurate assessment is essential. Cardiovascular magnetic resonance (CMR) has been proposed as the gold standard. However, there is debate in the literature as to whether papillary muscles and trabeculations (P&T) should be included in LV mass (LVM). Methods/results: We examined the accuracy of 2 CMR methods of assessing LVM and LV volumes, including (MincP&T) or excluding (MexP&T) P&T, in a cohort of Fabry disease subjects (n = 20) compared to a matched control group (n = 20). Significant differences between the two measurement methods were observed for LV end-diastolic volume, LV end-systolic volume, LVM, and LV ejection fraction (LVEF) in both groups. These differences were significantly greater in the Fabry group compared to controls, except for LVEF. P&T contributed to a greater percentage of LVM in Fabry subjects than controls (20 ± 1% vs 13 ± 2%, p = 0.01). In the control group, both volume-derived methods (MincP&T or MexP&T) provided accurate SV measurements compared with the internal reference of velocity-encoded aortic flow. In the Fabry group, inclusion of P&T (MincP&T) resulted in good concordance with phase contrast flow imaging (difference between flow and volume techniques: 1 ± 3 ml, p = 0.7). Conclusion: The volumetric contribution of P&T in Fabry disease is markedly increased relative to healthy controls. Failure to account for this results in significant underestimation of LVM and results in misclassification of a proportion of subjects. [ABSTRACT FROM AUTHOR]

The article discusses a study which hypothesized that cardiac magnetic resonance (CMR) imaging of regurgitant volume (RVol) is more reproducible than transthoracic echocardiography (TTE). Patients with chronic aortic regurgitation (AR) or mitral regurgitation (MR) underwent TTE and CMR. The results indicate that CMR may be superior for serial measurements due to its lower intraobserver and interobserver variabilities for RVol.

GadaCAD2 was 1 of 2 international, multicenter, prospective, Phase 3 clinical trials that led to U.S. Food and Drug Administration approval of gadobutrol to assess myocardial perfusion and late gadolinium enhancement (LGE) in adults with known or suspected coronary artery disease (CAD). A prespecified secondary objective was to determine if stress perfusion cardiovascular magnetic resonance (CMR) was noninferior to single-photon emission computed tomography (SPECT) for detecting significant CAD and for excluding significant CAD. Participants with known or suspected CAD underwent a research rest and stress perfusion CMR that was compared with a gated SPECT performed using standard clinical protocols. For CMR, adenosine or regadenoson served as vasodilators. The total dose of gadobutrol was 0.1 mmol/kg body weight. The standard of reference was a 70% stenosis defined by quantitative coronary angiography (QCA). A negative coronary computed tomography angiography could exclude CAD. Analysis was per patient. CMR, SPECT, and QCA were evaluated by independent central core lab readers blinded to clinical information. Participants were predominantly male (61.4% male; mean age 58.9 ± 10.2 years) and were recruited from the United States (75.0%), Australia (14.7%), Singapore (5.7%), and Canada (4.6%). The prevalence of significant CAD was 24.5% (n = 72 of 294). Stress perfusion CMR was statistically superior to gated SPECT for specificity (P = 0.002), area under the receiver operating characteristic curve (P < 0.001), accuracy (P = 0.003), positive predictive value (P < 0.001), and negative predictive value (P = 0.041). The sensitivity of CMR for a 70% QCA stenosis was noninferior and nonsuperior to gated SPECT. Vasodilator stress perfusion CMR, as performed with gadobutrol 0.1 mmol/kg body weight, had superior diagnostic accuracy for diagnosis and exclusion of significant CAD vs gated SPECT. [Display omitted] [ABSTRACT FROM AUTHOR]

An abstract of the paper "Quantitative Blush Evaluator (QuBE) Accurately Quantifies Microvascular Dysfunction in Patients With ST-Elevation Myocardial Infarction; Comparison With Cardiovascular Magnetic Resonance," by Italo Porto and colleagues is presented.

Coronary artery disease (CAD) remains the leading cause of adult mortality globally. Targeting known modifiable risk factors has had substantial benefit, but there remains a need for new approaches. Improvements in invasive and noninvasive imaging techniques have enabled an increasing recognition of distinct quantitative phenotypes of coronary atherosclerosis that are prognostically relevant. There are marked differences in plaque phenotype, from the high-risk, lipid-rich, thin-capped atheroma to the low-risk, quiescent, eccentric, nonobstructive calcified plaque. Such distinct phenotypes reflect different pathophysiologic pathways and are associated with different risks for acute ischemic events. Noninvasive coronary imaging techniques, such as computed tomography, positron emission tomography, and coronary magnetic resonance imaging, have major potential to accelerate cardiovascular drug development, which has been affected by the high costs and protracted timelines of cardiovascular outcome trials. This may be achieved through enrichment of high-risk phenotypes with higher event rates or as primary end points of drug efficacy, at least in phase 2 trials, in a manner historically performed through intravascular coronary imaging studies. Herein, we provide a comprehensive review of the current technology available and its application in clinical trials, including implications for sample size requirements, as well as potential limitations. In its effort to accelerate drug development, the US Food and Drug Administration has approved surrogate end points for 120 conditions, but not for CAD. There are robust data showing the beneficial effects of drugs, including statins, on CAD progression and plaque stabilization in a manner that correlates with established clinical end points of mortality and major adverse cardiovascular events. This, together with a clear mechanistic rationale for using imaging as a surrogate CAD end point, makes it timely for CAD imaging end points to be considered. We discuss the importance of global consensus on these imaging end points and protocols and partnership with regulatory bodies to build a more informed, sustainable staged pathway for novel therapies. [ABSTRACT FROM AUTHOR]

Background: Coronary artery disease (CAD) remains a major public health problem in Australia and globally. A variety of imaging techniques allow for both anatomical and functional assessment of CAD and selection of the optimal investigation pathway is challenging. Cardiovascular magnetic resonance (CMR) is not widely used in Australia, partly due to perceived cost and lack of Federal Government reimbursement compared to the alternative techniques. The aim of this study was to estimate the cost-effectiveness of different diagnostic strategies in identifying significant CAD in patients with chest pain suggestive of angina using the evidence gathered in the Clinical Evaluation of Magnetic Resonance Imaging in Coronary Heart Disease 2 (CE-MARC trial), analysed from the Australian health care perspective.Methods: A decision analytic model coupled with three distinct Markov models allowed eight potential clinical investigation strategies to be considered; combinations of exercise electrocardiogram stress testing (EST), single-photon emission computed tomography (SPECT), stress CMR, and invasive coronary angiography (ICA). Costs were from the Australian health care system in Australian dollars, and outcomes were measured in terms of quality-adjusted life-years. Parameter estimates were derived from the CE-MARC and EUropean trial on Reduction Of cardiac events with Perindopril in patients with stable coronary Artery disease (EUROPA) trials, and from reviews of the published literature.Results: The most cost-effective diagnostic strategy, based on a cost-effectiveness threshold of $45,000 to $75,000 per QALY gained, was EST, followed by stress CMR if the EST was positive or inconclusive, followed by ICA if the stress CMR was positive or inconclusive; this held true in the base case and the majority of scenario analyses.Conclusions: This economic evaluation shows that an investigative strategy of stress CMR if EST is inconclusive or positive is the most cost-effective approach for diagnosing significant coronary disease in chest pain patients within the Australian health care system. [ABSTRACT FROM AUTHOR]