Cardiovascular magnetic resonance in autoimmune rheumatic diseases: a clinical consensus document by the European Association of Cardiovascular Imaging.

Autoimmune rheumatic diseases (ARDs) involve multiple organs including the heart and vasculature. Despite novel treatments, patients with ARDs still experience a reduced life expectancy, partly caused by the higher prevalence of cardiovascular disease (CVD). This includes CV inflammation, rhythm disturbances, perfusion abnormalities (ischaemia/infarction), dysregulation of vasoreactivity, myocardial fibrosis, coagulation abnormalities, pulmonary hypertension, valvular disease, and side-effects of immunomodulatory therapy. Currently, the evaluation of CV involvement in patients with ARDs is based on the assessment of cardiac symptoms, coupled with electrocardiography, blood testing, and echocardiography. However, CVD may not become overt until late in the course of the disease, thus potentially limiting the therapeutic window for intervention. More recently, cardiovascular magnetic resonance (CMR) has allowed for the early identification of pathophysiologic structural/functional alterations that take place before the onset of clinically overt CVD. CMR allows for detailed evaluation of biventricular function together with tissue characterization of vessels/myocardium in the same examination, yielding a reliable assessment of disease activity that might not be mirrored by blood biomarkers and other imaging modalities. Therefore, CMR provides diagnostic information that enables timely clinical decision-making and facilitates the tailoring of treatment to individual patients. Here we review the role of CMR in the early and accurate diagnosis of CVD in patients with ARDs compared with other non-invasive imaging modalities. Furthermore, we present a consensus-based decision algorithm for when a CMR study could be considered in patients with ARDs, together with a standardized study protocol. Lastly, we discuss the clinical implications of findings from a CMR examination.

Churg-Strauss syndrome with cardiac involvement: case illustration and contribution of CMR in the diagnosis and clinical follow-up.

This report summarises three cases of Churg-Strauss syndrome (CSS) illustrating the diagnostic challenges associated with the cardiac manifestation of this disease. Here, we illustrate the role of cardiac magnetic resonance (CMR) for diagnosis and follow-up of CSS with a focus on new non-contrast T2-weighted imaging sequences for quantification of myocardial scar tissue and quantitative T2 mapping techniques, which allow the detection of myocardial edema.

2015 Update on Acute Adverse Reactions to Gadolinium based Contrast Agents in Cardiovascular MR. Large Multi-National and Multi-Ethnical Population Experience With 37788 Patients From the EuroCMR Registry.

OBJECTIVES: Specifically we aim to demonstrate that the results of our earlier safety data hold true in this much larger multi-national and multi-ethnical population. BACKGROUND: We sought to re-evaluate the frequency, manifestations, and severity of acute adverse reactions associated with administration of several gadolinium- based contrast agents during routine CMR on a European level. METHODS: Multi-centre, multi-national, and multi-ethnical registry with consecutive enrolment of patients in 57 European centres. RESULTS: During the current observation 37,788 doses of Gadolinium based contrast agent were administered to 37,788 patients. The mean dose was 24.7 ml (range 5-80 ml), which is equivalent to 0.123 mmol/kg (range 0.01 - 0.3 mmol/kg). Forty-five acute adverse reactions due to contrast administration occurred (0.12%). Most reactions were classified as mild (43 of 45) according to the American College of Radiology definition. The most frequent complaints following contrast administration were rashes and hives (15 of 45), followed by nausea (10 of 45) and flushes (10 of 45). The event rate ranged from 0.05% (linear non-ionic agent gadodiamide) to 0.42% (linear ionic agent gadobenate dimeglumine). Interestingly, we also found different event rates between the three main indications for CMR ranging from 0.05% (risk stratification in suspected CAD) to 0.22% (viability in known CAD). CONCLUSIONS: The current data indicate that the results of the earlier safety data hold true in this much larger multi-national and multi-ethnical population. Thus, the "off-label" use of Gadolinium based contrast in cardiovascular MR should be regarded as safe concerning the frequency, manifestation and severity of acute events.

Indications for cardiovascular magnetic resonance in children with congenital and acquired heart disease: an expert consensus paper of the Imaging Working Group of the AEPC and the Cardiovascular Magnetic Resonance Section of the EACVI.

This article provides expert opinion on the use of cardiovascular magnetic resonance (CMR) in young patients with congenital heart disease (CHD) and in specific clinical situations. As peculiar challenges apply to imaging children, paediatric aspects are repeatedly discussed. The first section of the paper addresses settings and techniques, including the basic sequences used in paediatric CMR, safety, and sedation. In the second section, the indication, application, and clinical relevance of CMR in the most frequent CHD are discussed in detail. In the current era of multimodality imaging, the strengths of CMR are compared with other imaging modalities. At the end of each chapter, a brief summary with expert consensus key points is provided. The recommendations provided are strongly clinically oriented. The paper addresses not only imagers performing CMR, but also clinical cardiologists who want to know which information can be obtained by CMR and how to integrate it in clinical decision-making.

Indications for cardiovascular magnetic resonance in children with congenital and acquired heart disease: an expert consensus paper of the Imaging Working Group of the AEPC and the Cardiovascular Magnetic Resonance Section of the EACVI.

This article provides expert opinion on the use of cardiovascular magnetic resonance (CMR) in young patients with congenital heart disease (CHD) and in specific clinical situations. As peculiar challenges apply to imaging children, paediatric aspects are repeatedly discussed. The first section of the paper addresses settings and techniques, including the basic sequences used in paediatric CMR, safety, and sedation. In the second section, the indication, application, and clinical relevance of CMR in the most frequent CHD are discussed in detail. In the current era of multimodality imaging, the strengths of CMR are compared with other imaging modalities. At the end of each chapter, a brief summary with expert consensus key points is provided. The recommendations provided are strongly clinically oriented. The paper addresses not only imagers performing CMR, but also clinical cardiologists who want to know which information can be obtained by CMR and how to integrate it in clinical decision-making.

Quantification of aortic flow by phase-contrast magnetic resonance in patients with bicuspid aortic valve.

AIMS: Bicuspid aortic valve (BAV) causes complex flow patterns in the ascending aorta (AAo), which may compromise the accuracy of flow measurement by phase-contrast magnetic resonance (PC-MR). Therefore, we aimed to assess and compare the accuracy of forward flow measurement in the AAo, where complex flow is more dominant in BAV patients, with flow quantification in the left ventricular outflow tract (LVOT) and the aortic valve orifice (AV), where complex flow is less important, in BAV patients and controls. METHODS AND RESULTS: Flow was measured by PC-MR in 22 BAV patients and 20 controls at the following positions: (i) LVOT, (ii) AV, and (iii) AAo, and compared with the left ventricular stroke volume (LVSV). The correlation between the LVSV and the forward flow in the LVOT, the AV, and the AAo was good in BAV patients (r = 0.97/0.96/0.93; P < 0.01) and controls (r = 0.96/0.93/0.93; P < 0.01). However, in relation with the LVSV, the forward flow in the AAo was mildly underestimated in controls and much more in BAV patients [median (inter-quartile range): 9% (4%/15%) vs. 22% (8%/30%); P < 0.01]. This was not the case in the LVOT and the AV. The severity of flow underestimation in the AAo was associated with flow eccentricity. CONCLUSION: Flow measurement in the AAo leads to an underestimation of the forward flow in BAV patients. Measurement in the LVOT or the AV, where complex flow is less prominent, is an alternative means for quantifying the systolic forward flow in BAV patients.

[Cardiac MR in development: the large multicenter CMR studies in 2012]. / IRM cardiaque en évolution: de grandes études multicentriques en 2012.

The results of several large multicenter CMR studies were reported in 2012, thus, constantly corroborating the evidence on CMR performance. In this review, we present results of the MR-IMPACT programme and the CE-MARC study, which demonstrated the superiority of perfusion-CMR over gated SPECT for the workup of suspected CAD, the currently available data from the European CMR registry, comprising almost 30,000 patients from 57 participating centers in 15 European countries, and finally, the results of the Advisa-MRI study, which documented the safety of a MRI-compatible pacemaker system. These large trials and others set the basis for the recommendations in the new European guidelines on heart failure to use CMR as a first line method if echocardiographic quality is inadequate or the etiology of heart failure is unclear.

[Cardiology]. / Cardiologie.

The present review provides a selected choice of clinical research in the field of interventional cardiology, heart failure and cardiac imaging. We also focused on the new guidelines published by the European society of cardiology in 2012.

[Cardiac MRI in the follow-up of adult congenital cardiomyopathy patients]. / IRM cardiaque dans le suivi des cardiopathies congénitales à l'âge adulte.

Magnetic resonance imaging is a rapidly developing modality in cardiology. It offers an excellent image definition and a large field of view, allowing a more accurate morphological assessment of cardiac malformations. Due to its unique versatility and its ability to provide myocardial tissue characterization, cardiac magnetic resonance (CMR) is now recognized as a central imaging modality for a wide range of congenital heart diseases, including assessment of post-surgical cardiac anatomy, quantification of valvular disease and detection of myocardial ischemia. CMR provides useful diagnostic information without any radiation exposure, and improves the global management of patients with congenital heart disease.

Hypertrophic cardiomyopathy due to beta-myosin heavy chain mutation with extreme phenotypic variability within a family.

Hypertrophic cardiomyopathy (HCM) affects 1 in 500 persons and shows high variability in severity of disease, in genetic heterogeneity and phenotypic patterns. Many affected individuals remain undetected throughout their lives. In this case report a family with proven beta-myosin heavy chain mutation (MYH7) with 3 affected family members with huge phenotypic variability is described. The index patient (male, age 21 years) has severe phenotypic expression with a pathological ECG and maximal septal wall thickness of 29 mm, there is no significant obstruction in the left ventricular outflow tract. The sister (age 16 years), mutation carrier, has no detectable hypertrophy and no ECG changes. The mother (age 44 years), also carrying the mutation, has a normal ECG and shows only mild septal hypertrophy of 12 mm and systolic anterior motion of her mitral valve chordae with no gradient. The maternal grandmother died suddenly at age 65 years of presumed coronary artery disease, and the maternal great-grandmother had a sudden cardiac death at age 50 years of unknown etiology. To conclude, this family shows impressively the wide spectrum of phenotypic presentation and outcome in one family.

The future of cardiovascular imaging and non-invasive diagnosis: a joint statement from the European Association of Echocardiography, the Working Groups on Cardiovascular Magnetic Resonance, Computers in Cardiology, and Nuclear Cardiology, of the European Society of Cardiology, the European Association of Nuclear Medicine, and the Association for European Paediatric Cardiology.

Advances in medical imaging now make it possible to investigate any patient with cardiovascular disease using multiple methods which vary widely in their technical requirements, benefits, limitations, and costs. The appropriate use of alternative tests requires their integration into joint clinical diagnostic services where experts in all methods collaborate. This statement summarises the principles that should guide developments in cardiovascular diagnostic services.

Detection of coronary artery disease by magnetic resonance myocardial perfusion imaging with various contrast medium doses: first European multi-centre experience.

AIMS: Magnetic resonance (MR) first-pass myocardial perfusion imaging during hyperaemia detects coronary artery stenoses in humans with test sensitivity depending on contrast medium (CM)-induced signal change in myocardium. In this prospective multi-centre study, the effect of CM dose on myocardial signal change and on diagnostic performance was evaluated using a stress-only approach. METHODS AND RESULTS: Ninety-four patients with known or suspected coronary artery disease (CAD) were randomised to 0.05,0.10, or 0.15 mmol/kg body weight of an extravascular CM (Gd-DTPA) and X-ray coronary angiography was performed within 30 days prior/after the MR examination. A multi-slice MR technique with identical hardware and software in all centres was used during hyperaemia (adenosine 0.14 mg/kg/min) to monitor myocardial CM wash-in kinetics and data were analysed semi-automatically in a core laboratory. Protocol violations resulted in 80 complete studies with CAD (defined as > or =1 vessel with diameter stenosis > or =50% on quantitative coronary angiography) present in 19/29, 13/24, and 20/27 patients for doses 1, 2, and 3, respectively. In normal myocardium, the upslope increased with CM dose (overall-p<0.0001, ANOVA). For CAD detection the area under the receiver operator characteristics curve for subendocardial data (3 slices with quality score<4 representing 86% of cases) was 0.91+/-0.07 and 0.86+/-0.08 for doses 2 and 3, respectively, and was lower for dose 1 (0.53+/-0.13, p<0.01 and p<0.02 vs. doses 2 and 3, respectively). Corresponding sensitivities/specificities (95% confidence intervals) for pooled doses 2/3 were 93% (77-99%; ns vs. dose 1) and 75% (48-92%;p<0.05 vs. dose 1), respectively. CONCLUSIONS: With increasing doses of CM, a higher signal response in the myocardium was achieved and consequently this stress-only protocol, with CM doses of 0.10-0.15 mmol/kg combined with a semi-automatic analysis, yielded a high diagnostic performance for the detection of CAD.

Myocardial first-pass perfusion magnetic resonance imaging: a multicenter dose-ranging study.

BACKGROUND: MRI can identify patients with obstructive coronary artery disease by imaging the left ventricular myocardium during a first-pass contrast bolus in the presence and absence of pharmacologically induced myocardial hyperemia. The purpose of this multicenter dose-ranging study was to determine the minimally efficacious dose of gadopentetate dimeglumine injection (Magnevist Injection; Berlex Laboratories) for detecting obstructive coronary artery disease. METHOD AND RESULTS: A total of 99 patients scheduled for coronary artery catheterization as part of their clinical evaluation were enrolled in this study. Patients were randomized to 1 of 3 doses of gadopentate dimeglumine: 0.05, 0.10, or 0.15 mmol/kg. First-pass perfusion imaging was performed during hyperemia (induced by a 4-minute infusion of adenosine at a rate of 140 microg x kg(-1) x min(-1)) and then again in the absence of adenosine with otherwise identical imaging parameters and the same contrast dose. Perfusion defects were evaluated subjectively by 4 blinded reviewers. Receiver-operating curve analysis showed that the areas under the receiver-operating curve were 0.90, 0.72, and 0.83 for the low-, medium-, and high-contrast doses, respectively, compared with quantitative coronary angiography (diameter stenosis > or =70%). For the low-dose group, mean sensitivity was 93+/-0%, mean specificity was 75+/-7%, and mean accuracy was 85+/-3%. CONCLUSIONS: First-pass perfusion MRI is a safe and accurate test for identifying patients with obstructive coronary artery disease. A low dose of 0.05 mmol/kg gadopentetate dimeglumine is at least as efficacious as higher doses.

Integration of vascular biology and magnetic resonance imaging in the understanding of atherothrombosis and acute coronary syndromes.

The interaction between the vulnerable atherosclerotic plaque prone to disruption and thrombus formation is the cornerstone of acute coronary syndrome (ACS). Although distinct from one another, the atherosclerotic and thrombotic processes appear to be interdependent, hence the term atherothrombosis. Inflammation is a crucial common pathophysiological mechanism. Overall, the association of plaque vulnerability and ACS has been well documented. Given the multifactorial origin of atherothrombosis the best preventive approach should be aggressive management of all the risk factors. New interventions should be directed toward decreasing vulnerability of the lesions thereby decreasing the risk of ACS.

Magnetic resonance myocardial first-pass perfusion imaging: parameter optimization for signal response and cardiac coverage.

Fast imaging techniques allow monitoring of contrast medium (CM) first-pass kinetics in a multislice mode. Employing shorter recovery times improves cardiac coverage during first-pass conditions, but potentially flattens signal response in the myocardium. The aim of this study was therefore to compare in patients with suspected coronary artery disease (CAD) two echo-planar imaging strategies yielding either extended cardiac coverage or optimized myocardial signal response (protocol A/B, six/four slices; preparation pulse, 60 degrees /90 degrees; delay time, 10/120 msec; readout flip angle, 10 degrees /50 degrees; respectively). In phantoms and myocardium of normal volunteers (N= 10) the CM-induced signal increase was 2.5-3 times higher with protocol B (P < 0.005) than with protocol A. For the detection of individually diseased coronary arteries (> or =1 stenosis with > or =50% diameter reduction on quantitative coronary angiography (QCA)), receiver-operator characteristics of protocol B (signal upslope in 32 sectors/heart) yielded a sensitivity/specificity of 82%/73%, which was superior to protocol A (P < 0.05, N= 14). For the overall detection of CAD, the sensitivity/specificity of protocol B was 85%/81%. An adequate signal response in the myocardium is crucial for a reliable detection of perfusion deficits during first-pass conditions. The presented protocol B detects CAD with a sensitivity and specificity similar to scintigraphic techniques.

Effect of exercise training on postexercise oxygen uptake kinetics in patients with reduced ventricular function.

BACKGROUND: The time required for oxygen uptake (O(2)) to return to baseline level (recovery kinetics) is prolonged in patients with reduced ventricular function, and the degree to which it is prolonged is related to the severity of heart failure, markers of abnormal ventilation, and prognosis. In the present study, we sought to determine the effect of exercise training on O(2) recovery kinetics in patients with reduced ventricular function. METHODS: Twenty-four male patients with reduced ventricular function after a myocardial infarction were randomized to either a 2-month high-intensity residential exercise training program or to a control group. O(2) kinetics in recovery from maximal exercise were calculated before and after the study period and expressed as the slope of a single exponential relation between O(2) and time during the first 3 min of recovery. RESULTS: Peak O(2) increased significantly in the exercise group (19.4 +/- 3.0 mL/kg/min vs 25.1 +/- 4.7 mL/kg/min, p < 0.05), whereas no change was observed in control subjects. The O(2) half-time in recovery was reduced slightly after the study period in both groups (108.7 +/- 33.1 to 102.1 +/- 50.5 s in the exercise group and 122.3 +/- 68.7 to 107.5 +/- 36.0 s in the control group); neither the change within or between groups was significant. The degree to which O(2) was prolonged in recovery was inversely related to measures of exercise capacity (peak O(2), watts achieved, and exercise time; r = - 0.48 to - 0.57; p < 0.01) and directly related to the peak ventilatory equivalents for oxygen (r = 0.59, p < 0.01) and carbon dioxide (r = 0.57, p < 0.01). CONCLUSION: Two months of high-intensity training did not result in a faster recovery of O(2) in patients with reduced ventricular function. This suggests that adaptations to exercise training manifest themselves only during, but not in, recovery from exercise.

Aortic and mitral regurgitation: quantification using moving slice velocity mapping.

Comprehensive assessment of the severity of valvular insufficiency includes quantification of regurgitant volumes. Previous methods lack reliable slice positioning with respect to the valve and are prone to velocity offsets due to through-plane motion of the valvular plane of the heart. Recently, the moving slice velocity mapping technique was proposed. In this study, the technique was applied for quantification of mitral and aortic regurgitation. Time-efficient navigator-based respiratory artifact suppression was achieved by implementing a prospective k-space reordering scheme in conjunction with slice position correction. Twelve patients with aortic insufficiency and three patients with mitral insufficiency were studied. Aortic regurgitant volumes were calculated from diastolic velocities mapped with a moving slice 5 mm distal to the aortic valve annulus. Mitral regurgitant flow was indirectly assessed by measuring mitral inflow at the level of the mitral annulus and net aortic outflow. Regurgitant fractions, derived from velocity data corrected for through-plane motion, were compared to data without correction for through-plane motion. In patients with mild and moderate aortic regurgitation, regurgitant fractions differed by 60% and 15%, on average, when comparing corrected and uncorrected data, respectively. Differences in severe aortic regurgitation were less (7%). Due to the large orifice area of the mitral valve, differences were still substantial in moderate-to-severe mitral regurgitation (19%). The moving slice velocity mapping technique was successfully applied in patients with aortic and mitral regurgitation. The importance of correction for valvular through-plane motion is demonstrated.

Assessment of myocardial perfusion in coronary artery disease by magnetic resonance: a comparison with positron emission tomography and coronary angiography.

BACKGROUND: Monitoring contrast medium wash-in kinetics in hyperemic myocardium by magnetic resonance (MR) allows for the detection of stenosed coronary arteries. In this prospective study, the quality of a multislice MR approach with respect to the detection and sizing of compromised myocardium was determined and compared with positron emission tomography (PET) and quantitative coronary angiography. METHODS AND RESULTS: A total of 48 patients and healthy subjects were studied by MR using a multislice hybrid echo-planar pulse sequence for monitoring the myocardial first pass kinetics of gadolinium-diethylenetriamine pentaacetic acid bismethylamide (Omniscan; 0.1 mmol/kg injected at 3 mL/s IV) during hyperemia (dipyridamole 0.56 mg/kg). Signal intensity upslope as a measure of myocardial perfusion was calculated in 32 sectors per heart from pixelwise parametric maps in the subendocardial layer and for full wall thickness. Before coronary angiography, coronary flow reserve (hyperemia induced by dipyridamole 0.56 mg/kg) was determined in corresponding sectors by (13)N-ammonia PET. Receiver-operator characteristic analysis of subendocardial upslope data revealed a sensitivity and specificity of 91% and 94%, respectively, for the detection of coronary artery disease as defined by PET (mean coronary flow reserve minus 2SD of controls) and a sensitivity and specificity of 87% and 85%, respectively, in comparison with quantitative coronary angiography (diameter stenosis >/=50%). The number of pathological sectors per patient on PET and MR studies correlated linearly (slope, 0.94; r=0.76; P<0.0001). CONCLUSIONS: The presented MR approach reliably identifies patients with coronary artery stenoses and provides information on the amount of compromised myocardium, even when perfusion abnormalities are confined to the subendocardial layer. This modality may qualify for its clinical application in the management of coronary artery disease.

Valvular heart disease: assessment of valve morphology and quantification using MR.

For clinical evaluation and decision-making in patients with valvular heart disease, the diagnostic armamentarium expands steadily. This evolution makes it difficult to choose the most appropriate approach for a specific valvular lesion. It may also reflect our uncertainty of what are the findings that best predict clinical outcome of patients, e.g. after surgery. Accordingly, for each type of valvular lesion, some pathopysiological considerations are stated in order to derive the most important measures that would allow optimal guidance of patients. Based on these considerations the value of an MR study is discussed for each valvular lesion. Newest advances in MR technology allow for highly accurate measurements of regurgitant volumes and hence, MR may be the method of choice for a quantitative evaluation of regurgitant valves. For assessment of stenosis severity, measurement of transvalvular pressure gradient is an appropriate measure and MR may not confer benefits over echocardiography, provided the ultrasound window is adequate (and stroke volume is in the normal range). With respect to surgical treatment, valvular morphology is of pivotal importance, particularly for the mitral valve, and echocardiography still appears to be the first line method. Little data relate lesion severity and/or morphology to clinical outcome. Conversely, the extent of cardiac adaptation to pressure- and/or volume-overload, i.e. ventricular remodeling, is a strong predictor of outcome, and is therefore most important for final judgement of the patient. For assessment of left and right ventricular remodeling, echocardiography typically provides all the necessary information. However, in special cases with discrepant findings, with inadequate ultrasound window, or in the preoperative work-up, MR may provide important information regarding cardiac adaptation to valvular lesion.