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PRKAG2 cardiomyopathy is a rare genetic disorder that manifests early in life with an autosomal dominant inheritance pattern. It harbors left ventricular hypertrophy (LVH), ventricular pre-excitation and progressively worsening conduction system defects. Its estimated prevalence among patients with LVH ranges from 0.23 to about 1%, but it is likely an underdiagnosed condition. We report the association of the PRKAG2 missense variant c.1006G>A p. (Val336Ile) with LVH, conduction abnormalities (short PR interval and incomplete right bundle branch bock) and early-onset arterial hypertension (AH) in a 44-year-old Caucasian patient. While cardiac magnetic resonance (CMR) showed a mild hypertrophic phenotype with maximal wall thickness of 17 mm in absence of tissue alterations, the electric phenotype was relevant including brady–tachy syndrome and recurrent syncope. The same variant has been detected in the patient's sister and daughter, with LVH + early-onset AH and electrocardiographic (ECG) alterations + lipothymic episodes, respectively. Paying close attention to the coexistence of LVH and ECG alterations in the proband has been helpful in directing genetic tests to exclude primary cardiomyopathy. Hence, identifying the genetic basis in the patient allowed for familial screening as well as a proper follow-up and therapeutic management of the affected members. A review of the PRKAG2 cardiomyopathy literature is provided alongside the case report. [ABSTRACT FROM AUTHOR]

Diastolic vortex ring (VR) plays a key role in the blood-pumping function exerted by the left ventricle (LV), with altered VR structures being associated with LV dysfunction. Herein, we sought to characterize the VR diastolic alterations in ischemic cardiomyopathy (ICM) patients with systo-diastolic LV dysfunction, as compared to healthy controls, in order to provide a more comprehensive understanding of LV diastolic function. 4D Flow MRI data were acquired in ICM patients (n = 15) and healthy controls (n = 15). The λ2 method was used to extract VRs during early and late diastolic filling. Geometrical VR features, e.g., circularity index (CI), orientation (α), and inclination with respect to the LV outflow tract (ß), were extracted. Kinetic energy (KE), rate of viscous energy loss ( EL ˙ ), vorticity (W), and volume (V) were computed for each VR; the ratios with the respective quantities computed for the entire LV were derived. At peak E-wave, the VR was less circular (p = 0.032), formed a smaller α with the LV long-axis (p = 0.003) and a greater ß (p = 0.002) in ICM patients as compared to controls. At peak A-wave, CI was significantly increased (p = 0.034), while α was significantly smaller (p = 0.016) and β was significantly increased (p = 0.036) in ICM as compared to controls. At both peak E-wave and peak A-wave, EL ˙ VR / EL ˙ LV , WVR/WLV, and VVR/VLV significantly decreased in ICM patients vs. healthy controls. KEVR/VVR showed a significant decrease in ICM patients with respect to controls at peak E-wave, while VVR remained comparable between normal and pathologic conditions. In the analyzed ICM patients, the diastolic VRs showed alterations in terms of geometry and energetics. These derangements might be attributed to both structural and functional alterations affecting the infarcted wall region and the remote myocardium. [ABSTRACT FROM AUTHOR]

This article discusses the use of cardiac magnetic resonance (CMR) imaging with the late gadolinium enhancement (LGE) technique to assess the presence of a myocardial scar in patients with an anomalous right coronary artery originating from the left sinus of Valsalva (R-ACAOS). The study included 48 patients with R-ACAOS, and 21% of them showed an ischaemic pattern of LGE. These patients were older and had high-risk anatomical features. Although most patients were asymptomatic and had negative provocative tests, the presence of an ischaemic scar suggests a potential risk for sudden cardiac death. CMR may be a useful tool for risk stratification in R-ACAOS patients. [Extracted from the article]

Background: Cardiac involvement in systemic sclerosis (SSc) affects the prognosis of the disease. Echocardiography is the first line imaging tool to detect cardiac involvement, but it is not able to routinely detect myocardial fibrosis. Late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) is the gold standard for replacement myocardial fibrosis assessment, but its availability is currently limited. Aim: We aimed to assess the clinical and instrumental parameters that would be useful for predicting the presence of LGE-CMR, to achieve a better selection of patients with SSc that could benefit from third-level CMR imaging. Methods: 344 SSc patients underwent a comprehensive echocardiogram and LGE-CMR on the same day; for 189 patients, a 24 h ECG Holter monitoring was available. Results: CMR showed non-junctional replacement myocardial fibrosis via LGE in 25.1% patients. A history of digital ulcers (OR 2.188; 95% C.I. 1.069–4.481) and ventricular arrhythmias at ECG Holter monitoring (OR 3.086; 95% C.I. 1.191–7.998) were independent predictors of replacement myocardial fibrosis. Conclusions: CMR can detect patterns of clinical and subclinical cardiac involvement, which are frequent in SSc. A history of digital ulcers and evidence of ventricular arrhythmias at ECG Holter monitoring are red flags for the presence of replacement myocardial fibrosis in CMR. The association between digital ulcers and myocardial fibrosis suggests that a similar pathological substrate of abnormal vascular function may underlie peripheral vascular and cardiac complications. [ABSTRACT FROM AUTHOR]

Anomalous aortic origin of the coronary artery (AAOCA) is a rare disease associated with sudden cardiac death, usually related to physical effort in young people. Clinical routine tests fail to assess the ischemic risk, calling for novel diagnostic approaches. To this aim, some recent studies propose to assess the coronary blood flow (CBF) in AAOCA by computational simulations but they are limited by the use of data from literature retrieved from normal subjects. To overcome this limitation and obtain a reliable assessment of CBF, we developed a fully patient-specific lumped parameter model based on clinical imaging and in-vivo data retrieved during invasive coronary functional assessment of subjects with AAOCA. In such a way, we can estimate the CBF replicating the two hemodynamic conditions in-vivo analyzed. The model can mimic the effective coronary behavior with high accuracy and could be a valuable tool to quantify CBF in AAOCA. It represents the first step required to move toward a future clinical application with the aim of improving patient care. The study was registered at Clinicaltrial.gov with (ID: NCT05159791, date 2021-12-16). [ABSTRACT FROM AUTHOR]

Background: Most recent cardiac implantable electronic devices (CIEDs) can safely undergo a cardiovascular magnetic resonance (CMR) scan under certain conditions, but metal artifacts may degrade image quality. The aim of this study was to assess the overall diagnostic yield of CMR and the extent of metal artifacts in a multicenter, multivendor study on CIED patients referred for CMR. Methods: We analyzed 309 CMR scans from 292 patients (age 57 ± 16 years, 219 male) with an MR-conditional pacemaker (n = 122), defibrillator (n = 149), or loop recorder (n = 38); CMR scans were performed in 10 centers from 2012 to 2020; MR-unsafe implants were excluded. Clinical and device parameters were recorded before and after the CMR scan. A visual analysis of metal artifacts was performed for each sequence on a segmental basis, based on a 5-point artifact score. Results: The vast majority of CMR scans (n = 255, 83%) were completely performed, while only 32 (10%) were interrupted soon after the first sequences and 22 (7%) were only partly acquired; CMR quality was non-diagnostic in 34 (11%) scans, poor (<1/3 sequences were diagnostic) in 25 (8%), or acceptable (1/3 to 2/3 sequences were diagnostic) in 40 (13%), while most scans (n = 201, 68%) were of overall good quality. No adverse event or device malfunctioning occurred, and only nonsignificant changes in device parameters were recorded. The most affected sequences were SSFP (median score 0.32 [interquartile range 0.07–0.91]), followed by GRE (0.18 [0.02–0.59]) and LGE (0.14 [0.02–0.55]). ICDs induced more artifacts (median score in SSFP images 0.87 [0.50–1.46]) than PMs (0.11 [0.03–0.28]) or ILRs (0.11 [0.00–0.56]). Moreover, most artifacts were located in the anterior, anteroseptal, anterolateral, and apical segments of the LV and in the outflow tract of the RV. Conclusions: CMR is a versatile imaging technique, with a high safety profile and overall good image quality even in patients with MR-conditional CIEDs. Several strategies are now available to optimize image quality, substantially enhancing overall diagnostic yield. [ABSTRACT FROM AUTHOR]

Background: The use of apical views focused on the left atrium (LA) has improved the accuracy of LA volume evaluation by two-dimensional (2D) echocardiography. However, routine cardiovascular magnetic resonance (CMR) evaluation of LA volumes still uses standard 2- and 4-chamber cine images focused on the left ventricle (LV). To investigate the potential of LA-focused CMR cine images, we compared LA maximuml (LAVmax) and minimum (LAVmin) volumes, and emptying fraction (LAEF), calculated on both standard and LA-focused long-axis cine images, with LA volumes and LAEF obtained by short-axis cine stacks covering the LA. LA strain was also calculated and compared between standard and LA-focused images. Methods: LA volumes and LAEF were obtained from 108 consecutive patients by applying the biplane area-length algorithm to both standard and LA-focused 2- and 4-chamber cine images. Manual segmentation of a short-axis cine stack covering the LA was used as the reference method. In addition, LA strain reservoir (εs), conduit (εe) and booster pump (εa) were calculated using CMR feature-tracking. Results: Compared to the reference method, the standard approach significantly underestimated LA volumes (LAVmax: bias − 13 ml; LOA = + 11, − 37 ml; LAVmax i: bias − 7 ml/m2; LOA = + 7, − 21 ml/m2; LAVmin; bias − 10 ml, LOA: + 9, − 28 ml; LAVmin i: bias − 5 ml/m2, LOA: + 5, − 16 ml/m2), and overestimated LA-EF (bias 5%, LOA: + 23, − 14%). Conversely, LA volumes (LAVmax: bias 0 ml; LOA: + 10, − 10 ml; LAVmax i: bias 0 ml/m2; LOA: + 5, − 6 ml/m2; LAVmin: bias − 2 ml; LOA: + 7, − 10 ml; LAVmin i: bias − 1 ml/m2; LOA: + 3, − 5 ml/m2) and LAEF (bias 2%, LOA: + 11, − 7%) by LA-focused cine images were similar to those measured using the reference method. LA volumes by LA-focused images were obtained faster than using the reference method (1.2 vs 4.5 min, p < 0.001). LA strain (εs: bias 7%, LOA = 25, − 11%; εe: bias 4%, LOA = 15, − 8%; εa: bias 3%, LOA = 14, − 8%) was significantly higher in standard vs. LA-focused images (p < 0.001). Conclusion: LA volumes and LAEF measured using dedicated LA-focused long-axis cine images are more accurate than using standard LV-focused cine images. Moreover, LA strain is significantly lower in LA-focused vs. standard images. [ABSTRACT FROM AUTHOR]

In the past few years, the wide application of cardiac magnetic resonance (CMR) significantly changed the approach to the study of cardiac involvement in Fabry Disease (FD). The possibility to perform non-invasive tissue characterization, including new sequences such as T1/T2 mapping, offered a powerful tool for differential diagnosis with other forms of left ventricular hypertrophy. In patients with confirmed diagnosis of FD, CMR is the most sensitive non-invasive technique for early detection of cardiac involvement and it provides new insight into the evolution of cardiac damage, including gender-specific features. Finally, CMR multiparametric detection of subtle changes in cardiac morphology, function and tissue composition is potentially useful for monitoring the efficacy of specific treatment over time. This paper aims to provide a comprehensive review of current knowledge regarding the application of CMR in FD cardiac involvement and its clinical implication. [ABSTRACT FROM AUTHOR]

Management of congenital coronary artery anomalies (CAA) is not standardized due to the variety of conditions included and their rare prevalence. Detection of CAA during myocardial infarction with non‐obstructive coronary arteries (MINOCA) may induce clinicians to address the patient for surgery as CAA is not included in any algorithm1,2 for the management of MINOCA and American Association for Thoracic Surgery evidence‐based guidelines suggest surgical repair for patients with anomalous aortic origin of a coronary artery and symptoms compatible with myocardial ischaemia.3 We present the case of a 35‐year‐old man with an anomalous origin of left coronary artery from right Valsalva sinus with pre‐pulmonic course detected during urgent coronary angiography for suspected myocardial infarction. Stress cardiac magnetic resonance did not show signs of ischaemia at high‐dose dobutamine but did reveal a recent myocarditis. This clinical case highlights the need for accurate risk stratification in CAA especially when confounding clinical scenarios co‐exist. [ABSTRACT FROM AUTHOR]

Background: Time‐resolved three‐directional velocity‐encoded (4D flow) magnetic resonance imaging (MRI) enables the quantification of left ventricular (LV) intracavitary fluid dynamics and energetics, providing mechanistic insight into LV dysfunctions. Before becoming a support to diagnosis and patient stratification, this analysis should prove capable of discriminating between clearly different LV derangements. Purpose: To investigate the potential of 4D flow in identifying fluid dynamic and energetics derangements in ischemic and restrictive LV cardiomyopathies. Study Type: Prospective observational study. Population: Ten patients with post‐ischemic cardiomyopathy (ICM), 10 patients with cardiac light‐chain cardiac amyloidosis (AL‐CA), and 10 healthy controls were included. Field Strength/Sequence: 1.5 T/balanced steady‐state free precession cine and 4D flow sequences. Assessment: Flow was divided into four components: direct flow (DF), retained inflow, delayed ejection flow, and residual volume (RV). Demographics, LV morphology, flow components, global and regional energetics (volume‐normalized kinetic energy [KEV] and viscous energy loss [ELV]), and pressure‐derived hemodynamic force (HDF) were compared between the three groups. Statistical Tests: Intergroup differences in flow components were tested by one‐way analysis of variance (ANOVA); differences in energetic variables and peak HDF were tested by two‐way ANOVA. A P‐value of <0.05 was considered significant. Results: ICM patients exhibited the following statistically significant alterations vs. controls: reduced KEV, mostly in the basal region, in systole (−44%) and in diastole (−37%); altered flow components, with reduced DF (−33%) and increased RV (+26%); and reduced basal–apical HDF component on average by 63% at peak systole. AL‐CA patients exhibited the following alterations vs. controls: significantly reduced KEV at the E‐wave peak in the basal segment (−34%); albeit nonstatistically significant, increased peaks and altered time‐course of the HDF basal–apical component in diastole and slightly reduced HDF components in systole. Data Conclusion: The analysis of multiple 4D flow‐derived parameters highlighted fluid dynamic alterations associated with systolic and diastolic dysfunctions in ICM and AL‐CA patients, respectively. Level of Evidence: 2 Technical Efficacy Stage: 3 [ABSTRACT FROM AUTHOR]

We investigated the effect of the design of bioprosthetic pericardial valves on the downstream fluid flow pattern through four-dimensional flow magnetic resonance imaging (4D Flow). A dedicated in vitro test bench, including a paradigmatic aortic root phantom, was used to compare, under steady flow conditions, three commercially used pericardial bioprostheses (TrifectaTM, Carpentier-Edwards PERIMOUNT Magna, Crown PRT®), selecting the two smallest and comparable valve sizes. In-house 4D Flow post-processing provided the downstream flow pattern of velocity, the velocity profile at vena contracta, its effective orifice area (EOA) and the corresponding hydraulic diameter (DH). Trifecta reported the lowest peak of velocity for both the tested sizes, with vena contracta position being the most proximal to the free margin of leaflets. Conversely, in both Crown and Magna, jet flow continued to increase its downstream velocity, resulting in a farther position of vena contracta. EOA shape was trilobal for Magna, triangular for Crown and circular for Trifecta, the last one maximising EOA. The percentage of nominal luminal area effectively exploited by the flow was largely above 80% in Trifecta, below 75% in Crown and below 70% in Magna. Hence, the design of pericardial bioprostheses directly impacts on the downstream flow field pattern and its fluid dynamic performance. [ABSTRACT FROM AUTHOR]

Aims Fabry cardiomyopathy is characterized by glycosphingolipid storage and increased myocardial trabeculation has also been demonstrated. This study aimed to explore by cardiac magnetic resonance whether myocardial trabecular complexity, quantified by endocardial border fractal analysis, tracks phenotype evolution in Fabry cardiomyopathy. Methods and results Study population included 20 healthy controls (12 males, age 32±9) and 45 Fabry patients divided into three groups: 15 left ventricular hypertrophy (LVH)-negative patients with normal T1 (5 males, age 28±13; Group 1); 15 LVH-negative patients with low T1 (9 males, age 33±9.6; Group 2); 15 LVH-positive patients (11 males, age 53.5±9.6; Group 3). Trabecular fractal dimensions (Dfs) (total, basal, mid-ventricular, and apical) were evaluated on cine images. Total Df was higher in all Fabry groups compared to controls, gradually increasing from controls to Group 3 (1.27±0.02 controls vs. 1.29±0.02 Group 1 vs. 1.30±0.02 Group 2 vs. 1.34±0.02 Group 3; P <0.001). Group 3 showed significantly higher values of all Dfs compared to the other Groups. Both basal and total Dfs were significantly higher in Group 1 compared with controls (basal: 1.30±0.03 vs. 1.26±0.04, P  =0.010; total: 1.29±0.02 vs. 1.27±0.02, P =0.044). Total Df showed significant correlations with: (i) T1 value (r =−0.569; P <0.001); (ii) LV mass (r =0.664, P <0.001); (iii) trabecular mass (r =0.676; P  <0.001); (iv) Mainz Severity Score Index (r =0.638; P <0.001). Conclusion Fabry cardiomyopathy is characterized by a progressive increase in Df of endocardial trabeculae together with shortening of T1 values. Myocardial trabeculation is increased before the presence of detectable sphingolipid storage, thus representing an early sign of cardiac involvement. [ABSTRACT FROM AUTHOR]

Congenital bicuspid aortic valve (BAV) consists of two fused cusps and represents a major risk factor for calcific valvular stenosis. Herein, a fully coupled fluid–structure interaction (FSI) BAV model was developed from patient-specific magnetic resonance imaging (MRI) and compared against in vivo 4-dimensional flow MRI (4D Flow). FSI simulation compared well with 4D Flow, confirming direction and magnitude of the flow jet impinging onto the aortic wall as well as location and extension of secondary flows and vortices developing at systole: the systolic flow jet originating from an elliptical 1.6 cm2 orifice reached a peak velocity of 252.2 cm/s, 0.6% lower than 4D Flow, progressively impinging on the ascending aorta convexity. The FSI model predicted a peak flow rate of 22.4 L/min, 6.7% higher than 4D Flow, and provided BAV leaflets mechanical and flow-induced shear stresses, not directly attainable from MRI. At systole, the ventricular side of the non-fused leaflet revealed the highest wall shear stress (WSS) average magnitude, up to 14.6 Pa along the free margin, with WSS progressively decreasing towards the belly. During diastole, the aortic side of the fused leaflet exhibited the highest diastolic maximum principal stress, up to 322 kPa within the attachment region. Systematic comparison with ground-truth non-invasive MRI can improve the computational model ability to reproduce native BAV hemodynamics and biomechanical response more realistically, and shed light on their role in BAV patients' risk for developing complications; this approach may further contribute to the validation of advanced FSI simulations designed to assess BAV biomechanics. [ABSTRACT FROM AUTHOR]

Aims To investigate the prognostic relevance of coronary anatomy, coronary function, and early revascularization in patients with stable coronary artery disease (CAD). Methods and results From March 2009 to June 2012, 430 patients with suspected CAD (61 ± 9 years, 62% men) underwent coronary anatomical imaging by computed tomography coronary angiography (CTCA) and coronary functional imaging followed by invasive coronary angiography (ICA) if at least one non-invasive test was abnormal. Obstructive CAD was documented by ICA in 119 patients and 90 were revascularized within 90 days of enrolment. Core laboratory analysis showed that 134 patients had obstructive CAD by CTCA (>50% stenosis in major coronary vessels) and 79 significant ischaemia by functional imaging [>10% left ventricular (LV) myocardium]. Over mean follow-up of 4.4 years, major adverse events (AEs) (all-cause death, non-fatal myocardial infarction, or hospital admission for unstable angina or heart failure) or AEs plus late revascularization (LR) occurred in 40 (9.3%) and 58 (13.5%) patients, respectively. Obstructive CAD at CTCA was the only independent imaging predictor of AEs [hazard ratio (HR) 3.2, 95% confidence interval (CI) 1.10–9.30; P  = 0.033] and AEs plus LR (HR 4.3, 95% CI 1.56–11.81; P  = 0.005). Patients with CAD in whom early revascularization was performed in the presence of ischaemia and deferred in its absence had fewer AEs, similar to patients without CAD (HR 2.0, 95% CI 0.71–5.51; P  = 0.195). Conclusion Obstructive CAD imaged by CTCA is an independent predictor of clinical outcome. Early management of CAD targeted to the combined anatomical and functional disease phenotype improves clinical outcome. [ABSTRACT FROM AUTHOR]

Aim: This study aimed at evaluating the cost-effectiveness of different non-invasive imaging-guided strategies for the diagnosis of obstructive coronary artery disease (CAD) in a European population of patients from the Evaluation of Integrated Cardiac Imaging in Ischemic Heart Disease (EVINCI) study.Methods and Results: Cost-effectiveness analysis was performed in 350 patients (209 males, mean age 59 ± 9 years) with symptoms of suspected stable CAD undergoing computed tomography coronary angiography (CTCA) and at least one cardiac imaging stress-test prior to invasive coronary angiography (ICA) and in whom imaging exams were analysed at dedicated core laboratories. Stand-alone stress-tests or combined non-invasive strategies, when the first exam was uncertain, were compared. The diagnostic end-point was obstructive CAD defined as > 50% stenosis at quantitative ICA in the left main or at least one major coronary vessel. Effectiveness was defined as the percentage of correct diagnosis (cd) and costs were calculated using country-specific reimbursements. Incremental cost-effectiveness ratios (ICERs) were obtained using per-patient data and considering "no-imaging" as reference. The overall prevalence of obstructive CAD was 28%. Strategies combining CTCA followed by stress ECHO, SPECT, PET, or stress CMR followed by CTCA, were all cost-effective. ICERs values indicated cost saving from - 969€/cd for CMR-CTCA to - 1490€/cd for CTCA-PET, - 3092€/cd for CTCA-SPECT and - 3776€/cd for CTCA-ECHO. Similarly when considering early revascularization as effectiveness measure.Conclusion: In patients with suspected stable CAD and low prevalence of disease, combined non-invasive strategies with CTCA and stress-imaging are cost-effective as gatekeepers to ICA and to select candidates for early revascularization. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Post-infarction myocardial scar causes adverse left ventricular remodelling and negatively affects the prognosis. We sought to investigate whether scar extent and location obtained by cardiac magnetic resonance may affect the reverse remodelling and survival of heart failure patients undergoing surgical ventricular reconstruction. METHODS: From January 2011 to December 2015, 151 consecutive patients with previous myocardial infarction and left ventricular remodelling underwent surgical ventricular reconstruction at our Institution, of which 88 (58%) patients had a preoperative protocol-standardized late gadolinium enhancement (LGE)-cardiac magnetic resonance examination during the week before surgery. We excluded 40 patients with devices (26%), 15 patients with irregular heart rhythm (permanent atrial fibrillation, 10% not included in the device group) or mixed contraindications (severe claustrophobia or presence of material magnetic resonance not compatible). Among the 145 survivors, 11 patients received an implantable cardioverter defibrillator after surgery (mostly for persistent low ejection fraction) and were excluded as well, yielding a total of 59 patients (48 men, aged 65 ± 9 years) who repeated a protocol-standardized LGE-cardiac magnetic resonance examination even 6 months postoperatively and therefore represent the study population. Patients were grouped according to the presence of LGE in the antero-basal left ventricular segments (Group A) or the absence of LGE in the same segments (Group B). The postoperative left ventricular end-systolic volume index was considered the primary end-point. RESULTS: After surgery, left ventricular end-systolic volume index and end-diastolic volume index significantly decreased (P < 0.001, for both), while diastolic sphericity index and ejection fraction significantly increased (P = 0.015 and P < 0.001, respectively). The presence of LGE in the antero-basal left ventricular segments (10 patients, Group A) was the only independent predictor of outcome (P = 0.02) at multivariate analysis, being the postoperative left ventricular end-systolic volume index significantly higher compared to that of patients of Group B (49 patients) (78 ± 26 ml/m2 vs 55 ± 20 ml/m2, P = 0.003). Furthermore, patients with a postoperative left ventricular end-systolic volume index >60 ml/m2 showed a higher risk of cardiac events (hazard ratio = 3.67, P = 0.02). CONCLUSIONS: In patients undergoing surgical ventricular reconstruction, LGE scar location affects the left ventricular reverse remodelling, which in turn might limit the survival benefit. [ABSTRACT FROM AUTHOR]

Purpose: To define reference values of cardiac volumes, dimensions, and new morpho-functional parameters normalized for age, gender, and body surface area by cine-bSSFP (balanced steady-state free-precession) magnetic resonance (MR).Materials and Methods: We enrolled 308 healthy subjects subdivided by gender and by six age classes: class I, >15-20 years; class II, >20-30 years; class III, >30-40 years; class IV, >40-50 years; class V, >50-60 years; and class VI >60 years. Dimensional, volumetric and morpho-functional parameters of the left (LV) and right (RV) ventricles were measured using cine-bSSFP MRI at 1.5T.Results: The LV and RV end-diastolic volume indexes (EDVi) were inversely related to age (P < 0.0001 r = -0.34 and P < 0.0001 r = -0.37, respectively). In addition, the LV mass index decreased with age (P = 0.0004, r = -0.21). The LV longitudinal shortening was not significantly different among groups: ≥15% in all populations (95% confidence interval [CI]: 16-31). The sphericity index measured in end-diastole was higher in females than in males (P < 0.03): the upper limit was 40% for males and 42% for females. The normality cutoff of LV global function index was ≥33% in males and ≥35% in females. The end-diastolic volume (EDV) of RV and LV was balanced (RV/LV ratio 0.85-1.15) without differences in the population. The LV EDV/mass was 1.0-1.8 in males and 1.0-2.1 in females.Conclusion: This study provides potential age- and gender-specific reference.Level Of Evidence: 2 J. Magn. Reson. Imaging 2017;45:1055-1067. [ABSTRACT FROM AUTHOR]

Background: Coronary artery disease (CAD) continues to be one of the top public health burden. Perfusion cardiovascular magnetic resonance (CMR) is generally accepted to detect CAD, while data on its cost effectiveness are scarce. Therefore, the goal of the study was to compare the costs of a CMR-guided strategy vs two invasive strategies in a large CMR registry. Methods: In 3'647 patients with suspected CAD of the EuroCMR-registry (59 centers/18 countries) costs were calculated for diagnostic examinations (CMR, X-ray coronary angiography (CXA) with/without FFR), revascularizations, and complications during a 1-year follow-up. Patients with ischemia-positive CMR underwent an invasive CXA and revascularization at the discretion of the treating physician (=CMR + CXA-strategy). In the hypothetical invasive arm, costs were calculated for an initial CXA and a FFR in vessels with ≥50 % stenoses (=CXA + FFR-strategy) and the same proportion of revascularizations and complications were applied as in the CMR + CXA-strategy. In the CXA-only strategy, costs included those for CXA and for revascularizations of all ≥50 % stenoses. To calculate the proportion of patients with ≥50 % stenoses, the stenosis-FFR relationship from the literature was used. Costs of the three strategies were determined based on a third payer perspective in 4 healthcare systems. Results: Revascularizations were performed in 6.2 %, 4.5 %, and 12.9 % of all patients, patients with atypical chest pain (n =1'786), and typical angina (n = 582), respectively; whereas complications (=all-cause death and non-fatal infarction) occurred in 1.3 %, 1.1 %, and 1.5 %, respectively. The CMR + CXA-strategy reduced costs by 14 %, 34 %, 27 %, and 24 % in the German, UK, Swiss, and US context, respectively, when compared to the CXA + FFR-strategy; and by 59 %, 52 %, 61 % and 71 %, respectively, versus the CXA-only strategy. In patients with typical angina, cost savings by CMR + CXA vs CXA + FFR were minimal in the German (2.3 %), intermediate in the US and Swiss (11.6 % and 12.8 %, respectively), and remained substantial in the UK (18.9 %) systems. Sensitivity analyses proved the robustness of results. Conclusions: A CMR + CXA-strategy for patients with suspected CAD provides substantial cost reduction compared to a hypothetical CXA + FFR-strategy in patients with low to intermediate disease prevalence. However, in the subgroup of patients with typical angina, cost savings were only minimal to moderate. [ABSTRACT FROM AUTHOR]

Cardiac Magnetic Resonance (CMR) allows evaluation of the functional and flow changes in pericardial constriction as well as detection of acute pericardial inflammation, fusion and thickening of pericardial layers and pericardial effusion. We sought to evaluate the diagnostic role of tissue characterization by CMR in constrictive pericarditis (CP). We performed a CMR exam in 70 patients (mean age 58 ± 16) with clinical suspicion of constrictive pericarditis and constrictive pattern at echocardiography and/or catheterization. A multiparametric CMR approach was used to evaluate the initial diagnostic suspicion. A clinical follow-up was performed in all patients for a median of 551 days. The diagnosis of CP was confirmed in 53 patients while 12 patients presented signs of predominant pericardial active inflammation suggesting a diagnosis of transient constrictive pericarditis and five presented effusive-constrictive pericarditis. Patients with a final diagnosis of CP had worse prognosis than those with transient constrictive or effusive constrictive pericarditis. The presence of myocardial late gadolinium enhancement was associated to adverse events. Results of the current study confirmed the value of CMR in the differential diagnosis of pericardial disease. A multiparametric CMR approach allowed to distinguish between active inflammation, chronic pericarditis with constriction and effusion without inflammation. [ABSTRACT FROM AUTHOR]

Prognostic implication of right ventricular dysfunction and infarction scar in the chronic phase of the myocardial infarction has been little analyzed. In 299 consecutive patients (age 63 ± 11 years) with >3 months old myocardial infarction, we quantified right and left ventricular volumes and ejection fractions by cine cardiac magnetic resonance, and right and left ventricular scar tissue by late gadolinium enhancement. During follow-up (median, 2.4 years) cardiac events (cardiac-related deaths or appropriate intra-cardiac defibrillator shocks) occurred in 21 patients. Right ventricular systolic dysfunction (ejection fraction lower the reference mean values-2 SD) was present in 67 patients (22 %), right ventricular late gadolinium enhancement was observed in 15 patients (5 %). After adjustment for left ventricular end-diastolic volume, wall motion score index, and global extent of late gadolinium enhancement, right ventricular dysfunction was an independent and incremental predictor of cardiac events ( p = 0.0053), while right ventricular scar tissue extent was not. Right ventricular dysfunction is an independent and incremental predictor of cardiac events also in the chronic phase of the myocardial infarction. In these patients, right ventricular dysfunction does not necessarily mean right ventricular infarction scar, but likely reflects the effects of hemodynamic and biohumoral factors. [ABSTRACT FROM AUTHOR]

The placement of temporary epicardial pacing wires (EPWs) at the completion of cardiac surgery is a routine procedure in most centers. Complications related to their insertion, removal and retention are infrequent, yet potentially severe, including hemorrhage, tamponade, infection and death. Here, we describe an unusual case of retained temporary EPWs migration. [ABSTRACT FROM AUTHOR]

Background: Myocardial hyperintensity on T2-weighted short-tau inversion recovery (STIR) (HyT2) cardiac magnetic resonance (CMR) images has been demonstrated in patients with hypertrophic cardiomyopathy (HCM) and is considered a sign of acute damage. The aim of the current study was to evaluate the relationship between HyT2 and both a) markers of ventricular electrical instability and b) clinical and CMR parameters. Methods: Sixty-five patients underwent a thorough clinical examination, consisting of 24-h ECG recording and CMR examination including functional evaluation, T2-STIR images and late gadolinium enhancement (LGE). Results: HyT2 was detected in 27 patients (42%), and subjects with HyT2 showed a greater left ventricle (LV) mass index (p<0.001), lower LV ejection fraction (p = 0.05) and greater extent of LGE (p<0.001) compared to those without HyT2. Twenty-two subjects (34%) presented non-sustained ventricular tachycardia (NSVT) on the 24-h ECG recording, 21 (95%) of whom exhibited HyT2. Based on the logistic regression analysis, HyT2 (odds ratio [OR]: 165, 95% CI 11–2455, p<0.001) and LGE extent (1.1, 1.0–1.3, p<0.001) served as independent predictors of NSVT, while the presence of LGE was not associated with NSVT occurrence (p = 0.49). The presence of HyT2 was associated with lower heart rate variability (p = 0.006) and a higher number of arrhythmic risk factors (p<0.001). Conclusions: In HCM patients, HyT2 upon CMR examination is associated with more advanced disease and increased arrhythmic burden. [ABSTRACT FROM AUTHOR]

Purpose To determine whether systematic differences were present between myocardial R2* values obtained with two different decay models: truncation and exponential + constant (Exp-C). Methods Single-center cohorts were used to compare black and bright blood sequences separately, and a multicenter cohort of mixed bright and black blood studies was used to assess the generalizability. Truncated exponential estimates were calculated with CMRtools, which uses a single region of interest (ROI) method. Exp-C estimates were calculated using a pixelwise approach. Results No differences could be distinguished based upon whether a white or black blood sequence was examined. The two fitting algorithms yielded similar R2* values, with R-squared values exceeding 0.997 and a coefficient of variation of 3% to 4%. Results using the pixelwise method yielded a small systematic bias (∼3%) that became apparent in patients with severe iron deposition. This disparity disappeared when Exp-C fitting was used on a single ROI, suggesting that the use of pixelwise mapping was responsible for the bias. In the multicenter cohort, the strong agreement between the two fitting approaches was reconfirmed. Conclusion Cardiac R2* values are independent of the signal model used for its calculation over clinically relevant ranges. Clinicians can compare results among centers using these disparate approaches with confidence. Magn Reson Med 72:485-491, 2014. © 2013 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Purpose In steady state free precession (SSFP) images acquired with a repetition time/echo time (TR/TE) ≈ 2, fat is surrounded by a black boundary, called 'India Ink' artifact. Indian Ink artifact may improve detection of intramyocardial fat. Aims of this study were: (i) to assess the accuracy of SSFP technique for the detection of fat metaplasia in remote myocardial infarction (RMI); (ii) to evaluate the inter- and intraobserver reproducibility for the quantification of intramyocardial fat using SSFP and fast spin echo/short TI inversion recovery (FSE/STIR) techniques. Materials and Methods A total of 200 patients (age 64 ± 10 years) with RMI (>1000 days) underwent MRI using a 1.5 Tesla (T) scanner. SSFP images (with a TR/TE ≈2), FSE and STIR images were acquired in short and long axis views. Fat was detected in FSE/STIR and SSFP images and its extent manually measured . The inter- and intraobserver agreement for the quantification of fat metaplasia using both the SSFP image and the FSE images was evaluated. Results Left ventricle intramyocardial fat was detected in SSFP images of 95 patients (47.5%) and in FSE/STIR images of 84 patients (42%). A very good agreement was found using the SSFP technique between investigators. Conclusion SSFP sequence with TR/TE=2 is a valuable technique for identifying and quantifying the presence of fat tissue within the left ventricle myocardium in RMI. J. Magn. Reson. Imaging 2014;40:126-132. © 2013 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Purpose Realizing the challenges and opportunities of effective transverse relaxation rate ( R2*) mapping at high and ultrahigh fields, this work examines magnetic field strength ( B0) dependence and segmental artifact distribution of myocardial R2* at 1.5, 3.0, and 7.0 T. Methods Healthy subjects were considered. Three short-axis views of the left ventricle were examined. R2* was calculated for 16 standard myocardial segments. Global and mid-septum R2* were determined. For each segment, an artifactual factor was estimated as the deviation of segmental from global R2* value. Results The global artifactual factor was significantly enlarged at 7.0 T versus 1.5 T ( P = 0.010) but not versus 3.0 T. At 7.0 T, the most severe susceptibility artifacts were detected in the inferior lateral wall. The mid-septum showed minor artifactual factors at 7.0 T, similar to those at 1.5 and 3.0 T. Mean R2* increased linearly with the field strength, with larger changes for global heart R2* values. Conclusion At 7.0 T, segmental heart R2* analysis is challenging due to macroscopic susceptibility artifacts induced by the heart-lung interface and the posterior vein. Myocardial R2* depends linearly on the magnetic field strength. The increased R2* sensitivity at 7.0 T might offer means for susceptibility-weighted and oxygenation level-dependent MR imaging of the myocardium. Magn Reson Med 71:2224-2230, 2014. © 2013 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Purpose To evaluate the efficiency of a novel transit time (Δt) estimation method from cardiovascular magnetic resonance flow curves. Materials and Methods Flow curves were estimated from phase contrast images of 30 patients. Our method (TT-GD: transit time group delay) operates in the frequency domain and models the ascending aortic waveform as an input passing through a discrete-component 'filter,' producing the observed descending aortic waveform. The GD of the filter represents the average time delay (Δt) across individual frequency bands of the input. This method was compared with two previously described time-domain methods: TT-point using the half-maximum of the curves and TT-wave using cross-correlation. High temporal resolution flow images were studied at multiple downsampling rates to study the impact of differences in temporal resolution. Results Mean Δts obtained with the three methods were comparable. The TT-GD method was the most robust to reduced temporal resolution. While the TT-GD and the TT-wave produced comparable results for velocity and flow waveforms, the TT-point resulted in significant shorter Δts when calculated from velocity waveforms (difference: 1.8 ± 2.7 msec; coefficient of variability: 8.7%). The TT-GD method was the most reproducible, with an intraobserver variability of 3.4% and an interobserver variability of 3.7%. Conclusion Compared to the traditional TT-point and TT-wave methods, the TT-GD approach was more robust to the choice of temporal resolution, waveform type, and observer. J. Magn. Reson. Imaging 2014;39:550-558. © 2013 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Current cardiac magnetic resonance (CMR) quantitative signs for the diagnosis of myocarditis include myocardial edema, fibrosis and myocardial hyperemia (Hyp). Methods for the assessment of Hyp are actually complex and time-consuming. To test a simple and fast method to assess Hyp, using contrast enhancement steady state free precession (ceSSFP) technique. CMR imaging at 1.5T was performed on 39 patients with diagnosis of acute myocarditis and in 20 healthy controls. Hyp was evaluated in systolic and diastolic frames (Hyp-SYS and Hyp-DIA) as areas of myocardial hyperintensity in ceSSFP images early after gadolinium injection. Myocardial edema was evaluated using T2-STIR images. Myocardial fibrosis was assessed in conventional late gadolinium enhancement (LGE) images. A value of ≤12.1 g of Hyp-DIA was obtained as cut-off of normality in healthy controls. Using this threshold, Hyp was detected in 30 patients (77 %) with myocarditis. LGE was detected in 36 patients (92 %), and myocardial edema in 38 (97 %) patients with myocarditis A linear relation was found between Hyp-DIA and the extent of myocardial edema (R 0.48, 95 % CI 0.47-0.85, p < 0.001) and the extent of LGE (R 0.41, 95 % CI 0.31-0.61, p < 0.001). Patients with hyperemia had higher levels of C-reactive protein ( p < 0.001), a higher extent of LGE ( p < 0.05) and a larger left atrial area ( p < 0.05). ceSSFP sequence at CMR is a novel and fast method to assess myocardial hyperemia in patient with acute myocarditis. Compared with non-Hyp subjects, patients with Hyp had more signs of inflammation and myocardial damage. [ABSTRACT FROM AUTHOR]

The age at which it is necessary to start Cardiovascular Magnetic Resonance ( CMR) T2* screening in thalassaemia major ( TM) is still uncertain. To clarify this point, we evaluated the prevalence of myocardial iron overload ( MIO), function and fibrosis by CMR in TM patients younger than 10 years. We retrospectively selected 35 TM patients enrolled in the Myocardial Iron Overload in Thalassaemia network. MIO was measured by T2* multislice multiecho technique. Biventricular function parameters were evaluated by cine images. To detect myocardial fibrosis, late gadolinium enhancement images were acquired. Patients' age ranged from 4·2 to 9·7 years. All scans were performed without sedation. Nine patients showed no MIO, 22 patients had heterogeneous MIO with a T2* global value ≥20 ms; two patients had heterogeneous MIO with a T2* global value <20 ms and two patients showed homogeneous MIO. No patient showed myocardial fibrosis. Among the patients with heart T2*<20 ms, the youngest was 6 years old, none showed heart dysfunction and the iron transfused was <35 g in all cases. Cardiac iron loading can occur much earlier than previously described. The first cardiac T2* assessment should be performed as early as feasible without sedation, especially if chelation is started late or if poor compliance is suspected. [ABSTRACT FROM AUTHOR]

Purpose: CZT technology allows ultrafast low-dose myocardial scintigraphy but its accuracy in assessing left ventricular function is still to be defined. Methods: The study group comprised 55 patients (23 women, mean age 63 ± 9 years) referred for myocardial perfusion scintigraphy. The patients were studied at rest using a CZT camera (Discovery NM530c; GE Healthcare) and a low-dose Tc-tetrofosmin clinical protocol (mean dose 264 ± 38 MBq). Gated SPECT imaging was performed as a 6-min list-mode acquisition, 15 min after radiotracer injection. Images were reformatted (8-frame to 16-frame) using Lister software on a Xeleris workstation (GE Healthcare) and then reconstructed with a dedicated iterative algorithm. Analysis was performed using Quantitative Gated SPECT (QGS) software. Within 2 weeks patients underwent cardiac magnetic resonance imaging (cMRI, 1.5-T unit CVi; GE Healthcare) using a 30-frame acquisition protocol and dedicated software for analysis (MASS 6.1; Medis). Results: The ventricular volumes obtained with 8-frame QGS showed excellent correlations with the cMRI volumes (end-diastolic volume (EDV), r = 0.90; end-systolic volume (ESV), r = 0.94; p < 0.001). However, QGS significantly underestimated the ventricular volumes (mean differences: EDV, −39.5 ± 29 mL; ESV, −15.4 ± 22 mL; p < 0.001). Similarly, the ventricular volumes obtained with 16-frame QGS showed an excellent correlations with the cMRI volumes (EDV, r = 0.92; ESV, r = 0.95; p < 0.001) but with significant underestimations (mean differences: EDV, −33.2 ± 26 mL; ESV, −17.9 ± 20 mL; p < 0.001). Despite significantly lower values (47.9 ± 16 % vs. 51.2 ± 15 %, p < 0.008), 8-frame QGS mean ejection fraction (EF) was closely correlated with the cMRI values ( r = 0.84, p < 0.001). The mean EF with 16-frame QGS showed the best correlation with the cMRI values ( r = 0.91, p < 0.001) and was similar to the mean cMRI value (49.6 ± 16 %, p not significant). Regional analysis showed a good correlation between both 8-frame and 16-frame QGS and cMRI wall motion score indexes (8-frame WMSI, r = 0.85; 16-frame WMSI, r = 0.89; p < 0.01). Conclusion: Low-dose gated SPECT with a CZT camera provides ventricular volumes that correlate well with cMRI results despite significant underestimation in the measure values. EF estimation appeared to be more accurate with 16-frame reformatted images than with 8-frame images. [ABSTRACT FROM AUTHOR]