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Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular dysfunction and excessive fibrosis, involving internal organs including the heart. The estimated prevalence of cardiac involvement in SSc is high and remains subclinical until the late stages. It is either primary, related to myocardial inflammation and fibrosis, or secondary, due to pulmonary arterial hypertension (SSc-PAH) or systemic hypertension, in those patients with renal involvement. Cardiovascular magnetic resonance (CMR) is a useful tool for the early assessment of cardiac involvement in SSc. It is the gold standard technique to assess ventricular volumes,ejection fraction, and in particular is very useful to reliably and non-invasively detect myocardial inflammation, early perfusion defects, and myocardial fibrosis. However, the CMR evaluation in SSc may be problematic, because of cardiac and respiratory artefacts, commonly found in these patients. Therefore, a high level of expertise is necessary for both acquisition and interpretation of CMR images in SSc., (Copyright © 2017 Elsevier Inc. All rights reserved.)

The current document was developed by the working group on the 'application of cardiac magnetic resonance' of the Italian Society of Cardiology to provide a perspective on the current state of technical advances and clinical cardiac magnetic resonance applications and to inform cardiologists how to implement their clinical and diagnostic pathway with the introduction of this technique in the clinical practice. Appropriateness criteria were defined using a score system: score 1-3 = inappropriate (test is not generally acceptable and is not a reasonable approach for the indication), score 4-6 = uncertain (test may be generally acceptable and may be a reasonable approach for the indication but more research and/or patient information is needed to classify the indication definitively) and score 7-9 = appropriate (test is generally acceptable and is a reasonable approach for the indication).

Cardiac magnetic resonance (CMR) imaging has witnessed substantial progress with the advent of parametric mapping techniques, most notably T1 and T2 mapping. These advanced techniques provide valuable insights into a wide range of cardiac conditions, including ischemic heart disease, cardiomyopathies, inflammatory cardiomyopathies, heart valve disease, and athlete's heart. Mapping could be the first sign of myocardial injury and oftentimes precedes symptoms, changes in ejection fraction, and irreversible myocardial remodeling. The ability of parametric mapping to offer a quantitative assessment of myocardial tissue properties addresses the limitations of conventional CMR methods, which often rely on qualitative or semiquantitative data. However, challenges persist, especially in terms of standardization and reference value establishment, hindering the wider clinical adoption of parametric mapping. Future developments should prioritize the standardization of techniques to enhance their clinical applicability, ultimately optimizing patient care pathways and outcomes. In this review, we endeavor to provide insights into the potential contributions of CMR mapping techniques in enhancing the diagnostic processes across a range of cardiac conditions. [ABSTRACT FROM AUTHOR]

Aims The 2010 Task Force Criteria (TFC) require that both right ventricular (RV) regional wall-motion abnormalities (WMA) and specific RV size cut-offs be met in order to fulfil one of the major criterion for arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnosis. Currently, 2D echocardiography (2DE) and cardiovascular magnetic resonance imaging (cMRI) are used to determine if these criteria are met. Little is known about the diagnostic value of 3D echocardiography (3DE) in ARVC. The aim of this study was to determine whether a combination of 2DE-3DE is non-inferior to the currently used 2DE-cMRI combination in the diagnosis of patients with ARVC. Methods and results Thirty-nine individuals (47±15 years) with suspected ARVC underwent evaluation of the RV with cMRI, 2DE, and 3DE. 3DE and cMRI were independently used to obtain RV volumes, ejection fraction (EF) and determine the presence of segmental RV WMA. Studies were blindly classified as meeting criteria for ARVC in accordance with the 2010 TFC. Kappa statistics were used to test the concordance between 2DE–cMRI and 2DE–3DE approaches. Using the 2DE–cMRI approach, 3/39 were not affected, 5/39 possible, 8/39 borderline, and 23/39 definite ARVC. The proposed 2DE–3DE approach yielded 5/39 not affected, 7/39 possible, 8/39 borderline, and 19/39 definite diagnoses. The two approaches were highly concordant (k = 0.71; 95% confidence interval: 0.44–0.84). Although 3DE underestimated RV volumes in comparison with cMRI, interfering, in some instances with the fulfilment of a major criterion, it was able to identify more RV WMA (28/39) than 2DE (11/39), with a detection-rate comparable to cMRI (33/39) highlighting a unique advantage. Conclusion The combination of 2DE–3DE for ARVC diagnosis is comparable to the conventional 2DE–cMRI approach. 3DE should be performed in all suspected ARVC patients to aide in the detection of WMA. [ABSTRACT FROM AUTHOR]

Objectives Cardiac rhythm disturbances constitute the most frequent cardiovascular cause of death in SSc. However, electrocardiographic findings are not a part of risk stratification in SSc. We aimed to translate 24 h Holter findings into a tangible risk prediction score using cardiovascular magnetic resonance. Methods The Scleroderma Arrhythmia Clinical Utility Study (SAnCtUS) was a prospective multicentre study including 150 consecutive SSc patients from eight European centres, assessed with 24 h Holter and cardiovascular magnetic resonance, including ventricular function, oedema (T2 ratio) and late gadolinium enhancement (%LGE). Laboratory/clinical parameters were included in multivariable corrections. A combined endpoint of sustained ventricular tachycardia requiring hospitalization and sudden cardiac death at a median (interquartile range) follow-up of 1 (1.0–1.4) year was generated. Results Only T2 ratio and %LGE were significant predictors of ventricular rhythm disturbances, but not of supraventricular rhythm disturbances, after multivariable correction and adjustment for multiple comparisons. Using decision-tree analysis, we created the SAnCtUS score, a four-category scoring system based on T2 ratio and %LGE, for identifying SSc patients at high risk of experiencing ventricular rhythm disturbance at baseline. Increasing SAnCtUS scores were associated with a greater disease and arrhythmic burden. All cases of non-sustained ventricular tachycardia (n = 7) occurred in patients with the highest SAnCtUS score (=4). Having a score of 4 conveyed a higher risk of reaching the combined endpoint in multivariable Cox regression compared with scores 1/2/3 [hazard ratio (95% CI): 3.86 (1.14, 13.04), P = 0.029] independently of left ventricular ejection fraction and baseline ventricular tachycardia occurrence. Conclusion T2 ratio and %LGE had the greatest utility as independent predictors of rhythm disturbances in SSc patients. [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: In previous studies, the Valsalva graft''s compliance at the level of the Dacron pseudosinuses was found similar to that of normal sinuses shortly (2 ± 1 months) after the operation. We sought to investigate with cardiac magnetic resonance the compliance of the Valsalva graft pseudosinuses at midterm follow-up. Methods: Seven patients (group A) and 7 age-matched controls (group B) were studied with steady-state free precession and phase-contrast cardiac magnetic resonance for aortic root and ascending aorta evaluation. Blood pressure was measured during phase-contrast acquisition to derive the following mechanical properties of the vascular prosthesis: pulsatility, compliance, distensibility, and elastic modulus. Results: Mean postoperative follow-up was 55 ± 9.84 months. Mean age was 69.2 ± 4.98 years in group A, and 65.7 ± 7.16 years in group B. All the studied variables were coherent in showing a significant difference between the two groups, and between aortic root (skirt portion of the graft) and ascending aorta (tubular part of the graft) in group A. The presence of periaortic fibrosis did not show any correlation with the ascending aorta''s mechanical properties. Conclusions: At midterm follow-up, the pseudosinuses compliance of the Valsalva graft is still appreciable and significantly greater than the tubular portion. [Copyright &y& Elsevier]

The article describes the case of a 41-year-old female suffering from chest pain who underwent a successful resection of a right atrial mass detected at cardiac ultrasound and where a diagnosis of hamartoma of mature cardiac myocytes (HMCMs) was made at histological examination.

An abstract of the article "Usefulness of semi-quantification of ischemic myocardium after adenosine stress magnetic resonance," by Lorenzo Monti, Marco Tramarin, Margherita Calcagnino, Veronica Lisignoli, Barbara Nardi, and Luca Balzarini is presented.

A photograph of cardiac magnetic resonance (CMR) of a 29-year-old man with left ventricular cleft is presented.