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Aims: Myocardial involvement is common in patients with systemic sclerosis (SSc) and causes myocardial fibrosis and subtle ventricular dysfunction. However, the temporal onset of myocardial involvement during the progression of the disease and its prognostic value are yet unknown. We used cardiovascular magnetic resonance (CMR) to investigate subclinical functional impairment and diffuse myocardial fibrosis in patients with very early diagnosis of SSc (VEDOSS) and established SSc and examined whether this was associated with mortality., Methods and Results: One hundred and ten SSc patients (86 established SSc, 24 VEDOSS) and 15 healthy controls were prospectively recruited. The patients were followed-up for a median duration of 7.0 years (interquartile range 6.0-7.3 years). Study subjects underwent CMR including assessment of myocardial fibrosis [native T1 and extracellular volume (ECV)] and measurement of global longitudinal (GLS) and circumferential (GCS) myocardial strain. Native T1 values and ECV were elevated in VEDOSS and SSc patients compared with controls (P < 0.001). GLS was similar in VEDOSS and controls but significantly impaired in patients with established SSc (P < 0.001). GCS was similar over all groups (P = 0.88). There were 12 deaths during follow-up. Elevated native T1 [hazard ratio (HR) 5.8, 95% confidence interval (CI): 1.7-20.4; P = 0.006] and reduced GLS (HR 6.1, 95% CI: 1.3-29.9; P = 0.038) identified subjects with increased risk of death. Only native T1 was predictive for cardiovascular mortality (P < 0.001)., Conclusion: Subclinical myocardial involvement first manifests as diffuse myocardial fibrosis identified by the expansion of ECV and increased native T1 in VEDOSS patients while subtle functional impairment only occurs in established SSc. Native T1 and GLS have prognostic value for all-cause mortality in SSc patients., Competing Interests: Conflict of interest: B.M. has/had grant/research support from Abbvie, Protagen, Novartis Biomedical Research, received speaker fees from Böhringer Ingelheim as well as congress support from Pfizer, Roche, Actelion, Mepha, and MSD. C.M. has received congress support from Actelion and Roche, and personal fees from Boehringer-Ingelheim, Mepha, and MEDtalks Switzerland, outside the submitted work. R.D. reports grants/research support from Pfizer, Actelion, and personal fees (speaker/consultancy) from Actelion and Boehringer-Ingelheim, outside the submitted work. O.D. has/had consultancy relationship with and/or has received research funding from or has served as a speaker for the following companies in the area of potential treatments for systemic sclerosis and its complications in the last 3 years: Abbvie, Acceleron, Alcimed, Amgen, AnaMar, Arxx, AstraZeneca, Baecon, Blade, Bayer, Boehringer-Ingelheim, ChemomAb, Corbus, CSL Behring, Galapagos, Glenmark, GSK, Horizon (Curzion), Inventiva, iQvia, Kymera, Lupin, Medac, Medscape, Miltenyi Biotec, Mitsubishi Tanabe, Novartis, Prometheus, Roche, Roivant, Sanofi, Serodapharm, Topadur and UCB. Patent issued ‘mir-29 for the treatment of systemic sclerosis’ (US8247389, EP2331143). O.D. and B.M. have a patent for ‘mir-29 for the treatment of systemic sclerosis’ (US8247389, EP2331143). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Aims: Coronary artery disease (CAD) is a leading cause of morbidity and mortality in women and non-invasive testing for CAD in women can be more challenging than in men. This study compared the diagnostic performance of whole-heart dynamic 3D cardiovascular magnetic resonance (CMR) stress perfusion imaging in female and male patients with quantitative coronary angiography (QCA) and fractional flow reserve (FFR) as reference tests., Methods and Results: Four hundred sixteen patients with suspected or known CAD were enrolled in five European centres. CMR imaging was performed prior to clinically indicated coronary angiography. QCA was performed in all patients and FFR in 357 of 416 patients. Whole-heart dynamic 3D CMR first-pass perfusion imaging was conducted at rest and during adenosine stress. All CMR analyses were operated by experienced investigators blinded to all clinical data. One hundred nineteen female and 297 male patients were included and successfully examined (mean age 65 ± 11 and 63 ± 11 years, respectively). FFR was performed in 106 female and 251 male patients. Sensitivity and specificity of whole-heart dynamic 3D CMR stress perfusion imaging were 89% (95% CI: 77-96) and 82% (95% CI: 70-90) in the female population and 83% (95% CI: 77-86) and 79% (95% CI: 71-86) in the male population relative to QCA (P = 0.474 and P = 0.83, P-values for comparison between genders). Sensitivity and specificity were 95% (95% CI: 82-99) and 84% (95% CI: 73-92) in the female population and 83% (95% CI: 76-89) and 82% (95% CI: 74-88) in the male population when using FFR as the reference (P = 0.134 and P = 0.936, P-values for comparison between genders). Diagnostic accuracy in females was 92% (95% CI: 85-96) and 86% (95% CI: 81-90) in males when using FFR as the reference. The prevalence of CAD as defined by FFR (<0.8) was 36% in females and 53% in males., Conclusion: Whole-heart dynamic 3D CMR stress perfusion imaging has a high diagnostic accuracy for the detection of significant CAD irrespective of gender and is therefore a suitable non-invasive testing tool to detect myocardial ischaemia in both genders., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.)

Aims: Dynamic three-dimensional-cardiac magnetic resonance (3D-CMR) perfusion proved highly diagnostic for the detection of angiographically defined coronary artery disease (CAD) and has been used to assess the efficacy of coronary stenting procedures. The present study aimed to relate significant coronary lesions as assessed by fractional flow reserve (FFR) to the volume of myocardial hypoenhancement on 3D-CMR adenosine stress perfusion imaging and to define the inter-study reproducibility of stress inducible 3D-CMR hypoperfusion., Methods and Results: A total of 120 patients with known or suspected CAD were examined in two CMR centres using 1.5 T systems. The protocol included cine imaging, 3D-CMR perfusion during adenosine infusion, and at rest followed by delayed enhancement (DE) imaging. Fractional flow reserve was recorded in epicardial coronary arteries and side branches with ≥2 mm luminal diameter and >40% severity stenosis (pathologic FFR < 0.75). Twenty-five patients underwent an identical repeat CMR examination for the determination of inter-study reproducibility of 3D-CMR perfusion deficits induced by adenosine. Three-dimensional CMR perfusion scans were visually classified as pathologic if one or more segments showed an inducible perfusion deficit in the absence of DE. Myocardial ischaemic burden (MIB) was measured by segmentation of the area of inducible hypoenhancement and normalized to left ventricular myocardial volume (MIB, %). Three-dimensional CMR perfusion resulted in a sensitivity, specificity, and diagnostic accuracy of 90, 82, and 87%, respectively. Substantial concordance was found for inter-study reproducibility [Lin's correlation coefficient: 0.98 (95% confidence interval: 0.96-0.99)]., Conclusion: Three-dimensional CMR stress perfusion provided high diagnostic accuracy for the detection of functionally significant CAD. Myocardial ischaemic burden measurements were highly reproducible and allowed the assessment of CAD severity.