EACVI Study on Multimodality Cardiovascular Imaging of Inflammatory Cardiovascular Diseases

Inflammatory Cardiovascular Diseases and Autoimmune Rheumatic diseases (ICARDs) encompass the cardiovascular manifestations of connective tissue diseases and vasculitis, along with primary inflammation affecting all layers of the heart - such as myocarditis, pericarditis and Tako-Tsubo. ICARDs can result in a vast array of clinical cardiovascular manifestations, including coronary artery disease, acute heart failure, ventricular and supra-ventricular arrhythmia. Autoimmune rheumatic disease have been identified as independent risk factors of major cardiovascular events, even after adjusting for traditional cardiovascular risk factors, and accumulated evidence shows increased cardiovascular morbidity and mortality in ICARDs compared to the general population.

Various pathophysiological mechanisms have been proposed to explain the increased risk of cardiovascular events. Suspected mechanisms include prolonged systemic inflammation, accelerated atherosclerosis, autoantibody and immune complex formation, as well as an increase in sympathetic tone which may contribute to heart failure and arrhythmia. Cardiovascular involvement in patients with ICARDs represents a diagnostic challenge due to the heterogeneity in their clinical presentation, and establishing prognosis following cardiovascular involvement is equally difficult. Multimodality cardiovascular imaging, including cardiovascular magnetic resonance (CMR), transthoracic echocardiography and positron emission tomography (PET) nuclear imaging have emerged as useful diagnostic tools to help differentiate inflammatory diseases among themselves and from other diseases, and identify prognostic markers.

Multimodality cardiovascular imaging has the potential to play a major role in assessing the extent of cardiovascular involvement of both primary inflammatory cardiomyopathies and autoimmune rheumatic diseases, by allowing for an early and more accurate diagnosis, phenotypic classification and monitoring. The additive value of cardiovascular imaging, notably MRI, cardiac PET scan, and multimodality imaging in connective tissue disease and vasculitis remains unclear and has been mentioned as a research topic of interest. Moreover, this study will help acquire data on drug tapering, treatment modalities adapted to different etiologies, as well as data on ICARDs unresponsive to treatment. A thorough assessment of the current practices, diagnostic and prognostic value of multimodality imaging in the context of ICARDs has the potential to help shape future guidelines for early assessment of cardiovascular involvement in ICARDs, and potentially change treatment algorithms.

The establishment of this study is particularly pertinent with the forthcoming ESC guidelines on the management of inflammatory cardiomyopathies, as it will provide real-life data on the application of these guidelines. Due to the lack of evidence in the literature on the topic of the diagnostic and prognostic value of multimodality imaging in the context of ICARDs, this "EACVI-INFLAME" international study will aim to determine the proportion of patients with confirmed cardiovascular involvement in patients with suspected or established ICARDs undergoing CMR and/or cardiac PET in a multicentric international cohort.

• Baseline characteristics [Time frame: At each diagnostic assessment point, up to study completion, an average of 1 year.]
• Proportion of patients presenting with the clinical composite outcome at 1year follow-up [Time frame: At each diagnostic assessment point, up to study completion, an average of 1 year.]
• Proportion of patients with all-cause mortality at 1-year follow-up. [Time frame: At each diagnostic assessment point, up to study completion, an average of 1 year.]
• Proportion of patients with cardiovascular mortality at 1-year follow-up. [Time frame: At each diagnostic assessment point, up to study completion, an average of 1 year.]
• Proportion of patients hospitalized for heart failure at 1-year follow-up. [Time frame: At each diagnostic assessment point, up to study completion, an average of 1 year.]
• Proportion of patients hospitalized for an acute episode of ICARD at 1-year follow-up. [Time frame: At each diagnostic assessment point, up to study completion, an average of 1 year.]
• Proportion of patients achieving the remission of ICARD at 1-year follow-up. [Time frame: At each diagnostic assessment point, up to study completion, an average of 1 year.]
• Proportion of participants for whom cardiac imaging led to a guideline-concordant diagnosis, according to the cardio-rheumatology team, and specific to each ICARD category. [Time frame: At each diagnostic assessment point, up to study completion, an average of 1 year.]