Inflammatory Cardiomyopathy Bern Registry

Overview

Inflammatory cardiomyopathies are associated with inflammation and impaired function of the heart muscle and encompass myo- and pericarditis and cardiac sarcoidosis. Due to the heterogeneity of the clinical manifestations, establishing the diagnosis and prediction of outcome is challenging. Specifically for myocarditis, it is associated with acute and chronic heart failure and sudden cardiac death. Cardiac magnetic resonance imaging (CMR) allows imaging of tissue characteristics (i.e. edema and fibrosis). CMR is the primary diagnostic tool in myocarditis and can also be used for differentiating other inflammatory diseases. Beside the presence of edema, also hyperemia/capillary leak, fibrosis and myocardial function can be assessed and quantified. Previous studies demonstrated the prognostic role of CMR features beyond traditional markers of LV function, but are limited to smaller cohorts and single-center studies. Furthermore, CMR is a rapidly developing modality and as new features of the modality become available, additional research is needed to identify which combination of parameters optimize risk stratification of this heterogenous inflammatory cardiomyopathy. Hence, the goal of the registry is to investigate the diagnostic and prognostic role of clinical techniques in inflammatory cardiomyopathies, particularly CMR, and which combination of features provide the highest potential. This analysis will include new advanced CMR techniques but will also assess the role of other techniques that may be more cost-efficient and more widely available, which could be used as a precursor to CMR imaging exams.

Inflammatory cardiomyopathies, like myocarditis and cardiac sarcoidosis are associated with inflammation of the heart muscle and or pericardium, which is the likely cause of impaired function of the myocardium. Clinical presentation of these diseases present high variability - from asymptomatic cases to patients with cardiac arrest or severe heart failure. Due to the heterogeneity of the clinical manifestations, establishing the diagnosis and prognosis remains challenging. Due to its high spatial resolution and excellent tissue characterization (i.e. the identification of edema and fibrosis), cardiac magnetic resonance imaging (CMR) is the cornerstone in the workup of acute myocarditis and can also be used for differentiating other inflammatory diseases. One of the main criteria for the non-invasive diagnosis of myocarditis has been the Lake Louise Criteria (LLC), where specifically the inclusion of edema and fibrosis as assessed by CMR are necessary to make the diagnosis of myocarditis. The first recommendations released in 2009 indicated 2 of 3 of the following criteria were needed; presence of edema, hyperemia/capillary leak and/or fibrosis. More recently, 2018 updated guidelines are broadened to non-ischemic inflammation to include sarcoidosis, systemic lupus erythematosus and a variety of types of myocarditis. These criteria now include a 2 of 2 criteria with at least one T2-based (edema) and one T1-based (necrosis/fibrosis mostly) criterion, which can be supported by pericardial effusion or systolic LV wall motion abnormalities. Further, CMR has been reported to be a very strong prognosticator. Traditional markers such as late gadolinium enhancement (LGE) and left ventricular ejection fraction (LVEF), assessed in CMR are outcome predictors for future adverse cardiac events in myocarditis patients. Similar findings have been shown in sarcoidosis as well. However, CMR is a rapidly developing modality and as new features of the modality frequently become available, additional research is needed to identify the best combination of parameters for risk stratification in patients with inflammatory. CMR feature tracking (CMR-FT) is a developing contrast-free quantitative method that uses cine images in routinely acquired CMR scanning and it is able to quantify systolic and diastolic myocardial deformation in different orientations. CMR-FT has recently been shown to have diagnostic and prognostic value beyond ejection fraction in patients with coronary artery disease or dilated cardiomyopathy. Recently, the investigators could show in collaboration with the Brigham and Women's Hospital at Harvard Medical School that myocardial strain using CMR-FT provides independent and incremental prognostic value over clinical features, ejection fraction and scar (LGE) in patients with myocarditis. CMR-FT may serve as a novel marker to improve risk stratification in myocarditis. Yet this has not yet been investigated yet with parametric mapping techniques, nor with other inflammatory cardiomyopathies. The goal of the registry is to investigate the diagnostic and prognostic role of clinical techniques in inflammatory cardiomyopathies, particularly CMR, and which combination of features provide the highest potential. This analysis will include new advanced CMR techniques like feature tracking and T1- and T2 mapping but will also assess the role of other techniques that may be more cost-efficient and more widely available, which could be used as a precursor to CMR imaging exams. Further, other imaging modalities such as echocardiography and nuclear imaging (I.e. Positron Emission Tomography) and computed tomography (CT) findings will be assessed and associated with biomarkers and outcome.