Early Identification and Treatment of Rare Cardiomyopathy Cohorts

Early Identification and Treatment of Rare Myocardium by Multimodal Imaging (EARLY-MYO-RARE)

This study aims to further develop an imaging-guided cohort of rare cardiomyopathies based on the existing database. The investigators will standardize the construction of a cohort that integrates a clinical data repository, serum biobank, myocardial tissue bank, and imaging database. In the current cohort, the investigators will systematically screen for biomarkers indicative of pathological changes in challenging cardiomyopathies. Multidimensional data will be integrated to establish and optimize a heart failure risk assessment model, which will then be validated in a prospective cohort. The effectiveness of the model in assessing different risk groups will be evaluated, with the goal of achieving precise prevention of heart failure from the source.

To answer what are the key clinical questions of patients with rare cardiomyopathies resulted in high risk of adverse outcomes and requiring intensified treatment, this study will systematically refine and expand the cohort. This study will combine multimodal imaging with clinical data, blood samples, myocardial tissue samples to retrospectively identify biomarkers associated with pathological changes in rare cardiomyopathies; thereby to integrate multi-dimensional data to develop and validate a prognostic risk assessment model and evaluate the effectiveness of treatments guided through prospective randomized controlled trials by this model. Ultimately, this study aims to offer an integrated solution for the diagnosis and treatment of rare cardiomyopathies.

Inclusion Criteria:

• Age 18-75 years old.
• Patients preliminarily diagnosed with heart failure and scheduled to receive drug therapy after being evaluated by cardiology departments.
• No history of structural heart disease, and the Framingham score <5 (for patients with the Framingham score ≥5, coronary artery disease will be excluded by coronary angiography/coronary CT/exercise platelet).
• Creatinine clearance ≥50ml/min (Cockcroft-Gault formula).
• LVEF ≥50% assessed by Echocardiography.
• QT interval < 470 ms.
• Providing written informed consent.

Exclusion Criteria:

• Presence of acute/chronic renal impairment (GFR <50/ml/min/1.73m2).
• History of cardiovascular disease such as confirmed coronary artery disease, valvular disease, cardiomyopathy, congenital heart disease, and heart failure.
• Presence of contraindications to CMR.