Characteristics and clinical associations of late gadolinium enhanced cardiovascular magnetic tesonance in lamin A/C, cardiac troponin T and myosin binding protein C gene mutation related cardiomyopathies

Background Dilated cardiomyopathy (DCM) is a common cause of heart failure (HF) and the most common diagnosis in patients referred for cardiac transplantation. Genetic studies in DCM have identified mutations in over 30 genes. To date, there is only scarce data regarding the interdependencies between the various genotypes and their impact on cardiac morphology and myocardial tissue appearance. Since non-contrast cardiac magnetic resonance (CMR) is considered the reference method for morpho-functional analysis and late gadolinium enhanced (LGE) CMR method of choice for myocardial tissue assessment, we sought to investigate the characteristics of LGE in asymptomatic and symptomatic carriers of known cardiomyopathy mutations and their clinical associations.