Truncating mutations in FLNC caused an overlapping phenotype of dilated and left-dominant arrhythmogenic cardiomyopathies complicated by frequent premature sudden death. Prompt implantation of a cardiac defibrillator should be considered in affected patients harboring truncating mutations in FLNC.

No abstract

In this study, HT recipients showed significantly lower values of average radial left ventricle strain, evaluated by means of 2DSTE, with the presence of ACR.

Burden of AS is higher than previously assumed. Degenerative aetiology is the main cause of AS. Most of the patients are elder with high prevalence of significant co-existing valvular disease. LFLG severe AS is present in an important proportion of patients, showing high grade of left ventricle remodelling.

We would like to thank the Reviewers for their thorough review of our manuscript which greatly improved its quality. In the following pages are our point-bypoint responses to each of the comments of the Reviewers. Reviewer's comments: Reviewer #1: The study by Belmonte and colleagues assessed the utility of peripheral miRNAs as biomarkers for DCM management. The authors profiled the expression of 179 miRNAs in the plasma of 254 subjects, including healthy subjects idiopathic DCM patients, ischemic DCM patients, and patients carrying DCM pathogenic variants in LMNA or BAG3 genes. The authors identified a 6-miRNA panel that can distinguish between familial and non-familial DCM, and specific DCM etiologies. Additionally, a separate 5-miRNA panel could discriminate between phenotypically negative carriers and healthy controls. The authors concluded that panels combining miRNAs into panels could serve as a robust biomarker to rule out the presence of pathogenic variants associated with familial DCM in both patients phenotypically positive and negative for the disease Overall, the study is straightforward and well designed. In this manuscript, the authors expanded the scope and findings of one of their recently published studies (J Mol Med (Berl) 2018; 96:845-856). There are several limitations that the authors have discussed.