Prevalence and evolution of right ventricular dysfunction among different genetic backgrounds in dilated cardiomyopathy.

Abstract Background Titin (TTN) related dilated cardiomyopathy (DCM) has a higher likelihood of left ventricular reverse remodeling (LVRR) compared to other genetic etiologies. No data regarding the evolution of right ventricular dysfunction (RVD) according to genetic background is available. Methods Consecutive 104 DCM patients with confirmed pathogenic genetic variants (51 TTN related DCM; 53 other genetic DCM) and a control group of 139 patients with negative genetic testing and available follow-up data at 12-24 months were analyzed. RVD was defined as a right ventricular fractional area change (RVFAC) Results At enrolment, RVD was present in 29.1% of genetically positive DCM without differences between genetic cohorts. At 14 months follow-up, 5.9% of TTN related DCM patients vs. 35.8% of other genetic DCM patients had residual RVD after treatment (p Conclusions The evolution of RVD in DCM is heterogeneous in different genetic backgrounds. TTN related DCM is associated with a higher chance of RVD recovery compared to other genetic etiologies.