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Updated: Oct 28, 2025

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Prevalence and Evolution of Right Ventricular Dysfunction Among Different Genetic Backgrounds in Dilated

Paolo Manca1, Antonio Cannatà2, Vincenzo Nuzzi1

  • 1Department of Cardiology, Azienda Sanitaria Universitaria Integrata Giuliano Isontina, University of Trieste, Trieste, Italy.

The Canadian Journal of Cardiology
|July 17, 2021
PubMed

Insights

Titin (TTN)-related dilated cardiomyopathy (DCM) patients show better right ventricular dysfunction (RVD) recovery than other genetic DCM types. RVD evolution varies by genetic cause, with TTN-related DCM offering a higher chance of RVD improvement.

Area of Science:

  • Cardiology
  • Genetics
  • Heart Failure

Background:

  • Dilated cardiomyopathy (DCM) is a heterogeneous heart muscle disease.
  • Titin (TTN)-related DCM is linked to better left ventricular reverse remodeling.
  • Data on right ventricular dysfunction (RVD) evolution in DCM based on genetic background are lacking.

Purpose of the Study:

  • To investigate the evolution of RVD in DCM patients based on their genetic etiology.
  • To compare RVD recovery and right ventricular fractional area change (RVFAC) in TTN-related DCM versus other genetic DCM.
  • To assess the impact of RVD evolution on clinical outcomes.

Main Methods:

  • Analysis of 104 DCM patients with confirmed genetic variants (51 TTN-related, 53 other) and 139 controls.
  • RVD defined as RVFAC < 35%.
  • Follow-up data at 12-24 months to track RVD and RVFAC changes.

Main Results:

  • RVD was initially present in 29.1% of genetically positive DCM patients without significant differences between groups.
  • At follow-up, only 5.9% of TTN-related DCM patients had residual RVD compared to 35.8% in other genetic DCM (P < 0.001).
  • RVFAC significantly improved in TTN-related DCM but remained impaired in other genetic DCM cohorts.

Conclusions:

  • The evolution of RVD in DCM is heterogeneous and depends on the genetic background.
  • TTN-related DCM is associated with a significantly higher likelihood of RVD recovery compared to other genetic DCM etiologies.
  • RVD evolution did not significantly impact the composite outcome measure after adjustment.
Abstract

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