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Cellular and molecular basis of RV hypertrophy in congenital heart disease
  1. D Iacobazzi1,
  2. M-S Suleiman1,
  3. M Ghorbel1,
  4. SJ George1,
  5. M Caputo1,2,
  6. RM Tulloh1,2
  1. 1School of Clinical Sciences, Bristol Royal Infirmary, Bristol, UK
  2. 2Department of Congenital Heart Disease, Bristol Royal Hospital for Children, Bristol, UK
  1. Correspondence to Professor RM Tulloh, Department of Congenital Heart Disease, Bristol Royal Hospital for Children, King David Building, Upper Maudlin Street, Bristol BS2 8BJ, UK; robert.tulloh{at}


RV hypertrophy (RVH) is one of the triggers of RV failure in congenital heart disease (CHD). Therefore, improving our understanding of the cellular and molecular basis of this pathology will help in developing strategic therapeutic interventions to enhance patient benefit in the future. This review describes the potential mechanisms that underlie the transition from RVH to RV failure. In particular, it addresses structural and functional remodelling that encompass contractile dysfunction, metabolic changes, shifts in gene expression and extracellular matrix remodelling. Both ischaemic stress and reactive oxygen species production are implicated in triggering these changes and will be discussed. Finally, RV remodelling in response to various CHDs as well as the potential role of biomarkers will be addressed.

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