Sushma Reddy1, Daniel Bernstein. 1. Department of Pediatrics (Cardiology) and the Stanford Cardiovascular Institute, Stanford University, Stanford, California, USA.
Abstract
PURPOSE OF REVIEW: The right ventricle (RV) is uniquely at risk in many patients with repaired or palliated congenital heart disease (CHD) such as tetralogy of Fallot, corrected transposition, single right ventricle, and in those with pulmonary hypertension. These patients live with abnormal cardiac loading conditions throughout their life, predisposing them to right heart failure. RECENT FINDINGS: Standard heart failure therapies, developed to treat left ventricular failure, have failed to improve function or survival in patients with RV failure, suggesting a divergence in the molecular mechanisms of right versus left ventricular failure. As surgical techniques for repair of the most complex forms of RV-affecting CHDs continue to improve, more children with CHD will survive into adulthood. Long-term survival and quality of life will ultimately depend on our ability to preserve RV function. SUMMARY: The purpose of this review is to highlight the differences between the right and left ventricular responses to stress, our current knowledge of how the RV adapts to the unique hemodynamic stressors experienced by patients with CHD, and the need to better understand the molecular mechanisms of RV failure, providing new targets for the development of RV-specific heart failure therapeutics.
PURPOSE OF REVIEW: The right ventricle (RV) is uniquely at risk in many patients with repaired or palliated congenital heart disease (CHD) such as tetralogy of Fallot, corrected transposition, single right ventricle, and in those with pulmonary hypertension. These patients live with abnormal cardiac loading conditions throughout their life, predisposing them to right heart failure. RECENT FINDINGS: Standard heart failure therapies, developed to treat left ventricular failure, have failed to improve function or survival in patients with RV failure, suggesting a divergence in the molecular mechanisms of right versus left ventricular failure. As surgical techniques for repair of the most complex forms of RV-affecting CHDs continue to improve, more children with CHD will survive into adulthood. Long-term survival and quality of life will ultimately depend on our ability to preserve RV function. SUMMARY: The purpose of this review is to highlight the differences between the right and left ventricular responses to stress, our current knowledge of how the RV adapts to the unique hemodynamic stressors experienced by patients with CHD, and the need to better understand the molecular mechanisms of RV failure, providing new targets for the development of RV-specific heart failure therapeutics.
Authors: Patrick M Cowley; Guanying Wang; Sunil Joshi; Philip M Swigart; David H Lovett; Paul C Simpson; Anthony J Baker Journal: Am J Physiol Heart Circ Physiol Date: 2017-08-19 Impact factor: 4.733
Authors: Patrick M Cowley; Guanying Wang; Philip M Swigart; Anaha Raghunathan; Nikitha Reddy; Pranavi Dulam; David H Lovett; Paul C Simpson; Anthony J Baker Journal: Am J Physiol Heart Circ Physiol Date: 2018-11-09 Impact factor: 4.733
Authors: William T Mahle; Chenwei Hu; Felicia Trachtenberg; JonDavid Menteer; Steven J Kindel; Anne I Dipchand; Marc E Richmond; Kevin P Daly; Heather T Henderson; Kimberly Y Lin; Michael McCulloch; Ashwin K Lal; Kurt R Schumacher; Jeffrey P Jacobs; Andrew M Atz; Chet R Villa; Kristin M Burns; Jane W Newburger Journal: J Heart Lung Transplant Date: 2018-02-16 Impact factor: 10.247