The vulnerable right ventricle.
OBJECTIVE
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.


RESULTS
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.


CONCLUSIONS
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.
Department of Pediatrics (Cardiology) and the Stanford Cardiovascular Institute, Stanford University, Stanford, California, USA.
Sushma
S
Reddy
Daniel
D
Bernstein
D000293:Adolescent; D006328:Cardiac Catheterization; D006348:Cardiac Surgical Procedures / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D006330:Heart Defects, Congenital / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006333:Heart Failure / Q000401:mortality / Q000503:physiopathology / Q000517:prevention & control*; D006801:Humans; D007223:Infant; D018497:Ventricular Dysfunction, Right / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D016278:Ventricular Function, Right