Right Ventricular Remodeling in Complex Congenital Heart Disease

In congenital heart diseases (CHDs) of moderate to great complexity involving the right ventricle (RV), the morphologic RV can be exposed to significant stressors across the lifespan, either in a biventricular circulation in a subpulmonary or subaortic position or as part of a univentricular circulation. These include pressure and/or volume overload, hypoxia, ischemia, and periprocedural surgical stress, leading to remodeling, maladaptation, dilation, hypertrophy, and dysfunction. In this review we examine the macroscopic remodeling of the RV in various forms of CHD and explore remodeling trajectories, along with the effects of surgeries and residual lesion repair, in tetralogy of Fallot, Ebstein anomaly, congenitally corrected transposition of the great arteries, transposition of the great arteries with atrial switch surgery, and single ventricle palliated by Fontan. In addition, the role of metabolism, genetic markers, and imaging criteria of RV remodeling are explored. Finally, the optimal timing for addressing residual lesions in CHD through surgery or percutaneous interventions is discussed, along with advanced heart failure management strategies and medical therapy aimed at preventing further RV dilation and/or systolic deterioration or promoting reverse remodeling.