Comparing Long-Term Sequelae of the Systemic Right Ventricle: An Overview of Single Versus Biventricular Arrangements

Patients with systemic right ventricles (RV) are at risk for heart failure and sudden cardiac death. Contributing factors to RV dysfunction include increased afterload from the systemic circulation, coronary insufficiency, progressive tricuspid valve regurgitation, the presence of residual lesions after palliation and arrhythmias. While all patients with a systemic right ventricle (SRV) are vulnerable to heart failure, there are distinct differences between patients with congenital dextro-transpostion of the great arteries (d-TGA) repaired by atrial switch, unrepaired congenitally corrected transposition of the great arteries (cc-TGA) and single systemic right ventricles palliated with a Fontan operation. Herein, we explore both the similarities and differences in progression of heart failure by phenotype as well as both the advancements and limitations in treatment options by each type of SRV.