Division of Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2022;25:2-10. doi: 10.1053/j.pcsu.2022.05.002.
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.
患有全身性右心室（RV）疾病的患者存在心力衰竭和心源性猝死的风险。导致 RV 功能障碍的因素包括来自全身循环的后负荷增加、冠状动脉供血不足、进行性三尖瓣反流、姑息治疗后的残留病变以及心律失常。虽然所有患有全身性右心室（SRV）的患者都容易发生心力衰竭，但房间隔转换术修复的先天性右旋-大动脉转位（d-TGA）、未修复的先天性矫正型大动脉转位（cc-TGA）和接受 Fontan 手术姑息性治疗的单发性全身性右心室患者之间存在明显差异。在此，我们探讨了心力衰竭表型进展的相似点和不同点，以及每种 SRV 的治疗选择的进展和局限性。
