Shah Ashish H, Garg Pankaj, Krasuski Richard A
Section of Cardiology, University of Manitoba, Winnipeg, Manitoba, Canada.
Department of Cardiovascular Medicine, University of East Anglia, Norwich, UK.
Ann Pediatr Cardiol. 2025 Mar-Apr;18(2):145-147. doi: 10.4103/apc.apc_92_25. Epub 2025 Aug 29.
The functional integrity of the systemic atrio-ventricular valve (AVV), particularly when tricuspid in morphology, is frequently compromised in patients with a systemic right ventricle (RV), such as those with ccTGA, d-TGA post-Mustard/Senning repair, or Fontan-palliated hypoplastic left heart syndrome. Systemic RV anatomy is associated with adverse long-term outcomes, largely driven by progressive AVV regurgitation and ventricular dysfunction. Unlike the systemic left ventricle (LV), the RV and its associated tricuspid valve have structural and developmental differences that predispose to failure under systemic load. Greater mechanistic understanding is needed to inform prognosis and guide management in this high-risk population.
系统性房室瓣（AVV）的功能完整性，尤其是形态为三尖瓣时，在系统性右心室（RV）患者中常常受损，例如那些患有完全性大动脉转位（ccTGA）、Mustard/Senning修复术后的d-TGA或Fontan姑息性左心发育不全综合征的患者。系统性右心室解剖结构与不良的长期预后相关，这在很大程度上是由进行性房室瓣反流和心室功能障碍驱动的。与系统性左心室（LV）不同，右心室及其相关的三尖瓣存在结构和发育差异，在系统性负荷下易发生功能衰竭。需要更深入的机制理解，以便为这一高危人群的预后提供信息并指导管理。
