The systemic right ventricle: Is the tricuspid valve its “Achilles” heel’?

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.