Clinical Outcomes of Systemic Tricuspid Valve Replacement in Adult Patients With Congenitally Corrected Transposition of Great Arteries

Background:Congenitally corrected transposition of great arteries (ccTGA), a rare congenital heart disease, is generally asymptomatic during infancy and childhood but can lead to dysfunction of the morphologic right ventricle (mRV) and severe insufficiency of the systemic tricuspid valve (sTV) in adulthood if left untreated. This study aims to evaluate clinical outcomes of systemic tricuspid valve replacement (sTVR) in adult patients with ccTGA.Methods:A total of 18 patients who underwent sTVR at Guangdong Provincial People’s Hospital between Jun 2009 and Sept 2024 were enrolled in this retrospective analysis. Comprehensive clinical data spanning perioperative evaluation through postoperative follow-up were analyzed. All patients experienced a smooth surgery and recovery, and had a median follow-up duration of 35.7 (17.0, 78.1) months.Results:The cohort comprised 12 males and 6 females, with a mean age of 34.5 ± 12.9 years, median height of 165.0 (157.3, 170.0) cm, and mean weight of 55.9 ± 14.0 kg. 13 patients were implanted with an On-X mechanical valve and the remaining received a St Jude Regent (SJR) mechanical valve. Three patients with the On-X developed newly occurring third-degree atrioventricular block (AVB) and accepted permanent pacemaker implantation. One patient underwent heart transplantation owing to heart failure at 63.5 months and two patients with SJR underwent redo tricuspid replacement as a result of prosthetic thrombosis at 63.4 and 131.6 months after the surgery. The remaining patients had no adverse events during follow-up. Mid-term follow-up of echocardiography revealed a significant decrease in right atrial diameter (RAD) (p = 0.034), mRV ejection fraction (mRVEF) (p = 0.018) and morphologic mitral regurgitation index (MRI) at 3 years post-surgery (p = 0.045) and a steady promotion of aortic flow velocity (AFV) (p < 0.05).Conclusions:sTVR is an effective option to address the severe left-sided atrioventricular regurgitation for ccTGA with preserved mRVEF but fails to prevent deterioration of systemic ventricular dysfunction. The On-X mechanical valve suggests a potential trend to avoid prosthetic thrombosis but there was an underlying trend toward third-degree AVB and the necessity for permanent cardiac pacing rather than the SJR.