Tricuspid valve surgery in transposition of the great arteries with a systemic right ventricle

Background: Adults with transposition of the great arteries (TGA) and systemic morphologic right ventricle (SRV) have a high prevalence of SRV failure with accompanying tricuspid valve regurgitation (TR). It is unknown if tricuspid valve surgery (TVS) impacts clinical outcomes in this setting. Methods: Data were gathered from an international cohort of patients with TGA and SRV. From this group, patients who had previously undergone tricuspid valve surgery (TVS) were propensity matched with patients who had not undergone surgery and followed for 10.5 ± 4.6 years. The impact of surgery on the probability of the combined endpoint of death/transplant/MCS was investigated. Variables associated with favorable outcomes were explored. Results: Among 2562 patients with TGA and SRV, 140 patients with prior TVS were propensity matched to 140 controls without previous TVS. In the TVS group, primary end point was noted in 20 cases with 15 deaths (10.7 %), 3 transplant listings (2.1 %) and 5 MCS implants (3.6 %). Among controls, primary end point occurred in 23 patients (16.4 %) with 15 deaths (10.7 %), 7 transplant listings (5.0 %) and 2 MCS implants (1.4 %). There was no difference between groups in event free survival. Lower rates of combined endpoint were observed in TVS group with moderate-severe TR and mild-moderate SRV dysfunction. Sensitivity analyses to address potential immortal-time bias were performed in the subset who underwent surgery during the observation period and produced similar results. Among patients with severe SRV dysfunction there was no difference in primary outcome between groups. Similarly, no difference in outcomes was observed between congenitally corrected (ccTGA) and atrial corrected transposition of the great arteries (acTGA) patients. Conclusions: For acTGA and ccTGA patients with SRV and moderate-severe TR, surgical intervention prior to development of severe RV dysfunction is recommended. More data are required to determine those with severe SRV dysfunction who may benefit most.