%0 Journal Article %T Right ventricular function in marfan patients remains stable despite multiple cardiac interventions. %A Mihalj M %A Nucera M %A Ferro C %A Mosbahi S %A Haynes A %A Yildiz M %A Heinisch PP %A Schoenhoff FS %J Eur J Cardiothorac Surg %V 0 %N 0 %D 2024 Aug 16 %M 39150778 %F 4.534 %R 10.1093/ejcts/ezae313 %X OBJECTIVE: Mitral valve regurgitation and left ventricular dysfunction are cardiovascular symptoms of Marfan syndrome. There is a paucity of information on tricuspid valve regurgitation and right ventricular function. In patients with Marfan syndrome, we looked at long-term changes in right ventricular function, tricuspid valve regurgitation, and freedom from tricuspid valve repair.
METHODS: Retrospective-observational single-centre analysis on right ventricular function and tricuspid regurgitation in Marfan patients who underwent surgery with cardioplegic arrest between 1995 and 2020. Patients were followed-up from first operation until death, with echocardiographic changes analysed longitudinally. Composite end-point was TAPSE ≤ 16mm, severe tricuspid regurgitation, or tricuspid repair.
RESULTS: The study included 135 patients who underwent 193 operations, 58 of those were reoperations in 40 patients. Median age at first operation was 35 years (IQR 26-46), median follow-up was 8.0 years (IQR 3.0-16.0), and median time-to-first-reoperation was 7.5 years (IQR 3.4-12.5). The composite end-point occurred in 81 observations in 40 patients, mostly as a recurrent event, after median 7.0 years (IQR 1.0-13.0). 10-year-cumulative-incidence for composite end-point was 22.0% (95% CI 15-31), and 9.0% (95% CI 4.4-16) for new-onset TAPSE ≤ 16mm, but no significant change in TAPSE was observed at 10 years. Tricuspid regurgitation was associated with increased risk of annual progression (P < 0.001), but not clinically relevant at 10 years. Actuarial 10-year-survival was 91.1%.
CONCLUSIONS: In Marfan patients with a history of cardiac surgery and subsequent reoperations, the right-ventricular function remains stable. The incidence of severe tricuspid regurgitation and tricuspid repair remain low.