Right ventricular function in Marfan patients remains stable despite (multiple) cardiac interventions

Abstract

Abstract Background Left-sided heart lesions, namely aortic root aneurysm, mitral valve prolapse and intrinsic left ventricular (LV) dysfunction are an essential part of the cardiovascular phenotype of Marfan syndrome (MFS). While recent data stresses the need for concomitant tricuspid valve repair (TVR) when addressing left-sided heart lesions in non-MFS patients, data on tricuspid valve disease and right ventricular (RV) function in MFS is scarce. Purpose We sought to determine changes in RV function, tricuspid valve regurgitation and need for TVR in MFS patients throughout their lifetime, from day of first contact. Methods We retrospectively analyzed 183 MFS patients fulfilling Ghent criteria seen at our institution between 1995 and 2019. In this patient population, 136 patients (74%) underwent 170 procedures with cardioplegic arrest. Aortic root surgery was performed in 90% and mitral valve surgery (MVR) in 19% of patients undergoing surgery. TVR was performed in 3.7% of all patients undergoing surgery and 18.5% of patients undergoing MVR. Mean age at time of first intervention was 37.8±14.4 years. Mean age in the group of patients without history of surgery was 20.6±17.5 years. Right ventricular function was evaluated using tricuspid annular plane systolic excursion (TAPSE) by echocardiographic assessment at baseline (defined as most recent exam before surgery, or first available exam in patients without history of surgery), and at last available follow-up (defined as last available exam after surgery, or last available exam prior to subsequent surgery). Mean follow-up (FU) was 7.2±7.1 years. Results Patients that underwent surgery with cardioplegic arrest did not experience a significant decrease in RV function over time (mean TAPSE at baseline 22.3±4.7 mm, mean difference −4.0±6.7 mm at last FU after surgery). The same was true for patients undergoing MVR with a mean TAPSE at baseline of 21.5±5.2 mm and a mean difference of −5.6±8.05 mm at last available FU (p=0.664). Freedom from impaired RV function (defined as TAPSE ≤15mm) at 5-, 10- and 15-years FU was 78%, 77%, 73%, respectively. Freedom from severe TR was 100% up to 15 years, and freedom from TVR at 5-, 10- and 15-years FU was 96%. Actuarial survival of all patients at 5-, 10- and 15-years FU was 95.6%, 94.5%, and 91.8%, respectively. Conclusions Current data suggests that right-sided heart lesions in MFS patients are rare. RV function remains stable over time despite (multiple) cardiac interventions. Furthermore, the need for TVR is low, independently of whether MVR was performed or not. Funding Acknowledgement Type of funding sources: None.