Eliminating left ventricular outlet stenosis lowers the risk for endocardial fibroelastosis recurrence†

Patients with endocardial fibroelastosis (EFE) in the setting of congenital critical aortic valve (AoV) stenosis and left ventricular outflow tract obstruction (LVOTO) are at risk for diastolic dysfunction, limiting biventricular circulation. EFE resection is the only available treatment option, but frequently recurs requiring re-resections. We aimed to investigate whether augmentation of a left ventricular outlet stenosis (AoV stenosis ± LVOTO) with a Ross/Ross-Konno procedure prevents EFE recurrence.

Patients born with AoV stenosis ± LVOTO and treated with primary left ventricular (LV) EFE resection at the study centres from January 2010 to December 2021 were included in the study. The inclusion criteria for this retrospective analysis was the presence or absence of a Ross/Ross-Konno procedure for the treatment of a modifiable risk factor of EFE recurrence. Retrospective allocation to either the non-Ross or Ross/Ross-Konno group was carried out accordingly. The primary outcome measure was EFE recurrence.

Ninety-three patients were screened, and 60/93 patients (64.5%) met all inclusion criteria. Within those 60 patients, 5/23 (20.7%) in the Ross/Ross-Konno group had EFE recurrence compared to 23/37 (62.2%) in the non-Ross group [difference = 40.5%, 95% confidence interval (CI) 14.6-58.7, P =  0.003] and were less likely to develop EFE recurrence with adjusted hazard ratio of 4.07 (95% CI 1.38-12.0, P =  0.011) and 3.69 (95% CI 1.31-10.42, P =  0.014) when including death as a competing event.

This study found that patients after a Ross/Ross-Konno procedure were less likely to experience EFE recurrence and reinterventions on the LVOT/AoV were significantly reduced. However, patient selection and timing of a Ross/Ross-Konno procedure to prevent EFE recurrence have yet to be identified through prospective trials.

IRB-P0038762 approved 4/29/2021 (Boston Children's Hospital) and Ek Nr: 1137/2023 approved 10/25/2023 (Medical Faculty of Johannes Kepler University Linz).