Clinical Outcomes in Patients with Bicuspid Aortic Valves and Ascending Aorta Dilatation Equal or above 50Mm

Abstract Background Little is known about clinical outcomes in patients with bicuspid aortic valve (BAV) and ascending aortic diameters ≥50 mm where the elective surgical-indication zone begins. Purpose To assess incident aortic dissection (AoD), post-surgical survival and guideline implementation. Methods Multi-center retrospective study of BAV patients (≥18 years) with maximal root or tubular-ascending aortic diameters ≥50mm detected by transthoracic echocardiography at baseline. Ascertainment of aortic surgery, AoD and death was carried-out at their respective institutions. The primary outcome was AoD: “confirmed” AoD by surgery or death certificate, or “possible” AoD defined as sudden cardiac death of unknown cause by death certificate. Secondary outcomes were aortic surgery, post-surgical survival and guideline implementation. Results We included 506 consecutive BAV patients, mean age 61±14 years, 83% men, mean maximal aortic diameter 52±2 mm at baseline, ascending aorta was the most common segment ≥50mm (85%). During a median follow-up of 7.7 years, 356 (71%) underwent elective surgery (89% of class 1 patients). Early-surgery (≤6 months from baseline) occurred in 195 patients and 311 patients remained “under-surveillance”. Surgery under-surveillance (>6 months from baseline) occurred in 161/311 (52%) patients at 2.6 [IQR: 1.5–4.7] years of follow-up. Surgical mortality was 1.4%. Ten-year post-surgical survival was >90% and similar between early-surgery and surgery under-surveillance (p=0.8). Of 8 AoD events, 3 confirmed and 5 possible (all occurring in unoperated patients under-surveillance), 5 events occurred with maximal aortic baseline diameter ≥55 mm and/or >1 year without clinical follow-up; therefore, under guideline non-compliance. Incidence of confirmed plus possible aortic dissection was 0.37% per year with size 50–54mm, 1.13% per year between 55–59mm, and 10.41% per year with size ≥60mm. Aortic stenosis was associated with AoD (p=0.04) and all-cause death in unoperated patients (p=0.001). Conclusions Our results suggest that BAV patients can be safely followed as per guidelines after aortic diameters enter the elective surgical zone (≥50mm), with excellent post-surgical survival regardless of whether surgery is early- or under-surveillance. Appropriate guideline implementation for surgical thresholds and interval clinical follow-up could have prevented most AoD events. Valvular disease guideline should also be followed to prevent AoD and improve survival in these patients. Funding Acknowledgement Type of funding sources: None.