Postoperative Outcomes and Valvular Involvement in Loeys-Dietz Syndrome: A Multicenter Cohort Study of Surgical Risk...

Loeys-Dietz syndrome (LDS) is a connective tissue disorder characterized by significant vascular and valvular abnormalities; however, perioperative outcomes involving aortic and valvular interventions remain underreported. This study aimed to evaluate surgical interventions and postoperative complications in LDS patients. We retrospectively reviewed 94 patients diagnosed with LDS from 2018 to 2024 across Mayo Clinic sites. Data on surgical procedures, postoperative complications, echocardiographic findings, and arrhythmic events were collected. Valve replacements, valve types, and anticoagulation regimens were analyzed. Categorical variables were reported as frequencies and percentages. Ascending aortic aneurysms were present in 77 patients (81.9%), mitral valve prolapse in 33 (35.1%), mitral regurgitation in 36 (38.3%), and tricuspid valve regurgitation in 37 (39.4%). A total of 61 patients (64.9%) underwent at least 1 cardiovascular surgical procedure, most commonly aortic root surgery in 59 patients (62.8%) and aortic valve repair/replacement in 39 patients (41.5%). The median follow-up period from the first surgery to the last follow-up was 6.1 years (IQR = 9.2 years). Major postoperative complications occurred in 32 patients (52.5%), including severe bleeding in 15 patients (24.6%) and neurological events in 6 patients (9.8%). New-onset arrhythmias were documented in 25 patients (41.0%), primarily atrial fibrillation/flutter in 12 patients (19.7%). Among patients with mechanical valves, 21 of 28 (75%) on warfarin experienced bleeding complications. In conclusion, this study highlights the high burden of both aortic and valvular surgical interventions in LDS patients and the associated postoperative risks, reflecting underlying connective tissue fragility. Tailored perioperative strategies are needed, and future studies incorporating LDS genotyping may improve risk stratification and management.