Outcomes After Surgery for Isolated Rheumatic Aortic Valve Disease in the Young: Preoperative Left Ventricular Dysfunction is a Risk Factor for Increased Mortality

Background: To improve understanding of indications and outcomes for cardiac surgery for rheumatic aortic valvular disease in the young. Methods: Single institution retrospective cohort aged < 18 years with rheumatic heart disease who underwent surgery for isolated rheumatic aortic valve disease between 2000 and 2019. Baseline, intermediate follow-up, and late follow-up data were collected. Left ventricular (LV) dysfunction defined as LV ejection fraction < 55% or LV shortening fraction < 27%. Results: Thirty-nine patients who were 8 to 18 years of age were included (median age 14 years), weighing 27 to 157 kg (median 78 kg) with follow up of 2 to 15 years (median 7 years). Index operations were valve repair 6 of 39 (15%), valve replacement 33 of 39 (84%) with homograft (n = 20), and mechanical valve (13). The 30-day mortality was zero. Overall mortality was 8 of 39 (20%); 17 of 39 (43%) underwent reoperation. At intermediate and late follow up, 27% (7/26) and 53% (18/34) had persistent dysfunction, respectively. Baseline LV dysfunction increased the risk of death (hazard ratio 13.3 [1.52-115.5], P = .003), which increased adjusting for higher body surface area (BSA). All those with baseline weight > 105 kg either died or had LV dysfunction at late follow up ( P = .001). Freedom from late valve-related complications at 5, 10, and 15 years was 72%, 31%, and 23%, respectively. Conclusions: Preoperative LV dysfunction is associated with an increased risk of death for pediatric patients undergoing isolated rheumatic aortic valve surgery. This risk increases further, adjusting for BSA. There was a high rate of reoperations and late complications.