Sickle cell disease patients experience a greater risk of post-operative complications following cardiac valve replacement procedures: a retrospective cohort study

Background: Sickle cell disease (SCD) is a condition caused by a mutation of the hemoglobin β-globin chain, producing red blood cells (RBCs) prone to deformation or “sickling”. Patients with SCD are at increased risk of vaso-occlusive crises and perioperative complications, particularly during cardiac surgery due to factors such as hypoxia, hypothermia, and hemodynamic stress. However, data on outcomes following cardiac valve replacement remain limited. This study aims to evaluate postoperative morbidity and mortality in patients with SCD undergoing cardiac valve replacement.

Methods: We performed a multicenter propensity-matched retrospective analysis of patients undergoing initial cardiac valve replacement surgery between January 1, 2009 and December 31, 2023 from over 120 institutions across the United States using the TriNetX database. Patients were stratified into cohorts by SCD diagnosis and matched 1:1 for age, sex, race, and ethnicity. Outcomes were 30-day postoperative complications (morbidity) and mortality.

Results: 453 matched pairs were identified. Patients with SCD had a statistically significant increase in risk of 30-day complications notably, cerebral infarction [risk ratio (RR) =2.47; 95% confidence interval (CI): 1.16–5.28; false discovery rate (FDR) =0.04], sepsis (RR =2.35; 95% CI: 1.47–3.76; FDR =0.003), and pericardial effusion (RR =2.16; 95% CI: 1.25–3.76; FDR =0.02). Other key outcomes such as device complications and heart failure were similar. There was no statistically significant difference in 30-day all-cause mortality between groups (RR =1.08; 95% CI: 0.51–2.27; FDR =0.85).

Conclusions: This multicenter propensity-matched analysis demonstrated that patients with SCD undergoing cardiac valve replacement face increased risk of post-operative complications, notably stroke, sepsis, and pericardial effusion, without a difference in 30-day mortality. These findings suggest that valve replacement is not associated with prohibitive risk in patients with SCD, but rather a distinct complication profile, and may support more informed risk stratification and shared decision making in this unique population.