Cardiac surgery with cardiopulmonary bypass (CPB) induces extreme disturbances in physiological homeostasis, which may trigger vaso‑occlusive crises and hemolysis in patients with sickle cell disease (SCD). The aim was to describe perioperative management, complications and outcomes in SCD patients undergoing cardiac surgery.

Retrospective study including all adult SCD patients who underwent cardiac surgery with CPB from 1996 to 2021 in a French referral center.

Twenty‑nine procedures were performed in 21 patients aged 36 [28–43] years. Baseline hemoglobin S (HbS) fraction and hemoglobin levels were 67 [56–83] % and 9 [8−9] g/dL, respectively. After preoperative transfusion management, HbS and hemoglobin were 25 [21–29] % and 10 [10−11] g/dL, respectively. Procedures mainly included valvular repair or replacement (n = 23) or heart transplantation (n = 3). Twenty‑six procedures required intraoperative red blood cell transfusion, with 4 [3−6] units administered. Minimum intraoperative temperature, arterial oxygen partial pressure, and hematocrit were 35.0 [34.4–35.8] °C, 192 [169–207] mmHg, and 19 [18–20.5] %, respectively. Maximum lactate level during CPB was 3.5 [2.9–5.1] mmol/L. Four patients developed infectious complications during the ICU stay; no vaso‑occlusive crisis occurred. ICU length of stay was 5 [3–9] days. Three patients died during the ICU course (two after heart transplantation and one after double valve replacement), all from septic shock. One‑year, five‑year and ten-year survival rates were 85.7%, 64.0% and 54.9%, respectively.

Despite the acute stress associated with CPB, adult SCD patients experienced acceptable postoperative complications and showed reasonable long‑term outcomes when managed with careful preoperative HbS optimization and a multidisciplinary approach.