Management of sickle cell disease in patients undergoing cardiac surgery

Abstract

Sickle cell disease is a life-limiting inherited hemoglobinopathy that poses inherent risk for surgical complications following cardiac operations. In this review, we discuss preoperative considerations, intraoperative decision-making, and postoperative strategies to optimize the care of a patient with sickle cell disease undergoing cardiac surgery.

Perioperative care of children with sickle cell disease: A systematic review and clinical recommendations

“…The use of preoperative transfusion is a source of significant controversy. Case‐by‐case decision‐making is the standard of care in the majority of reports . Prospective clinical studies on this question are presented in Table (all primary research in Table S2).…”

“…Some authors advocate for preoperative transfusion without giving a threshold, despite evidence from the abovementioned RCTs. Exchange transfusions (manual and automated) for a leve of HbS <30% is recommended for high risk surgery either preoperatively, or on cardiopulmonary bypass . In cardiac pulmonary bypass, some authors suggest a stricter HbS limit of <5% .…”

“…Intraoperative monitoring varies. Per standard anesthetic care, blood pressure, cardiac rhythm and rate and oxygenation are monitored strictly to avoid hypoxia . Some groups perform more invasive systematic monitoring such as the continuous measurement of central venous pressure, arterial blood pressure, regular arterial blood gas exchange and/or urine output determination (by Foley catheterization) .…”

“…Hydration to avoid hyperviscosity and dehydration is considered essential . Hypothermia is not tolerated . One review suggests that hypothermia remains possibly safe for cardiac and neurosurgical procedures .…”

“…Cardiopulmonary bypass can be done in normothermic or hypothermic conditions . Cold vs warm cardioplegia have been used without consensus. Hemofiltration is frequently used in combination with these procedures .…”