Modified cardiopulmonary bypass strategy for severe glucose-6-phosphate dehydrogenase deficiency during coronary...

BackgroundSevere glucose-6-phosphate dehydrogenase (G6PD) deficiency presents unique challenges during cardiopulmonary bypass (CPB) because of increased susceptibility to oxidative injury and haemolysis. Published guidance regarding optimal perfusion management remains limited, particularly in patients with a history of severe haemolytic complications and renal dysfunction.TechniqueA 63-year-old male with severe G6PD deficiency, a previous dialysis-requiring haemolytic crisis, chronic renal impairment and residual hemiparesis following a cerebrovascular accident underwent urgent three-vessel coronary artery bypass grafting. A modified CPB strategy was developed to minimise oxidative stress and mechanical haemolysis. Key components included controlled oxygenation (PaO₂ approximately 10 kPa), alpha-stat management, tepid blood cardioplegia, minimisation of cardiotomy suction and blood-air interface exposure, albumin-enriched priming, continuous haemofiltration, and avoidance of oxidant medications, including methylene blue. Oxygen delivery and haematocrit were maintained to support end-organ perfusion and reduce renal hypoxic stress.ResultsCPB duration was 112 min, with an aortic cross-clamp time of 76 min. No clinically significant intravascular haemolysis or haemoglobinuria occurred. Plasma free haemoglobin, bilirubin and lactate dehydrogenase elevations were modest and transient. A single postoperative haemodialysis session was required, after which renal function improved without the need for ongoing renal replacement therapy. The patient was discharged on postoperative day seven without evidence of delayed haemolysis.ConclusionA carefully modified CPB strategy focused on reducing oxidative stress, preventing haemolysis and protecting renal function may facilitate successful cardiac surgery in selected patients with severe G6PD deficiency. This report provides a practical perfusion approach for managing patients at exceptionally high risk of peri-operative haemolytic complications.