Perioperative challenges in a patient of severe G6PD deficiency undergoing open heart surgery

Abstract

We describe a successful perioperative management of a case of 38-year-old male, presented with chronic jaundice with severe mitral stenosis and moderate tricuspid regurgitation; upon evaluation, he was found to have severe glucose-6-phosphate dehydrogenase (G6PD) deficiency. Usually, patients deficient in G6PD exhibit increased hemolysis and therefore increased need for blood transfusion after cardiac surgery as well as impaired oxygenation in the postoperative period leading to prolonged ventilation. On reperfusion after a period of ischemia, the antioxidant system recruits all of its components in an attempt to neutralize the overwhelming oxidative stress of free radicals, as the free radical scavenging system is deficient in these patients, the chances of free-radical-induced injury is more. Our patient underwent mitral valve replacement and tricuspid annuloplasty under cardiopulmonary bypass with necessary precautions to reduce the formation of free radicals. Treatment was targeted toward the prevention of free radical injury in the G6PD-deficient patient. He had an uneventful intraoperative and postoperative course.

Precautionary Measures for Successful Open Heart Surgery in G6PD Deficient Patient- A Case Report

“…These patients are susceptible to oxidative stress manifesting as haemolysis when exposed to infections, certain precipitating drugs and extracorporeal circuits [6]. During CPB the oxidative stress along with the mechanical trauma from the roller pumps and cardiotomy suction leads to haemolysis, endothelial cell injury and capillary leaks during the post bypass surgery period [7]. Vital organs like lungs and kidneys are at most risk of injury [8].…”

Can Dexmedetomidine Prevent the Unwanted Hemolytic Events in the Glucose-6-Phosphate Dehydrogenase-deficient Person Undergoing Cardiac Surgery?

“…[3] Therefore, one of the most important considerations that anesthesiologists have to contemplate in the management of G6PD deficiency patients is avoiding these stressors and triggering factors in addition to reducing the pain and stress of surgery by selecting the suitable anesthetic and analgesic medications. [4]…”

“…The erythrocytes of a G6PD-deficient patient undergoing cardiac surgery are incapable of scavenging these free radicals, resulting in hemolysis; hence, G6PD deficiency can trigger the destructive effect of free radicals in such patients. [4] Due to the production and accumulation of toxic peroxides, injection of some anesthetic and analgesic agents before, during, and after cardiac surgery can initiate the oxidation of red blood cell membrane and hemoglobin in patients with G6PD deficiency, and thus, these drugs might have the potential to evoke acute episode of hemolysis. [5] As a result, the induction of anesthesia with oxidative agents in G6PD deficiency patients undergoing cardiac surgery multiplies the risk of hemolysis.…”

“…[6] Nevertheless, the evidence regarding the effects of currently used anesthetic and analgesic agents on G6PD enzymatic activity in patients undergoing cardiac surgery is lacking, and selecting the best anesthetic and analgesic agent in these patients is controversial. [47] Consequently, this inconsistency and uncertainty concerning conflicting data may limit the use of analgesics for sedation and pain control in patients with G6PD deficiency.…”

Ultrasound-Guided Regional Anesthesia in a Glucose-6-Phosphate Dehydrogenase (G6PD)-Deficient Geriatric Trauma Patient

“…List of Some Anesthesia and Perioperatively Relevant Drugs Used in Patients With G6PD-Deficient That (1) Can Be Administered Safely, (2) Should Be Avoided, and (3) Are Controversially Discussed in the Literature a 1,3,5,7,8,9,10. …”