Healing the orphaned heart: heart failure in a patient with glucose-6-phosphate dehydrogenase deficiency

Abstract

SUMMARYPatients with glucose-6-phosphate dehydrogenase (G6PD) deficiency are not represented in clinical trials for heart failure. Moreover, many of the recommended medications can cause haemolysis in this group of patients. We present the case of a 71-year-old woman with G6PD deficiency admitted for acute non-ischemic heart failure with reduced ejection fraction. Our experience showed that a combination of ethacrynic acid and spironolactone is safe and effective for relief of volume overload in this group of patients. Studies are needed to determine whether the morbidity and mortality benefits of established heart failure regimens extend to patients with G6PD deficiency. BACKGROUND

Clinical challenges in the treatment of a patient with decompensated heart failure and glucose-6-phosphate dehydrogenase deficiency (G6PDd)

“…Other loop diuretics are preferred over ethacrynic acid due to its increased likelihood to cause metabolic alkalosis and ototoxicity 20–22. One case report supports the use of ethacrynic acid in the treatment of decompensated patients known with G6PDd 23. Ethacrynic acid is marketed in Denmark, and more studies are needed to support the potential benefits of ethacrynic acid in the treatment of cardiac decompensation in patients with G6PDd.…”

Anesthetic Management in a Patient With Glucose-6-Phosphate Dehydrogenase Deficiency Undergoing Coblation Adenoidectomy With Septoplasty and Turbinectomy

“…During surgery, hematuria is an indicator of an acute hemolytic crisis [ 5 ]. Reaching to the diagnosis is substantial, and once that made, we have to cease the triggering agent and maintaining the urine output by infusing crystalloids as well as administration of non-sulfonamide diuretics [ 5 , 13 ]. Unfortunately, a Foley catheter was not inserted in our case because the surgical duration was short; but we recommend inserting a Foley catheter in such a patient to monitor the urine output regardless of the surgical duration.…”

Drug-induced Hemolysis in G6PD Deficiency: an Unusual Presentation of a Common Clinical Condition

“…Dechallenge confirmed the diagnosis as there was no further intravascular hemolysis, barring the immediate period following drug withdrawal, as determined via the hemoglobin levels, and there was an improvement in the hemoglobin status subsequently despite the continuation of all the other drugs. While furosemide is also known to cause hemolysis in G6PD deficient patients, the improvement in the patient's condition despite the con-tinuation of furosemide rules it out as the precipitating drug (17). A rechallenge was not performed as there was no pharmacological compulsion for continuing with isosorbide dinitrate plus hydralazine and, hence, would be unethical.…”