Related Experiment Video

Updated: Aug 24, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
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A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy

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Perioperative Management of Sickle Cell Disease in Complex Congenital Cardiac Surgery: A Compilation of Two Cases

Kunal A Soni1, Vishal V Bhende2, Tanishq S Sharma2

  • 1Cardiac Anesthesiology, Bhanubhai and Madhuben Patel Cardiac Centre, Shree Krishna Hospital, Bhaikaka University, Karamsad, IND.

Cureus
|October 24, 2022
PubMed

Preoperative exchange transfusion is frequently recommended in patients with homozygous sickle cell anemia (homozygous SS) who undergo cardiopulmonary bypass to reduce the concentration of circulated sickle hemoglobin. The information regarding the ideal level of sickle hemoglobin for sickle cell disease (SCD) patients who require surgery is still divergent in the literature. We present the successfully managed cases of two children aged 11 months and three years with homozygous SS who underwent cardiopulmonary bypass for double-outlet right ventricle and cor-triatriatum sinistrum, respectively. In both cases, we performed preoperative blood and exchange transfusion, as well as strict intraoperative invasive monitoring. We also maintained normothermia, avoided hypoxia and acidosis, and offered effective pain management.

Keywords:
cardiopulmonary bypasscortriatriatum sinistrumexchange transfusionintraventricular septal defectsickle cell anemia

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