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Updated: Feb 25, 2026

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Hemoglobin Level at Stage 1 Discharge has No Impact on Inter-stage Growth and Stability in Single Ventricle Infants
Claudia Delgado-Corcoran1, Deborah U Frank2, Stephanie Bodily3
1Department of Pediatrics, Division of Pediatric Critical Care Medicine, School of Medicine, University of Utah, 295 Chipeta Way, P.O. Box 581289, Salt Lake City, UT, 84108, USA. Claudia.Delgado@hsc.utah.edu.
Insights
High hemoglobin levels in single ventricle infants after stage 1 palliation do not improve growth or reduce medical events. Late transfusions to boost hemoglobin showed no benefits and were linked to increased illness severity.
Area of Science:
- Pediatric Cardiology
- Neonatal Medicine
- Congenital Heart Disease Research
Background:
- Infants with single ventricle (SV) physiology often require staged palliation.
- Maintaining high hemoglobin (Hgb) levels is a traditional strategy to optimize oxygen delivery during these stages.
- The benefit of higher Hgb achieved through late transfusions post-stage 1 palliation (S1P) is unclear.
Purpose of the Study:
- To investigate if red blood cell transfusions aiming for higher Hgb levels during the late S1P convalescent phase improve outcomes in infants with SV.
- To assess the association between higher Hgb and improved growth and reduced acute medical events during the interstage period (IS).
Main Methods:
- Retrospective cohort study of 137 infants (<1 year) with SV undergoing S1P.
- Evaluation of data from January 2008 to June 2015.
- Comparison of infants receiving late S1P transfusions versus those who did not, analyzing Hgb levels, growth, and acute events.
Main Results:
- Higher Hgb at S1P discharge was not associated with improved interstage growth (weight/length gain) or fewer acute medical events.
- Late S1P transfusions were associated with greater S1P illness severity and more complex S1P care.
- Median Hgb at S1P discharge was 15.9 g/dL and at S2P admission was 15.3 g/dL.
Conclusions:
- Late transfusions to increase hemoglobin levels in single ventricle infants after stage 1 palliation do not confer benefits for interstage growth or acute events.
- These transfusions may be associated with increased illness severity during S1P.
- Current data suggest that steadily recovering SV infants do not benefit from late transfusions to raise discharge hemoglobin levels.
Abstract:
Hemoglobin levels (Hgb) of infants with a single ventricle (SV) are traditionally maintained high to maximize oxygen-carrying capacity during stage 1 palliation (S1P), stage 2 palliation (S2P), and between stages (IS). A single-center observational cohort study was performed to determine if red blood cell transfusion during the convalescent phase of the S1P (late S1P transfusion) to achieve higher Hgb is associated with benefits during the IS including improved growth and decreased acute medical events. 137 infants <1 year with SV with SIP undergoing care from January 2008 to June 2015 were retrospectively evaluated. 78 (57%) infants received a late S1P transfusion. Median Hgb at S1P discharge was 15.9 g/dL (IQR 14.7-17.1) and median Hgb S2P at admission was 15.3 g/dL (IQR 14-16.3). Median daily weight gain was 22 g/day during IS (IQR 17-26) and median daily length gain was 0.09 cm (IQR 0.06-0.11). Hgb at SIP discharge was not associated with IS growth or fewer IS acute events. However, late S1P transfusions were associated with illness severity at S1P and more complicated S1P care. Our data suggest that SV infants after S1P, who are steadily recovering, do not benefit from late transfusion to raise their hemoglobin level at discharge.
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