BACKGROUND The aim of this study was to investigate the association between red blood cell (RBC) transfusion and haematocrit values with outcomes in infants with univentricular physiology undergoing surgery for a modified Blalock-Taussig shunt. METHODS This study included infants ≤ 2 months of age who underwent modified Blalock-Taussig shunt surgery at the Arkansas Children's Hospital (2006-2012). Infants undergoing a Norwood operation or Damus-Kaye-Stansel operation with modified Blalock-Taussig shunt were excluded. Demographics, pre-operative, operative, daily laboratory data, and post-operative variables were collected. We studied the association between haematocrit and blood transfusion with a composite clinical outcome. Multivariable logistic regression models were fitted to study the probability of study outcomes as a function of haematocrit values and RBC transfusions after operation. RESULTS Seventy-three patients qualified for inclusion. All study patients received blood transfusion within the first 48 hours after heart surgery. The median haematocrit was 44.3 (interquartile range [IQR] 42.5-46.2), and the median volume of RBC transfused was 28 mL/kg (IQR, 10-125) in the first 14 days after surgery. The overall in-hospital mortality rate was 13.6% (10 patients). A multivariable analysis adjusted for risk factors, including weight, prematurity, cardiopulmonary bypass and postoperative need for nitric oxide and dialysis, revealed no association between haematocrit values and RBC transfusion with the composite clinical outcome. CONCLUSIONS We did not find an association between higher haematocrit values and increasing RBC transfusions with improved outcomes in infants with shunt-dependent pulmonary blood flow and univentricular physiology. The power of our study was small, which prevents any strong statement on this lack of association. Future multi-centre, randomised controlled trials are needed to investigate this topic in further detail.