Disease Progression and Mutation Pattern in a Large Cohort of LGMD R1/LGMD 2A Patients from India.

Variability persists in intraoperative red blood cell (RBC) transfusion rates, despite evidence supporting associated adverse sequelae. We evaluated whether beliefs concerning transfusion risk and safety are independently associated with the inclination to transfuse. We surveyed intraoperative transfusion decision-makers from 33 cardiac surgery programs in Michigan. The primary outcome was a provider's reported inclination to transfuse (via a six-point Likert Scale) averaged across 10 clinical vignettes based on Class IIA or IIB blood management guideline recommendations. Survey questions assessed hematocrit threshold for transfusion ("hematocrit trigger"), demographic and practice characteristics, years and case-volume of practice, knowledge of transfusion guidelines, and provider attitude regarding perceived risk and safety of blood transfusions. Linear regression models were used to estimate the effect of these variables on transfusion inclination. Mixed effect models were used to quantify the variation attributed to provider specialties and hematocrit triggers. The mean inclination to transfuse was 3.2 (might NOT transfuse) on the survey Likert scale (SD: .86) across vignettes among 202/413 (48.9%) returned surveys. Hematocrit triggers ranged from 15% to 30% (average: 20.4%; SE: .18%). The inclination to transfuse in situations with weak-to-moderate evidence for supporting transfusion was associated with a provider's hematocrit trigger (p < .01) and specialty. Providers believing in the safety of transfusions were significantly more likely to transfuse. Provider specialty and belief in transfusion safety were significantly associated with a provider's hematocrit trigger and likelihood for transfusion. Our findings suggest that blood management interventions should target these previously unaccounted for blood transfusion determinants.