BridgeBio reports results from FORTIFY Phase 3 study of BBP-418

BridgeBio (BBIO) Pharma reports positive topline results from FORTIFY, the Company’s Phase 3 pivotal study of BBP-418 in individuals living with limb-girdle muscular dystrophy type 2I/R9. BridgeBio will host an investor call on October 27, 2025. Key results from the planned interim analysis at 12 months include the following: Highly statistically significant increase of 1.8x change from baseline in the primary interim analysis endpoint of glycosylated alpha-dystroglycan observed in the BBP-418 treated group compared to approximately no change in the placebo group from baseline to 3 months; Highly statistically significant increases in glycosylated alphaDG were sustained at 12 months in the BBP-418 treated group; Substantial and highly statistically significant increases in glycosylated alphaDG were observed both in individuals with the L276I homozygous genotype and other FKRP genotypes; Average reduction in serum creatine kinase, a marker of muscle breakdown, of 82% change from baseline in BBP-418 treated individuals; BBP-418 was well-tolerated with no new or unexpected safety findings observed.