- In exploratory analyses at Month 12, among individuals with LGMD2I/R9 (FKRP-related) with elevated HS troponin I at baseline, 100% treated with BBP-418 returned to the normal range by Month 12 versus 40% in the placebo arm (p=0.0248); HS troponin I, a blood marker of heart muscle injury, declined more with BBP-418 than placebo (LS mean difference -17.8 ng/L; 95% CI: -35.1 to -0.5; p=0.0443)

- 54% of BBP-418-treated individuals had stable or improved LVEF at Month 12 versus 25% in the placebo arm (p=0.0262)

- In data presented separately, BBP-418 treatment of LGMD2I/R9 in FORTIFY increased mean glycosylated αDG by Month 3 to the levels of asymptomatic heterozygous FKRP carriers, who do not exhibit disease; this result was sustained through Month 12

- Taken together, these findings support the potential of BBP-418 to act as a disease modifying therapy by addressing the condition at its source

- BBP-418 is under FDA Priority Review with a PDUFA target action date of November 27, 2026

PALO ALTO, Calif., Oct. 05, 2026 (GLOBE NEWSWIRE) -- BridgeBio Pharma, Inc. (NASDAQ:BBIO) ("BridgeBio" or the "Company"), a biopharmaceutical company focused on developing medicines for genetic conditions, today presented new exploratory cardiac data from the 12-month interim analysis of FORTIFY, the Phase 3 clinical trial of oral BBP-418 in individuals living with limb-girdle muscular dystrophy type 2I/R9 (LGMD2I/R9) (FKRP-related). The data were presented in an oral presentation at the 31st annual Congress of the World Muscle Society in Hiroshima, Japan by Volker Straub, M.D., Ph.D. of Newcastle University, UK.