Ribitol

- Presented cardiac magnetic resonance (CMR) imaging evidence consistent with clinical improvement observed in patients with transthyretin amyloid cardiomyopathy (ATTR-CM) in the ATTRibute-CM Phase 3 study of acoramidis at the American College of Cardiology Annual Scientific Sessions; additional detailed results from ATTRibute-CM are planned for presentation at 2024 medical meetings, including four abstracts accepted for the European Society of Cardiology Heart Failure conference and eleven abstracts accepted for the International Symposium on Amyloidosis in May 2024 - The Company believes there is potential to pursue Accelerated Approval for BBP-418 based on recent interactions with the FDA on the use of glycosylated alpha dystroglycan (αDG) levels as a surrogate endpoint; the FORTIFY Phase 3 trial of BBP-418 in limb-girdle muscular dystrophy type 2I/R9 (LGMD2I/R9) continues to enroll, with full enrollment of the interim analysis population expected in 2024 - BridgeBio secured up to $1.5 billion in a Q1 2024 capital campaign that included a European licensing agreement for acoramidis in ATTR-CM, a Japanese licensing agreement for infigratinib in skeletal dysplasias, a financing in exchange for a 5% royalty on future acoramidis net sales, a credit facility refinancing existing senior secured credit, and primary stock sales - The Company announced the launch of BridgeBio Oncology Therapeutics (BBOT) with a $200 million private financing to accelerate the development of its novel precision oncology pipeline - The Company’s Marketing Authorization Application (MAA) for acoramidis was accepted by review for the European Medicines Agency (EMA), with an expected approval in 2025; this is in addition to the November 29, 2024 PDUFA date set by the U.S. Food and Drug Administration (FDA) for the company’s New Drug Application (NDA) for acoramidis for the treatment of ATTR-CM - The Company ended the quarter with $520 million in cash, cash equivalents, marketable securities and short-term restricted cash PALO ALTO, Calif., May 02, 2024 (GLOBE NEWSWIRE) -- BridgeBio Pharma, Inc. (Nasdaq: BBIO) (BridgeBio or the Company), a commercial-stage biopharmaceutical company focused on genetic diseases and cancers, today reported its financial results for the first quarter ended March 31, 2024, and provided an update on the Company’s operations. “In addition to the important scientific work we have done to advance our patient mission this quarter, we have also taken a first step towards streamlining our firm and thereby unlocking value for patients and investors alike,” said Neil Kumar, Ph.D., founder and CEO of BridgeBio. “We believe the launch of BBOT better aligns investors with an opportunity that was being hidden in our pipeline, and our partnership with these new investors enables us to prosecute these oncology programs more quickly and with higher fidelity. In tandem with our progress in corporate simplification and balance sheet strengthening, we continue to prepare for what we believe will be a world class launch this year in ATTR-CM and we continue to enroll our achondroplasia, ADH1 and LGMD2I/R9 clinical trials, all of which are expected to complete enrollment this year.” BridgeBio’s late stage programs: Acoramidis (AG10) – Transthyretin (TTR) stabilizer for transthyretin amyloid cardiomyopathy (ATTR-CM): The EMA accepted the Company’s MAA for acoramidis for review, with an expected approval in 2025. This is in addition to the Prescription Drug User Fee Act (PDUFA) date of November 29, 2024 set by the U.S. FDA for the Company’s NDA for acoramidis for the treatment of ATTR-CM. In March 2024, the Company entered into a licensing agreement granting Bayer exclusive rights to commercialize acoramidis for ATTR-CM in Europe; in exchange, BridgeBio is entitled to receive among other consideration: Tiered royalties beginning in the low-thirties percent, and Up to $310 million in upfront and near-term milestone payments, as well as additional sales milestones. The Company presented cardiac magnetic resonance imaging evidence from an exploratory substudy where treatment with acoramidis was associated with possible cardiac structural and functional improvement compared with placebo, with potential cardiac amyloid regression. This evidence is consistent with clinical improvement observed in the Company’s ATTRibute-CM Phase 3 study of acoramidis in patients with ATTR-CM. Additional detailed results of the Company’s ATTRibute-CM study are planned for presentation at 2024 medical meetings, including four abstracts accepted for presentation at the European Society of Cardiology Heart Failure conference in May 2024, and eleven abstracts accepted for presentation at the International Symposium on Amyloidosis in May 2024. Low-dose infigratinib – FGFR1-3 inhibitor for achondroplasia and hypochondroplasia: The PROPEL 3 global Phase 3 registrational study of infigratinib in achondroplasia continues to enroll; study completion continues to be anticipated for 2025. The Company anticipates initiating its clinical program for hypochondroplasia in 2024, as part of its commitment to exploring the potential of infigratinib on the wider medical and functional impacts of achondroplasia, hypochondroplasia and other skeletal dysplasias. BBP-418 – Glycosylation substrate for limb-girdle muscular dystrophy type 2I/R9 (LGMD2I/R9): The Company believes there is potential to pursue Accelerated Approval for BBP-418 based on recent interactions with the FDA on the use of glycosylated αDG levels as a surrogate endpoint. FORTIFY, the global Phase 3 registrational trial of BBP-418 in LGMD2I/R9, continues to enroll in the U.S., Europe and Australia. Full enrollment of the interim analysis population is expected in 2024. Encaleret – Calcium-sensing receptor (CaSR) inhibitor for autosomal dominant hypocalcemia type 1 (ADH1): CALIBRATE, the Phase 3 clinical trial of encaleret in ADH1, continues to enroll; the Company anticipates sharing topline data from CALIBRATE in 2025. Recent Corporate Updates: The Company announced the launch of BridgeBio Oncology Therapeutics (BBOT) with a $200 million private financing to accelerate the development of its novel precision oncology pipeline. The $200 million financing was led by Cormorant Asset Management and co-led by Omega Funds, with participation from affiliates of Deerfield Management, GV (Google Ventures), EcoR1 Capital, Wellington Management, Enavate Sciences, Surveyor Capital (a Citadel company), Aisling Capital, Casdin Capital, and Longwood Fund. BBOT will be led by Eli Wallace, Ph.D. as CEO and Pedro Beltran, Ph.D. as CSO; the Board of Directors for BBOT will be chaired by Frank McCormick, Ph.D., David A. Wood Distinguished Professor of Tumor Biology and Cancer Research at UCSF and advisor to the National Cancer Institute’s RAS Initiative at Frederick National Laboratory for Cancer Research. Raymond Kelleher, M.D., Ph.D., Managing Director Cormorant Asset Management, Michelle Doig, Partner, Omega Funds, and Neil Kumar, Ph.D., CEO of BridgeBio will serve as directors. First Quarter 2024 Financial Results: Cash, Cash Equivalents, Marketable Securities and Short-term Restricted Cash Cash, cash equivalents, marketable securities and short-term restricted cash, totaled $519.8 million as of March 31, 2024, compared to $392.6 million of cash, cash equivalents and short-term restricted cash as of December 31, 2023. The $127.2 million net increase in cash, cash equivalents, marketable securities and short-term restricted cash was primarily attributable to net proceeds received from the term loan under the credit facility with Blue Owl of $437.7 million, net proceeds received of $315.3 million from various equity financings, proceeds from the sale of investments in equity securities of $63.2 million, and special cash dividends received from investments in equity securities of $25.7 million. These were primarily offset by refinancing of the Company’s previous senior secured credit and inclusive of prepayment fees and exit-related costs in aggregate of $473.4 million, net cash used in operating activities of $219.5 million and purchases of equity securities of $20.3 million during the three months ended March 31, 2024. Revenue Revenue for the three months ended March 31, 2024, were $211.1 million, as compared to $1.8 million for the same period in the prior year. The $209.3 million net increase for the three months ended March 31, 2024, compared to the same period in the prior year, was primarily due to the recognition of non-refundable upfront payments under the Bayer and the Kyowa Kirin exclusive license agreements. The remaining license and services revenue was recognized in connection with the Navire-BMS License Agreement, which was entered into in May 2022 and for which BMS provided notice of termination in March 2024 with a termination effective in June 2024. Operating Costs and Expenses Operating costs and expenses for the three months ended March 31, 2024 were $210.8 million compared to $128.0 million for the same period in the prior year. The overall increase of $82.8 million in operating costs and expenses for the three months ended March 31, 2024, compared to the same period in the prior year, was primarily due to an increase of $48.1 million in research and development and other expenses (R&D) to advance the Company’s pipeline of development programs, and an increase of $34.7 million in selling, general and administrative (SG&A) expenses mainly to support commercialization readiness efforts. Operating costs and expenses for the three months ended March 31, 2024, include $22.5 million of nonrecurring deal-related costs for transactions that were closed during the period. Restructuring, impairment and related charges for the three months ended March 31, 2024 amounted to $3.4 million. These charges primarily consisted of impairments and write-offs of long-lived assets, severance and employee-related costs, and exit and other related costs. Restructuring, impairment and related charges for the same period in the prior year was $3.4 million. These charges primarily consisted of winding down, exit costs, and severance and employee-related costs. Stock-based compensation expenses included in operating costs and expenses for the three months ended March 31, 2024 were $28.9 million, of which $12.8 million is included in R&D expenses, and $16.1 million is included in SG&A expenses. Stock-based compensation expenses included in operating costs and expenses for the same period in the prior year were $23.5 million, of which $11.8 million is included in R&D expenses, and $11.7 million is included in SG&A expenses. “With our recent equity financing activities, our licensing deal with Bayer for European commercial rights to acoramidis, our royalty funding agreement for $500 million upon FDA approval of acoramidis, and now the private financing of BBOT to advance our oncology pipeline into the clinic, we are in a strong financial position to launch acoramidis in the U.S. at the end of this year and deliver three Phase 3 readouts in 2025,” said Brian Stephenson, Ph.D., CFA, Chief Financial Officer of BridgeBio. BRIDGEBIO PHARMA, INC. Condensed Consolidated Statements of Operations (in thousands, except shares and per share amounts) Three Months Ended March 31, 2024 2023 (Unaudited) Revenue $ 211,120 $ 1,826 Operating costs and expenses: Research, development and other expenses 141,570 93,512 Selling, general and administrative 65,807 31,108 Restructuring, impairment and related charges 3,400 3,369 Total operating costs and expenses 210,777 127,989 Income (loss) from operations 343 (126,163 ) Other expense, net: Interest income 4,075 4,153 Interest expense (23,471 ) (20,121 ) Loss on extinguishment of debt (26,590 ) — Other income (expense), net 9,483 (601 ) Total other expense, net (36,503 ) (16,569 ) Net loss (36,160 ) (142,732 ) Net loss attributable to redeemable convertible noncontrolling interests and noncontrolling interests 944 2,576 Net loss attributable to common stockholders of BridgeBio $ (35,216 ) $ (140,156 ) Net loss per share, basic and diluted $ (0.20 ) $ (0.92 ) Weighted-average shares used in computing net loss per share, basic and diluted 178,705,310 152,645,635 Three Months Ended March 31, Stock-based Compensation 2024 2023 (Unaudited) Research, development and other expenses $ 12,779 $ 11,779 Selling, general and administrative 16,071 11,698 Total stock-based compensation $ 28,850 $ 23,477 BRIDGEBIO PHARMA, INC. Condensed Consolidated Balance Sheets (In thousands) March 31, December 31, 2024 2023 Assets (Unaudited) (1 ) Cash and cash equivalents and marketable securities $ 519,691 $ 375,935 Investments in equity securities - 58,949 Receivables from licensing and collaboration agreements 235,494 1,751 Short-term restricted cash 131 16,653 Prepaid expenses and other current assets 28,108 24,305 Property and equipment, net 11,414 11,816 Operating lease right-of-use assets 8,052 8,027 Intangible assets, net 25,721 26,319 Other assets 20,722 22,625 Total assets $ 849,333 $ 546,380 Liabilities, Redeemable Convertible Noncontrolling Interests and Stockholders’ Deficit Accounts payable $ 4,728 $ 10,655 Accrued and other liabilities 118,248 122,965 Operating lease liabilities 12,841 13,109 Deferred revenue 33,847 9,823 2029 Notes, net 737,392 736,905 2027 Notes, net 543,823 543,379 Term loan, net 434,717 446,445 Other long-term liabilities 595 5,634 Redeemable convertible noncontrolling interests 525 478 Total BridgeBio stockholders' deficit (1,049,528 ) (1,354,257 ) Noncontrolling interests 12,145 11,244 Total liabilities, redeemable convertible noncontrolling interests and stockholders’ deficit $ 849,333 $ 546,380 (1 ) The condensed consolidated financial statements as of and for the year ended December 31, 2023 are derived from the audited consolidated financial statements as of that date. BRIDGEBIO PHARMA, INC. Condensed Consolidated Statements of Cash Flows (Unaudited) (In thousands) Three Months Ended March 31, 2024 2023 Operating activities: Net loss $ (36,160 ) $ (142,732 ) Adjustments to reconcile net loss to net cash used in operating activities: Loss on extinguishment of debt 26,590 — Stock-based compensation 17,057 21,907 Accretion of debt 2,015 2,338 Depreciation and amortization 1,596 1,633 Noncash lease expense 1,069 1,032 Accrual of payment-in-kind interest on term loan — 3,339 Loss on deconsolidation of PellePharm — 1,241 Gain from investment in equity securities, net (8,136 ) (964 ) Other noncash adjustments 1,631 (314 ) Changes in operating assets and liabilities: Receivables from licensing and collaboration agreements (233,743 ) 6,318 Prepaid expenses and other current assets (3,345 ) (3,542 ) Other assets 444 (483 ) Accounts payable (5,927 ) (3,800 ) Accrued compensation and benefits (14,969 ) (18,369 ) Accrued research and development liabilities 11,168 (2,556 ) Operating lease liabilities (1,595 ) (1,250 ) Deferred revenue 24,024 (1,748 ) Accrued professional and other liabilities (1,256 ) (6,372 ) Net cash used in operating activities (219,537 ) (144,322 ) Investing activities: Purchases of marketable securities (44,395 ) — Maturities of marketable securities — 18,000 Purchases of investments in equity securities (20,271 ) (47,474 ) Proceeds from sales of investments in equity securities 63,229 42,287 Proceeds from special cash dividends received from investments in equity securities 25,682 — Payment for an intangible asset (797 ) — Purchases of property and equipment (695 ) (12 ) Decrease in cash and cash equivalents resulting from deconsolidation of PellePharm — (503 ) Net cash provided by investing activities 22,753 12,298 Financing activities: Proceeds from term loan under Financing Agreement 450,000 — Issuance costs and discounts associated with term loan under Financing Agreement (12,254 ) — Repayment of term loan under Loan and Security Agreement (473,417 ) — Proceeds from issuance of common stock through public offerings, net 315,254 143,016 Proceeds from BridgeBio common stock issuances under ESPP 2,364 1,809 Proceeds from stock option exercises, net of repurchases 537 193 Repurchase of RSU shares to satisfy tax withholding (2,936 ) (512 ) Other financing activities — 5,743 Net cash provided by financing activities 279,548 150,249 Net increase in cash, cash equivalents and restricted cash 82,764 18,225 Cash, cash equivalents and restricted cash at beginning of period 394,732 416,884 Cash, cash equivalents and restricted cash at end of period $ 477,496 $ 435,109 Three Months Ended March 31, 2024 2023 Supplemental Disclosure of Cash Flow Information: Cash paid for interest $ 35,315 $ 22,059 Supplemental Disclosures of Noncash Investing and Financing Information: Unpaid issuance costs associated with term loan under Financing Agreement $ 3,732 $ — Unpaid public offering issuance costs $ 513 $ — Deferred and unpaid issuance costs recorded to "Accrued professional and other accrued liabilities" $ 458 $ — Unpaid property and equipment $ 70 $ 96 Transfers to noncontrolling interests $ (1,857 ) $ (2,843 ) Reconciliation of Cash, Cash Equivalents and Restricted Cash: Cash and cash equivalents $ 475,222 $ 407,368 Restricted cash 131 25,503 Restricted cash — Included in “Other assets” 2,143 2,238 Total cash, cash equivalents and restricted cash at end of period shown in the condensed consolidated statements of cash flows $ 477,496 $ 435,109 About BridgeBio Pharma, Inc. BridgeBio Pharma, Inc. (BridgeBio) is a commercial-stage biopharmaceutical company founded to discover, create, test, and deliver transformative medicines to treat patients who suffer from genetic diseases and cancers with clear genetic drivers. BridgeBio’s pipeline of development programs ranges from early science to advanced clinical trials. BridgeBio was founded in 2015 and its team of experienced drug discoverers, developers and innovators are committed to applying advances in genetic medicine to help patients as quickly as possible. For more information visit bridgebio.com and follow us on LinkedIn and Twitter. BridgeBio Pharma, Inc. Forward-Looking Statements This press release contains forward-looking statements. Statements in this press release may include statements that are not historical facts and are considered forward-looking within the meaning of Section 27A of the Securities Act of 1933, as amended (the Securities Act), and Section 21E of the Securities Exchange Act of 1934, as amended (the Exchange Act), which are usually identified by the use of words such as “anticipates,” “believes,” “continues,” “estimates,” “expects,” “hopes,” “intends,” “may,” “plans,” “projects,” “remains,” “seeks,” “should,” “will,” and variations of such words or similar expressions. We intend these forward-looking statements to be covered by the safe harbor provisions for forward-looking statements contained in Section 27A of the Securities Act and Section 21E of the Exchange Act. These forward-looking statements, including statements relating to the clinical and therapeutic, market potential of the Company’s programs and product candidates, including the statements in Dr. Kumar’s and Dr. Stephenson’s quotes regarding the potential commercial launch of acoramidis (if approved), continued advancement in the Company’s pipeline, including enrollments in clinical trials and anticipated readouts, and other benefits resulting from recent financing activities; the statements related to the FDA’s planned actions regarding the Company’s NDA for acoramidis for the treatment of ATTR-CM; the potential outcomes of regulatory reviews by the FDA and the EMA; the timing and success of the Company’s clinical development programs, including the progress of the Company’s clinical development program for acoramidis for patients with ATTR-CM, and the Company’s plan for, and the expected timing of, presenting additional detailed results of ATTRibute-CM study at medical meetings; the potential payments we may receive under the recent license agreement with Bayer; the continuation of PROPEL 3, the Company’s Phase 3 study of infigratinib for achondroplasia and the expected timing for full enrollment in the study; the Company’s commitment to exploring the potential of infigratinib and the expectation and timing of the initiation of the Company’s clinical program for hypochondroplasia; the continuation and progress of FORTIFY, the Phase 3 trial of BBP-418 for LGMD2I/R9, including the ongoing enrollment in the United States, Europe and Australia, the expectation and timing of full enrollment of the interim analysis population, and the potential to pursue Accelerated Approval for BBP-418 based on recent interactions with the FDA; the continued enrollment in CALIBRATE, the Phase 3 clinical trial of encaleret, and the timing for sharing topline data from CALIBRATE; the expectation to accelerate the development of the Company’s novel precision oncology pipeline; and the Company’s financial performance, capitalization status, strategy, business plans and goals reflect the Company’s current views about the Company’s plans, intentions, expectations and strategies, which are based on the information currently available to us and on assumptions we have made. Although the Company believes that its plans, intentions, expectations and strategies as reflected in or suggested by those forward-looking statements are reasonable, we can give no assurance that the plans, intentions, expectations or strategies will be attained or achieved. Furthermore, actual results may differ materially from those described in the forward-looking statements and will be affected by a number of risks, uncertainties and assumptions, including, but not limited to, initial and ongoing data from the Company’s preclinical studies and clinical trials not being indicative of final data, the potential size of the target patient populations the Company’s product candidates are designed to treat not being as large as anticipated, the design and success of ongoing and planned clinical trials, future regulatory filings, approvals and/or sales, despite having ongoing and future interactions with the FDA or other regulatory agencies to discuss potential paths to registration for the Company’s product candidates, the FDA or such other regulatory agencies not agreeing with the Company’s regulatory approval strategies, components of the Company’s filings, such as clinical trial designs, conduct and methodologies, or the sufficiency of data submitted, the continuing success of the Company’s collaborations, the Company’s ability to obtain additional funding, including through less dilutive sources of capital than equity financings, potential volatility in the Company’s share price, the impacts of current macroeconomic and geopolitical events, including changing conditions from hostilities in Ukraine and in Israel and the Gaza Strip, increasing rates of inflation and rising interest rates, on business operations and expectations, as well as those risks set forth in the Risk Factors section of the Company’s most recent Annual Report on Form 10-K and the Company’s other filings with the U.S. Securities and Exchange Commission. Moreover, we operate in a very competitive and rapidly changing environment in which new risks emerge from time to time. These forward-looking statements are based upon the current expectations and beliefs of the Company’s management as of the date of this press release, and are subject to certain risks and uncertainties that could cause actual results to differ materially from those described in the forward-looking statements. Except as required by applicable law, we assume no obligation to update publicly any forward-looking statements, whether as a result of new information, future events or otherwise. BridgeBio Contact: Vikram Bali contact@bridgebio.com (650)-789-8220

- Submitted New Drug Application (NDA) to US Food and Drug Administration (FDA) for acoramidis for the treatment of transthyretin amyloid cardiomyopathy (ATTR-CM) based on positive results of Phase 3 ATTRibute-CM trial, which were published in the New England Journal of Medicine; NDA has been accepted for review with a PDUFA date of November 29, 2024; Marketing Authorization Application (MAA) for acoramidis has also been accepted by the European Medicines Agency (EMA) - Presented additional data from ATTRibute-CM at the American Heart Association Scientific Sessions, demonstrating separation at Month 3 of the placebo and acoramidis time-to-first-event Kaplan-Meier curves for a composite of all-cause mortality (ACM) and cardiovascular-related hospitalization (CVH); separation was sustained through Month 30 and represents the most rapid clinical benefit on the composite endpoint of ACM and CVH in ATTR-CM patients to the Company’s knowledge - Shared positive results of single-arm Phase 3 study of acoramidis in Japanese ATTR-CM patients, including no mortality reported over the 30 month acoramidis treatment period - PROPEL 3, the Company's Phase 3 study of infigratinib for achondroplasia continues to enroll with full enrollment expected in 2024; the Company has also announced a partnership granting Kyowa Kirin exclusive license on infigratinib for skeletal dysplasias in Japan in exchange for an upfront payment of $100 million, royalties up to the high twenties percent, and additional milestone-based payments - FORTIFY, the Company's Phase 3 study BBP-418 for limb-girdle muscular dystrophy type 2I (LMGD2I), continues to enroll, with full enrollment of interim analysis population expected in 2024 - CALIBRATE, the Company's Phase 3 study of encaleret for autosomal dominant hypocalcemia type 1 (ADH1) continues to enroll, with full enrollment expected in 2024 and topline data expected in 2025 - Secured up to $1.25 billion of capital from Blue Owl and CPP investments, including $500 million in cash in exchange for a 5% royalty on future global net sales of acoramidis and a $450 million credit facility from Blue Owl that refinanced existing senior secured credit, extending maturity from 2026 to 2029 subject to certain conditions - Ended the quarter with $393 million in cash, cash equivalents, and short-term restricted cash, and $59 million of investments in equity securities PALO ALTO, Calif., Feb. 22, 2024 (GLOBE NEWSWIRE) -- BridgeBio Pharma, Inc. (Nasdaq: BBIO) (BridgeBio or the Company), a commercial-stage biopharmaceutical company focused on genetic diseases and cancers, today reported its financial results for the fourth quarter and full year ended December 31, 2023, and provided an update on the Company’s operations. “Our focus this year is executing on the launch of acoramidis for patients with ATTR cardiomyopathy,” said Neil Kumar, Ph.D., founder and CEO of BridgeBio. “At the same time, we are also focused on fully enrolling three ongoing Phase 3 clinical trials by the end of 2024. Finally, we hope that reading out potentially exciting data from our Phase 1/2 trial in congenital adrenal hyperplasia later this year will let us take the next step in serving that patient community.” BridgeBio’s key programs: Acoramidis (AG10) – Transthyretin (TTR) stabilizer for transthyretin amyloid cardiomyopathy (ATTR-CM): The Company filed an NDA for acoramidis for the treatment of ATTR-CM with the US FDA; the NDA was accepted for review with a PDUFA date of November 29, 2024. The Company has also filed a Marketing Authorization Application for acoramidis with the EMA, which has been accepted for review.The regulatory filings were based on data from the Phase 3 ATTRibute-CM study, which met its primary endpoint (Win Ratio of 1.8) with a highly statistically significant p-value (p<0.0001). Additional results from ATTRibute-CM include: An 81% survival rate on acoramidis, which approaches the survival rate in the age-matched U.S. database (~85%), and a 0.29 mean annual CVH rate on acoramidis, which approaches the annual hospitalization rate observed in the broader U.S. Medicare population (~0.26);Improvements from baseline observed for a large proportion of participants treated with acoramidis on laboratory and functional measures including n-terminal prohormone of brain natriuiretic peptide (NT-proBNP) and 6-minute walk distance;Rapid clinical benefit on the composite endpoint of ACM and CVH in participants treated with acoramidis, demonstrated by placebo and acoramidis time-to-first event Kaplan-Meier curves for a composite of ACM and CVH that separated at Month 3 and continued to diverge steadily through Month 30 as presented at the American Heart Association Scientific Sessions in November 2023; andAcoramidis was well-tolerated with no safety signals of potential clinical concern identified. The Company also shared positive results of an open-label, single-arm Phase 3 study conducted in Japan by licensing partner Alexion, AstraZeneca Rare Disease, including that no mortality was reported over the 30 month acoramidis treatment period.Additional detailed results of ATTRibute-CM are planned for presentation at 2024 medical meetings. Low-dose infigratinib – FGFR1-3 inhibitor for achondroplasia and hypochondroplasia: In December 2023, the Company announced the dosing of the first child in PROPEL 3, its global Phase 3 registrational study of infigratinib in achondroplasia.In February 2024, the Company announced a partnership with Kyowa Kirin wherein the Company grants Kyowa Kirin an exclusive license to develop and commercialize infigratinib for achondroplasia, hypochondroplasia, and other skeletal dysplasias in Japan; in exchange, the Company will receive an upfront payment of $100 million as well as royalties up to the high-twenties percent on sales of infigratinib in Japan, with the potential for additional milestone-based payments.The Company is committed to exploring the potential of infigratinib on the wider medical and functional impacts of achondroplasia, hypochondroplasia and other skeletal dysplasias, and anticipates initiating its clinical program for hypochondroplasia in 2024. BBP-418 – Glycosylation substrate for limb-girdle muscular dystrophy type 2I/R9 (LGMD2I/R9): FORTIFY, the global Phase 3 registrational trial of BBP-418, continues to enroll in the U.S. with clinical trial sites planned for Europe and Australia. Full enrollment of the interim analysis population is expected in 2024. The Company believes there is potential to pursue Accelerated Approval for BBP-418 based on recent interactions with the FDA on the use of glycosylated αDG levels as a surrogate endpoint. Encaleret – Calcium-sensing receptor (CaSR) inhibitor for autosomal dominant hypocalcemia type 1 (ADH1): CALIBRATE, the Phase 3 clinical trial of encaleret, continues to enroll; the Company anticipates sharing topline data from CALIBRATE in 2025. Recent Corporate Updates: ·Secured up to $1.25 billion of capital from Blue Owl and CPP Investments: The raise includes $500 million in cash from Blue Owl and CPP Investments available upon FDA approval of acoramidis in exchange for a 5% royalty on future global net sales of acoramidis, as well as a $450 million credit facility from Blue Owl that refinanced existing senior secured credit, extending maturity from 2026 to 2029 subject to certain conditions. Fourth Quarter and Full Year 2023 Financial Results: Cash, Cash Equivalents, Marketable Securities and Short-term Restricted Cash Cash, cash equivalents and short-term restricted cash, totaled $392.6 million as of December 31, 2023, compared to cash, cash equivalents, marketable securities and short-term restricted cash of $466.2 million as of December 31, 2022. The net decrease of $73.6 million in cash, cash equivalents, marketable securities and short-term restricted cash was primarily attributable to net cash used in operating activities of $527.7 million and $6.9 million in repurchase of shares to satisfy tax withholdings, primarily offset by net proceeds received of $449.8 million from various equity financings, $6.0 million from stock option exercises, and $3.4 million from common stock issuances under our employee stock purchase plan during the year ended December 31, 2023. Revenue Revenue for the three months and year ended December 31, 2023 were $1.7 million and $9.3 million, respectively, as compared to $1.9 million and $77.6 million for the same periods in the prior year, respectively. The net decreases of $0.2 million and $68.3 million for the three months and year ended December 31, 2023, respectively, compared to the same periods in the prior year, were primarily due to license revenue recognized in 2022 upon the transfer of the license in accordance with the Navire-BMS License Agreement which was entered into in May 2022. Operating Costs and Expenses Operating costs and expenses for the three months and year ended December 31, 2023 were $179.2 million and $616.7 million, respectively, compared to $131.1 million and $589.9 million, for the same periods in the prior year, respectively. The overall increase of $48.1 million in operating costs and expenses for the three months ended December 31, 2023, compared to the same period in the prior year, was primarily due to an increase of $39.3 million in research and development and other expenses (R&D) to advance the Company's pipeline of development programs, an increase of $15.7 million in selling, general and administrative (SG&A) expenses to support commercialization readiness efforts, offset by a decrease of $6.9 million in restructuring, impairment and related charges given that the majority of the restructuring initiatives occurred in the prior year. The overall increase of $26.8 million in operating costs and expenses for the year ended December 31, 2023 , compared to the same period in the prior year, was primarily due to an increase of $55.2 million in R&D expenses to advance the Company's pipeline of development programs, an increase of $7.4 million in SG&A expenses to support commercialization readiness efforts, offset by a decrease of $35.8 million in restructuring, impairment and related charges given that the majority of the restructuring initiatives occurred in the prior year. Restructuring, impairment and related charges for the three months and year ended December 31, 2023, amounted to $0.8 million and $7.9 million, respectively. These charges primarily consisted of winding down, exit costs, and severance and employee-related costs. Restructuring, impairment and related charges for the same periods in the prior year were $7.7 million and $43.8 million, respectively. These charges primarily consisted of impairments and write-offs of long-lived assets, severance and employee-related costs, and exit and other related costs. Stock-based compensation expenses included in operating costs and expenses for the three months ended December 31, 2023 were $37.1 million, of which $22.5 million is included in R&D expenses, and $14.6 million is included in SG&A expenses. Stock-based compensation expenses included in operating costs and expenses for the same period in the prior year were $22.6 million, of which $8.9 million is included in R&D expenses, and $13.6 million is included in SG&A expenses. Stock-based compensation expenses included in operating costs and expenses for the year ended December 31, 2023 were $115.0 million, of which $61.6 million is included in R&D expenses, and $53.4 million is included in SG&A expenses. Stock-based compensation expenses included in operating costs and expenses for the same period in the prior year were $93.8 million, of which $38.0 million is included in R&D expenses, $54.7 million is included in SG&A expenses, and $1.2 million is included in restructuring, impairment and related charges. “Coming off of our recent royalty financing, we find ourselves well capitalized to launch acoramidis this year alongside strong new partners who share our confidence in acoramidis’ potential in the ATTR-CM market,” said Brian Stephenson, Ph.D., CFA, Chief Financial Officer of BridgeBio. “We are excited for this launch, as well as for the continued advancement of our late stage pipeline, which we hope will allow us to serve patients with genetic diseases both directly with the advancement of those medicines towards the market as well as by diversifying our top line revenue and enabling reinvestment into the R&D and business development opportunities that will allow us to be sustainable in the long term.” BRIDGEBIO PHARMA, INC.Condensed Consolidated Statements of Operations(in thousands, except shares and per share amounts) Three Months Ended December 31, Year Ended December 31, 2023 2022 2023 2022 (Unaudited) (Unaudited) (1) Revenue $1,745 $1,870 $9,303 $77,648 Operating costs and expenses: Research, development and other expenses 130,824 91,549 458,157 402,896 Selling, general and administrative 47,583 31,862 150,590 143,189 Restructuring, impairment and related charges 754 7,691 7,926 43,765 Total operating costs and expenses 179,161 131,102 616,673 589,850 Loss from operations (177,416) (129,232) (607,370) (512,202)Other income (expense), net: Interest income 5,578 4,092 18,038 7,542 Interest expense (20,268) (19,990) (81,289) (80,438)Gain from sale of priority review voucher, net — — — 107,946 Other income (expense), net 21,778 4,560 17,370 (7,500)Total other income (expense), net 7,088 (11,338) (45,881) 27,550 Net loss (170,328) (140,570) (653,251) (484,652)Net loss attributable to redeemable convertible noncontrolling interests and noncontrolling interests 2,180 2,979 10,049 3,469 Net loss attributable to common stockholders of BridgeBio $(168,148) $(137,591) $(643,202) $(481,183)Net loss per share, basic and diluted $(0.96) $(0.92) $(3.95) $(3.26)Weighted-average shares used in computing net loss per share, basic and diluted 174,462,332 149,344,380 162,791,511 147,473,076 Three Months Ended December 31, Year Ended December 31, Stock-based Compensation 2023 2022 2023 2022 (Unaudited) (Unaudited) (1) Research, development and others $22,495 $8,941 $61,647 $37,987 Selling, general and administrative 14,638 13,643 53,369 54,669 Restructuring, impairment and related charges — — — 1,172 Total stock-based compensation $37,133 $22,584 $115,016 $93,828 (1)The condensed consolidated financial statements as of and for the year ended December 31, 2022 are derived from the audited consolidated financial statements as of that date. BRIDGEBIO PHARMA, INC.Condensed Consolidated Balance Sheets(In thousands) December 31, December 31, 2023 2022 (Unaudited) (1) Assets Cash, cash equivalents and marketable securities $375,935 $428,269 Investment in equity securities 58,949 43,653 Receivable from licensing and collaboration agreements 1,751 17,079 Short-term restricted cash 16,653 37,930 Prepaid expenses and other current assets 24,305 21,922 Property and equipment, net 11,816 14,569 Operating lease right-of-use assets 8,027 10,678 Intangible assets, net 26,319 28,712 Other assets 22,625 20,224 Total assets $546,380 $623,036 Liabilities, Redeemable Convertible Noncontrolling Interests and Stockholders’ Deficit Accounts payable $10,655 $11,558 Accrued and other liabilities 129,061 106,195 Operating lease liabilities 13,109 15,949 2029 Notes, net 736,905 734,988 2027 Notes, net 543,379 541,634 Term loan, net 446,445 430,993 Other long-term liabilities 9,361 26,643 Redeemable convertible noncontrolling interests 478 (1,589)Total BridgeBio stockholders' deficit (1,354,257) (1,254,617)Noncontrolling interests 11,244 11,282 Total liabilities, redeemable convertible noncontrolling interests and stockholders’ deficit $546,380 $623,036 (1)The condensed consolidated financial statements as of and for the year ended December 31, 2022 are derived from the audited consolidated financial statements as of that date. BRIDGEBIO PHARMA, INC.Condensed Consolidated Statements of Cash Flows(In thousands) Year Ended December 31, 2023 2022 (Unaudited) (1 ) Operating activities: Net loss $(653,251) $(484,652)Adjustments to reconcile net loss to net cash used in operating activities: Stock-based compensation 108,710 91,559 Depreciation and amortization 6,494 6,771 Noncash lease expense 4,032 5,172 Accrual of payment-in-kind interest on term loan 10,207 13,562 Loss on deconsolidation of PellePharm 1,241 — (Gain) loss from investment in equity securities, net (18,314) 8,222 Fair value of shares issued under a license agreement — 4,567 Accretion of debt 8,907 8,570 Fair value adjustment of warrants (984) 1,571 Loss on sale of certain assets — 6,261 Impairment of long-lived assets — 12,720 Gain from sale of priority review voucher, excluding transaction costs — (110,000)Gain from recognition of receivable from licensing and collaboration agreement — (12,500)Other noncash adjustments 181 604 Changes in operating assets and liabilities: Receivable from licensing and collaboration agreements 15,328 15,169 Prepaid expenses and other current assets (2,702) 7,671 Other assets (1,546) 10,971 Accounts payable 2,780 (349)Accrued compensation and benefits 7,802 (2,362)Accrued research and development liabilities (9,855) (4,309)Operating lease liabilities (4,829) (6,245)Deferred revenue (5,438) 15,262 Accrued professional and other liabilities 3,517 (7,729)Net cash used in operating activities (527,720) (419,494)Investing activities: Purchases of marketable securities (29,726) (137,493)Maturities of marketable securities 82,550 479,688 Purchases of investment in equity securities (107,538) (55,562)Sales of investment in equity securities 110,556 52,835 Decrease in cash and cash equivalents resulting from deconsolidation of PellePharm (503) — Payment for intangible asset — (1,500)Proceeds from sale of priority review voucher — 110,000 Proceeds from sale of certain assets — 10,000 Purchases of property and equipment (1,306) (4,821)Net cash provided by investing activities 54,033 453,147 Financing activities: Proceeds from issuance of common stock through Private Placement offering, net 240,796 — Proceeds from issuance of common stock through Follow-on offering, net 144,049 — Proceeds from issuance of common stock through ATM offering, net 64,965 4,852 Transactions with noncontrolling interests (801) — Repayment of term loan — (20,486)Proceeds from BridgeBio common stock issuances under ESPP 3,398 2,558 Repurchase of RSU shares to satisfy tax withholding (6,880) (1,561)Proceeds from stock option exercises, net of repurchases 6,008 666 Other financing activities — 837 Net cash provided by (used in) financing activities 451,535 (13,134)Net increase (decrease) in cash, cash equivalents and restricted cash (22,152) 20,519 Cash, cash equivalents and restricted cash at beginning of period 416,884 396,365 Cash, cash equivalents and restricted cash at end of period $394,732 $416,884 Year Ended December 31, 2023 2022 (Unaudited) (1) Supplemental Disclosure of Cash Flow Information: Cash paid for interest $61,108 $54,443 Supplemental Disclosures of Noncash Investing and Financing Information: Unpaid property and equipment $100 $47 Recognized intangible asset recorded in “Other accrued and other long-term liabilities” $— $11,000 Transfers (to) from noncontrolling interests $(10,534) $(3,512)Payment-in-kind interest added to principal of term loan $— $1,763 Reconciliation of Cash, Cash Equivalents and Restricted Cash: Cash and cash equivalents $375,935 $376,689 Short-term restricted cash 16,653 37,930 Restricted cash — Included in “Other assets” 2,144 2,265 Total cash, cash equivalents and restricted cash at end of periods $394,732 $416,884 (1)The condensed consolidated financial statements as of and for the year ended December 31, 2022 are derived from the audited consolidated financial statements as of that date. About BridgeBio Pharma, Inc.BridgeBio Pharma, Inc. (BridgeBio) is a commercial-stage biopharmaceutical company founded to discover, create, test, and deliver transformative medicines to treat patients who suffer from genetic diseases and cancers with clear genetic drivers. BridgeBio’s pipeline of development programs ranges from early science to advanced clinical trials. BridgeBio was founded in 2015 and its team of experienced drug discoverers, developers and innovators are committed to applying advances in genetic medicine to help patients as quickly as possible. For more information visit bridgebio.com and follow us on LinkedIn and Twitter. BridgeBio Pharma, Inc. Forward-Looking Statements This press release contains forward-looking statements. Statements in this press release may include statements that are not historical facts and are considered forward-looking within the meaning of Section 27A of the Securities Act of 1933, as amended (the Securities Act), and Section 21E of the Securities Exchange Act of 1934, as amended (the Exchange Act), which are usually identified by the use of words such as “anticipates,” “believes,” “continues,” “estimates,” “expects,” “hopes,” “intends,” “may,” “plans,” “projects,” “remains,” “seeks,” “should,” “will,” and variations of such words or similar expressions. We intend these forward-looking statements to be covered by the safe harbor provisions for forward-looking statements contained in Section 27A of the Securities Act and Section 21E of the Exchange Act. These forward-looking statements, including statements relating to the clinical and therapeutic, market potential of our programs and product candidates, including the statements in Dr. Kumar’s and Dr. Stephenson's quotes regarding the potential commercial launch of acoramidis (if approved), continued advancement in our pipeline, including enrollments in clinical trials and anticipated readout, and other benefits resulting from recent financing; the statements related to the FDA's planned actions regarding our NDA for acoramidis for the treatment of ATTR-CM; the potential outcomes of regulatory reviews by the FDA and the EMA; the timing and success of our clinical development programs, including the progress of our clinical development program for acoramidis for patients with ATTR-CM, and our plan for, and the expected timing of, presenting additional detailed results of ATTRibute-CM study at medical meetings; the potential success of our partnership granting Kyowa Kirin an exclusive license on infigratinib for skeletal dysplasias in Japan and the potential payments we may receive under the license; the continuation of PROPEL 3, our Phase 3 study of infigratinib for achondroplasia and the expected timing for full enrollment in the study; our commitment to exploring the potential of infigratinib and the expectation and timing of the initiation of our clinical program for hypochondroplasia; the continuation and progress of FORTIFY, the Phase 3 trial of BBP-418 for LGMD2I, including the ongoing enrollment in the United States, the expectation to enroll in clinical trial sites planned in Europe and Australia, the expectation and timing of full enrollment of the interim analysis population, and the potential to pursue Accelerated Approval for BBP-418 based on recent interactions with the FDA; the continued enrollment in CALIBRATE, the Phase 3 clinical trial of encaleret, and the expectation and timing of full enrollment and sharing topline data from CALIBRATE; the Company’s financial performance, capitalization status, strategy, business plans and goals reflect our current views about our plans, intentions, expectations and strategies, which are based on the information currently available to us and on assumptions we have made. Although we believe that our plans, intentions, expectations and strategies as reflected in or suggested by those forward-looking statements are reasonable, we can give no assurance that the plans, intentions, expectations or strategies will be attained or achieved. Furthermore, actual results may differ materially from those described in the forward-looking statements and will be affected by a number of risks, uncertainties and assumptions, including, but not limited to, initial and ongoing data from our preclinical studies and clinical trials not being indicative of final data, the potential size of the target patient populations our product candidates are designed to treat not being as large as anticipated, the design and success of ongoing and planned clinical trials, future regulatory filings, approvals and/or sales, despite having ongoing and future interactions with the FDA or other regulatory agencies to discuss potential paths to registration for our product candidates, the FDA or such other regulatory agencies not agreeing with our regulatory approval strategies, components of our filings, such as clinical trial designs, conduct and methodologies, or the sufficiency of data submitted, the continuing success of our collaborations, the Company’s ability to obtain additional funding, including through less dilutive sources of capital than equity financings, potential volatility in our share price, uncertainty regarding any impacts due to global health emergencies such as COVID-19, including delays in regulatory review, manufacturing and supply chain interruptions, adverse effects on healthcare systems and disruption of the global economy, the impacts of current macroeconomic and geopolitical events, including changing conditions from hostilities in Ukraine and in Israel and the Gaza Strip, increasing rates of inflation and rising interest rates, on business operations and expectations, as well as those risks set forth in the Risk Factors section of our most recent Annual Report on Form 10-K and our other filings with the U.S. Securities and Exchange Commission. Moreover, we operate in a very competitive and rapidly changing environment in which new risks emerge from time to time. These forward-looking statements are based upon the current expectations and beliefs of our management as of the date of this press release, and are subject to certain risks and uncertainties that could cause actual results to differ materially from those described in the forward-looking statements. Except as required by applicable law, we assume no obligation to update publicly any forward-looking statements, whether as a result of new information, future events or otherwise. BridgeBio Contact: Vikram Bali contact@bridgebio.com (650)-789-8220