BridgeBio Pharma : BBIO Acoramidis (AG10) TTR ISA 2024 Featured research in the ATTR CM landscape

Featured research in the ATTR-CM landscape

May 29, 2024

Forward-Looking Statements and Disclaimer

Statements in this Presentation that are not statements of historical fact are forward-looking statements within the meaning of Section 27A of the Securities Act of 1933, as amended, and Section 21E of the Securities Exchange Act of 1934, as amended. Such forward-looking statements include, without limitation, statements regarding BridgeBio Pharma, Inc.'s (the "Company's") clinical outcomes including survival and hospitalization rates, predictors of improved survival, patient outcomes, anticipated future presentations and manuscript submissions, research and clinical development plans and strategy, prospects, plans, objectives of management, the Company's ability to complete certain milestones, and the timing and success of BridgeBio's clinical trials and development pipeline. Words such as "believe," "anticipate," "plan," "expect," "intend," "will," "may," "goal," "potential," "should," "could," "aim," "estimate," "predict," "continue" and similar expressions or the negative of these terms or other comparable terminology are intended to identify forward- looking statements, though not all forward-looking statements necessarily contain these identifying words. These forward-looking statements are neither forecasts, promises nor guarantees, and are based on the beliefs of the Company's management as well as assumptions made by and information currently available to the Company. Such statements reflect the current views of the Company with respect to future events and are subject to known and unknown risks, including business, regulatory, economic and competitive risks, uncertainties, contingencies and assumptions about the Company, including, without limitation, risks inherent in developing therapeutic products, the success, cost, and timing of the Company's product candidate research and development activities and ongoing and planned preclinical studies and clinical trials, the timing and success of major catalysts across the pipeline anticipated over the next 12 months, the success and timing of preclinical study and clinical trial results, the success of its clinical trial designs, the fact that successful preliminary preclinical study or clinical trial results may not result in future clinical trial successes and/or product approvals, trends in the industry, the legal and regulatory framework for the industry, the success of the Company's engagement with the U.S. Food and Drug Administration ("FDA") and other regulatory agencies, the Company's ability to obtain and maintain regulatory approval for its product candidates, the Company's ability to receive approval for and commercialize its product candidates and any FDA-approved products, the success of current and future agreements with third parties in connection with the development or commercialization of the Company's product candidates, the size and growth potential of the market for the Company's product candidates, the Company's ability to access additional funding upon achievement of portfolio milestones, the accuracy of the Company's estimates regarding expenses, future revenue, future expenditures and needs for and ability to obtain additional financing, the Company's ability to be a sustainable genetic medicine innovation engine and to build the next great genetic medicine company, the Company's ability to obtain and maintain intellectual property protection for its product candidates and approved products, the competitive environment and clinical and therapeutic potential of the Company's product candidates and FDA-approved products, the Company's international expansion plans, preclinical work, overall operations, regulatory review, manufacturing and supply chain interruptions, adverse effects on healthcare systems and disruption of the global economy, and those risks and uncertainties described under the heading "Risk Factors" in the Company's most recent Annual Report on Form 10-K filed with the U.S. Securities and Exchange Commission ("SEC") and in subsequent filings made by the Company with the SEC, which are available on the SEC's website at www.sec.gov. In light of these risks and uncertainties, many of which are beyond the Company's control, the events or circumstances referred to in the forward-looking statements, express or implied, may not occur. The actual results may vary from the anticipated results and the variations may be material. You are cautioned not to place undue reliance on these forward-looking statements, which speak to the Company's current beliefs and expectations only as of the date this Presentation is given. Except as required by law, the Company disclaims any intention or responsibility for updating or revising any forward-looking statements made or contained in this Presentation in the event of new information, future developments or otherwise. No representation is made as to the safety or effectiveness of the product candidates for the therapeutic use for which such product candidates are being studied.

Certain information communicated or contained in this Presentation relates to or is based on studies, publications, surveys and other data obtained from third-party sources and the Company's own internal estimates and research. While the Company believes these third-party sources to be reliable as of the date of this Presentation, it has not independently verified, and makes no representation as to the adequacy, fairness, accuracy or completeness of, any information obtained from third-party sources. In addition, all of the market data included in this Presentation involves a number of assumptions and limitations, and there can be no guarantee as to the accuracy or reliability of such assumptions. Finally, while the Company believes its own internal research is reliable, such research has not been verified by any independent source. Such information is provided as of the date of this Presentation and is subject to change without notice. The Company has not verified, and will not verify, any part of this Presentation, and the Company makes no representation or warranty, express or implied, as to the accuracy or completeness of the information to be communicated in this Presentation or as to the existence, substance or materiality of any information omitted from this Presentation. The Company disclaims any and all liability for any loss or damage (whether foreseeable or not) suffered or incurred by any person or entity as a result of anything contained or omitted from this Presentation and such liability is expressly disclaimed.

The Company is the owner of various trademarks, trade names and service marks. Certain other trademarks, trade names and service marks appearing in this Presentation are the property of third parties. Solely for convenience, the trademarks and trade names in this Presentation are referred to without the ® and TM symbols, but such references should not be construed as any indicator that their respective owners will not assert, to the fullest extent under applicable law, their rights thereto.

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A sincere THANK YOU to patients and families, advocates, physicians,

clinical research staff, and collaborating research partners

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Acoramidis was designed to achieve maximal stabilization and preserve native TTR

Design Objectives

1 Maximize TTR stabilization/minimize toxic monomer

Rationale

• Strong genotype/phenotype correlation between TTR instability and disease severity1
• Dose-dependentimprovements in both TTR stabilization and clinical outcomes demonstrated by tafamidis in ATTR-CM2
• Extent of TTR stabilization or knockdown associated with degree of clinical benefit in ATTR-PN3-6

2 Preserve circulating native	•	TTR has been highly conserved throughout evolution7
TTR	•	TTR is an abundant plasma protein with relatively rapid
		turnover requiring sustained metabolic energy expenditure

We plan to enter the ATTR-CM market with acoramidis, a next generation, potent TTR stabilizer

TTR = Transthyretin; ATTR-CM = TTR amyloid cardiomyopathy.

1Hammarstrom, P et al., PNAS. 2002;99:16427-16432.2Damy, T., et al., Eur J Heart Fail. 2021;23(2):277-285.3Coelho, T. et al., Neurology. 2012;79:785-792. DA. et al., N Engl J Med. 2018;379:11-21.6Benson, M.D., et al., N Engl J Med. 2018;379:22-31.7Richardson SJ, et al. Front Endocrinol. 2015;5:1-9.

4Berk, JL et al , JAMA. 2013;310:2658-2667.	5Adams,	4	4

Connecting the dots: near-complete TTR stabilization leads to improved clinical outcomes

		Evidence of Disease	Clinical and
Genetics	Stabilization	(Activation and	Functional
		Treatment Response)	Outcomes

Protective

T119M

mutation

ACM1,2

Serum TTR

CVM3

CVH4

NT-proBNP

6MWT

KCCQ

1. Maurer et al., ISA 2024 "Early Increase in Serum Transthyretin Level is an Independent Predictor of Improved Survival in ATTR Cardiomyopathy: Insights From Acoramidis Phase 3 Study ATTRibute-CM".
2. Law S, Petrie A, Chacko L, et al. Heart 2022;108:474-478.

3.	Ambardekar et al., ISA 2024 "Acoramidis Treatment-related Increase in Serum TTR is Associated with Lower Cardiovascular Mortality in ATTR-CM: Insights from ATTRibute-CM".	5
4.	Cheng et al., ISA 2024 "Acoramidis Treatment-Related Increase in Serum TTR is Associated with a Lower Risk of Cardiovascular Hospitalization in ATTR-CM Patients: Insights from the ATTRibute-CM Trial".

Patients on acoramidis are surviving more and going to the hospital less

Near-complete TTR stabilization

leads to improved clinical outcomes

A	B	C
Unprecedented	Evidence of	Early separation
absolute	improvement for
in composite
survival and	large proportion
outcomes
hospitalization	of patients
rates

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Patients on acoramidis are surviving more and going to the hospital less

Near-complete TTR stabilization

leads to improved clinical outcomes

A

Unprecedented

absolute

survival and

hospitalization

rates

B			C
Evidence of			Early separation
improvement for
		in composite
large proportion
		outcomes
of patients

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Patients on acoramidis are surviving more and going to the hospital less

Near-complete TTR stabilization

leads to improved clinical outcomes

A		B
Unprecedented		Evidence of
absolute		improvement for
survival and		large proportion
hospitalization		of patients
rates
81-100% absolute survival1,2;		Evidence of disease
42% reduction in frequency of		regression in CMR
composite ACM / CVH events3		imaging substudy4

C

Early separation

in composite

outcomes

3 months to start of

observed CV outcomes

benefit5,6

Higher serum TTR  lower mortality

1. Gilmore J, et al. Efficacy and Safety of Acoramidis in Transthyretin Amyloid Cardiomyopathy. N Engl J Med 2024; 390:132-142. 2. BridgeBio data on file (Acoramidis Ph3 Japan; NCT04622046). 3. ATTRibute-CM event frequency of
ACM and CVH. 4. Razvi et al. Acoramidis may improve cardiac function and promote regression in transthyretin amyloid cardiomyopathy: data from the ATTRibute-CM cardiac magnetic resonance (cmr) substudy; presented at Am Coll	8
Cardiol. 2024. 5. American Heart Association Presentation, BridgeBio - Acoramidis Improves Clinical Outcomes in ATTR-CM: Additional Data from ATTRibute-CM Phase 3 Study; presented Nov 12, 2023. 6. Alexander et al., ISA 2024

"Acoramidis Achieves Early Reduction in Cardiovascular Death or Hospitalization in Transthyretin Amyloid Cardiomyopathy (ATTR-CM): Results from the ATTRibute-CM Clinical Trial".

Acoramidis continues to expand upon its clinically differentiated safety and efficacy profile

Category

Title

First Author1

Congress

Clinical Outcomes

Early Increase in Serum Transthyretin Level is an Independent Predictor of Improved Survival in ATTR Cardiomyopathy: Insights	Maurer, M.	ISA '24
from Acoramidis Phase 3 Study ATTRibute-CM
Acoramidis Treatment-related Increase in Serum TTR is Associated with Lower Cardiovascular Mortality in ATTR-CM: Insights	Ambardekar, A.	ISA '24
from ATTRibute-CM
Acoramidis Treatment-Related Increase in Serum TTR is Associated with a Lower Risk of Cardiovascular Hospitalization in	Cheng, R.	ISA '24
ATTR-CM Patients: Insights from the ATTRibute-CM Trial
Acoramidis Achieves Early Reduction in Cardiovascular Death or Hospitalization in Transthyretin Amyloid Cardiomyopathy	Alexander, K.	ISA '24
(ATTR-CM): Results from the ATTRibute-CM Clinical Trial

8 total	Higher Risk of Mortality in Previously Hospitalized Patients: Insights from ATTRibute-CM	Masri, A.	ISA '24
		Acoramidis Improves Clinical Outcomes in Transthyretin Amyloid Cardiomyopathy	Judge, D.	AHA '23
		(Encore: ISA '24)
		ATTRibute-CM: ITT Sensitivity Analysis and Sub-Analysis Comparing Acoramidis and Placebo in Stage 4 CKD	Poulsen, S.	ESC-HF '24
		(Encore: ISA '24)
		BridgeBio Pharma Shares Positive Results of Single-Arm Phase 3 Study of Acoramidis in Japanese Patients with Transthyretin	--	Press Release
		Amyloid Cardiomyopathy (ATTR-CM) Including No Mortality Reported in the Trial at 30 Months
Quality of	Health-Related Quality of Life in Patients with Symptomatic Transthyretin Amyloid Cardiomyopathy Treated with Acoramidis:	Hanna, M.	ESC-HF '24
Life		An EQ-5D Analysis from the ATTRibute-CM Study		(Encore: ISA '24)
	Improved Health-Related Quality of Life in Acoramidis-Treated Patients with ATTR-CM, Demonstrated by Improvements in	Fontana, M.	ESC-HF '24
2 total	KCCQ Scores	(Encore: ISA '24)
Biomarkers	Acoramidis significantly improves NT-proBNP indices that indicate ATTR-CM disease progression and predict subsequent	Garcia-Pavia, P.	ESC-HF '24
mortality: Insights from the ATTRibute-CM Study	(Encore: ISA '24)
Imaging	Acoramidis May Improve Cardiac Function And Promote Regression In ATTR-CM: Data From The ATTRibute-CM Cardiac	Razvi, Y.	ACC '24
Magnetic Resonance (CMR) Substudy	(Encore: ISA '24)
Prevention	Rationale & Design of ACT-EARLY, the Acoramidis Transthyretin Amyloidosis Prevention Trial	Garcia-Pavia, P.	ISA '24

1Presenters may differ from first author due to presenter availability.

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New updates at ISA: Focus of today's call

• Greater stabilization leads to better clinical outcomes
• • For every 5 mg/dL increase in serum TTR level, the risk of death was reduced by 30.9% by the logistic model and by 26.1% by the Cox proportional hazards model1
• CVH is predictive of overall survival in ATTR-CM over 30 months, with acoramidis demonstrating the earliest time to separation (3 months) on composite clinical outcomes2,3
• • Acoramidis demonstrated unprecedented survival in both those with (62%) or without (87%) previous hospitalization4

1.	Maurer et al., ISA 2024 "Early Increase in Serum Transthyretin Level is an Independent Predictor of Improved Survival in ATTR Cardiomyopathy: Insights From Acoramidis Phase 3 Study ATTRibute-CM
2.	American Heart Association Presentation, BridgeBio - Acoramidis Improves Clinical Outcomes in ATTR-CM: Additional Data from ATTRibute-CM Phase 3 Study; presented Nov 12, 2023.	10
3.	Alexander et. al., ISA 2024 "Acoramidis Achieves Early Reduction in Cardiovascular Death or Hospitalization in Transthyretin Amyloid Cardiomyopathy (ATTR-CM): Results from the ATTRibute-CM Clinical Trial
4.	Masri et al., ISA 2024 "Higher Risk of Mortality in Previously Hospitalized Patients: Insights from ATTRibute-CM";ATTRibute-CM, Data on file