BridgeBio Pharma, Inc.

Good afternoon. I will be your conference operator today. All lines have been placed on mute to prevent any background noise. After the company's remarks, there will be a question and answer session. If you would like to ask a question, press star followed by the number one on your telephone keypad. If you would like to withdraw your question, press star one again. Before we begin, I would like to remind everyone that today's call may contain forward-looking statements within the meaning of the federal securities laws, including but not limited to statements about Bridge Bio's future operating and Financial Performance, Business Plans and Prospects and Strategy. These statements are based on current expectations and assumptions that are subject to risks and uncertainties, which could cause actual results to differ materially from those expressed or implied in these forward-looking statements. For a discussion of these risks and uncertainties, please refer to the disclosure in today's earnings release and BridgeBio's periodic reports and SEC filings. All statements made here are based on information available to BridgeBio as of today. and the company undertakes no obligation to update any forward-looking statements made during this call, except those required by law. With that completed, Bridge Bio, you may begin your conference.

Good afternoon, everyone, and thank you for joining Bridge Bio Pharma's second quarter 2026 earnings call. I am Chinmay Shukla, Senior Vice President, Strategic Finance. With me today are Neil Kumar, our CEO, who will walk through our commercial pipeline and business updates, Matt Outten, our Chief Commercial Officer, who will provide additional detail on a TrueBee and our launch readiness, and Tom Trimarchi, our President and CFO, who will review our financial results. During today's call, we will cover another quarter of consistent growth for a TrueBee, along with new data reinforcing its clinical differentiation, including the first evidence of direct kidney protection in ATTR-CM. We will then turn to the pipeline, where this quarter, All three of our late-stage programs moved from data into being with the FDA, with our first PDUFA date now set for November 27th. And we will review our financial position, including the billion-dollar preferred equity financing completed on July 1 and how it supports the three launches ahead of us. Following our prepared remarks, we will open the call for questions. For the Q&A session, we will be joined by Anant Sridhar, Christine Xu, and Justin To, who lead our programs with Incalaret, BBP418, and Infogratnet, respectively. With that, I'll turn it over to Neil.

Thanks, Chinmay, and thanks, everyone, for joining today. As always, these calls are where we communicate relevant aspects of our business to investors, and so we welcome your questions and feedback. In sessions past, we've had occasion to marry comments on the portfolio with comments regarding financing and strategy. Today, however, I want to focus entirely on the portfolio itself and the progress being made across research, development, and commercial. I'm going to do so because I believe, as I hope you might appreciate at the end of my somewhat lengthy comments, that this is an important transition point for Bridge Bio, one in which, if we continue executing at a high level, sets us up well for delivering substantial returns for patients and investors alike. Put more simply, It feels like we're at T equals zero in BridgeBio's next chapter. I don't say this glibly, but rather due to the following and overlapping advances. First, as we will discuss, the combination of learnings from CardioTransform, our own unique kidney protective data, and extraordinary real-world evidentiary results come together to provide the basis of what I'm calling Launch 2.0 for Atruvi. I believe we will start to see significant commercial fruit from this in the six to nine-month range and beyond, judging from analogs. We think the market is shaping up to be a stabilizer-first market with a constrained number of competitors and one in which we have increasing numbers of proof points that our near-complete stabilizer is superior to Pfizer's partial stabilizer. Second, all three NDAs for LGMD2I, ADH1, and achondroplasia have been submitted, with LGMD2I and ADH1 garnering priority review and are hoping that achondroplasia might too. Our commercial readiness work is on track, even ahead of what we were able to do with ATTR cardiomyopathy, given our relatively lean resourcing at the time, to deliver strong launches. Third, our chronic hypoparathyroid phase three, which we believe is overlooked, has commenced and will read out in the next 18 months, with potential to provide a differentiated efficacy and safety profile, as we will discuss, in addition to being the only oral in the space. Finally, we anticipate novel trials in areas like Turner and hypochondriplasia for infragradinib, a new trial in a to-be-disclosed high-putinuric orphan kidney disease for acramidus, and the advancement of a potentially best-in-class TTR antibody into the clinic in the coming 12 to 18 months. All of this activity together provides the substrate for well over $10 billion in risk-adjusted revenue, with $8 billion of that being post-Phase III today. In addition, our interest and earlier but still advanced genetic medicine R&D within our gondola pipeline continue to bear fruit. So this is a company with no dearth of pragmatic ideas that can drive a continued flux of important medicines on a risk-adjusted basis for the next decade or more to come. I'll begin my portfolio comments with a Truby. First and most importantly, we observed continued commercial momentum this quarter with a Truby being the fastest growing brand in the space at 23%. and this growth does not account for the impacts of CardioTransform, our kidney data, and most of the real-world evidence data to date since that occurred after the quarter end. We've always said that the most important thing commercially and medically in this whole space is diagnosing new patients. To that end, we were heartened to see the substantial overall market growth of 19% this quarter, representing a 51% increase year on year and substantially outstripping the market growth observed in the last three quarters. Consistent with these numbers is the growth in frontline patients where stabilizers have dominated share, a trend that we think will strengthen as we learn more from CardioTransform's important results. Indeed, we observed a slight downtick in numbers of second-line patients in the second quarter. We believe our share in frontline has grown some two to three percentage points, although it's hard to tell precisely given some of the inventory dynamics from our competitor Pfizer. Our growth also remains within the 30 to 40% that we have indicated previously. Going forward, we expect that the first-line market will continue to grow, and we intend to continue growing our share in it, which should translate into continued steady sales growth. Atruvi's strongest tailwind, however, is its continually growing clinical differentiation story, driven for the most part by the expanding body of real-world evidence, as well as the now-documented renal protective effect. In July of this year, We published in suric heart failure on acramidus driving the first ever early and sustained direct kidney protective effects in ATTR cardiomyopathy, including chronic EGFR slope improvement and urinary albumin to creatinine ratio reduction. The upshot of this is that a truby may protect the heart and the kidney simultaneously in ATTR patients. A hemodynamically mediated effect, which we do not observe with other ATTR cardiomyopathic medicines, either knockdowns or other stabilizers. Critically, as pointed out in the paper, the dynamics of this effect mirror the early separation uniquely observed with Atruvi in terms of clinical outcomes, helping to explain this early impact. Furthermore, and intriguingly, the magnitude of the acute dip in EGFR on Atruvi is actually important and suggestive of downstream benefits. More specifically, comparing acramidus versus placebo subgroups with acute EGFR dips greater than or equal to the median of 4.89 mL per meter per 1.73 m2 favored acramidus for all-cause mortality or cardiovascular-related hospitalization with a whopping hazard ratio of 0.42 with an associated p-value of 0.006. and cardiovascular related hospitalization alone with a similarly impressive hazard ratio of 0.34 with an associated p-value of 0.002. Intriguingly, within the placebo arm, EGFR dips portended worse outcomes. So something initially thought to be a crutch has now been shown to be an important differentiator for our product. The observed effect compares favorably to what we see in other kidney protective cardiac treatments like SGLT2 inhibitors. In a recently held meeting of nephrologists and cardiologists, one KOL explained to me, it looks like you have a kidney drug here. Building on that, as referred to above, we intend to further interrogate this signal by conducting clinical studies in an orphan kidney indication. More information on that in the weeks to come. Meanwhile, the generation of real-world evidence continues apace. When one looks at analogs in the cardiovascular space where double-blind head-to-heads were not immediately possible, real-world evidence sets the bedrock of ultimate commercial outperformance. The most storied of these analogs is likely the Eliquis Xeralto marketplace. Calling back to last quarter, there was an independent propensity score matched analysis presented at SCAI and since published, which continues to resonate with physicians. That analysis associated Atruvi with a 37% reduction in composite cardiovascular events and a 34% reduction in hospitalizations at six months relative to defamitis, with an effect deepening at nine months. Remarkably, there was no observed clinical outcome that did not favor Atruvi versus Vindamax in all measures except for dizziness and syncope reached statistical significance of less than 0.01 with an N just shy of 600 patients. Building on this data, we have our own now soon to be published and available online today preprint analysis that parenthetically has been downloaded more than 400 times now, showing again a true VL performance as compared to Vindimax. Importantly in this study, a 34% reduction in diuretic intensification, heart failure, hospitalization, and mortality was observed, again, statistically significantly. And separation is again observed as early as 30 days and continues to improve over time. These types of analyses are what the community has been asking for. Importantly, a large-scale independent EHR-based analysis will be coming at HFSA. Our hope is that Truby continues to perform well there and that then these several RWE studies will form the basis for decision making and guideline updates. The growing body of research supporting a Truby's clinical differentiation will take place alongside evidence from other studies in this rapidly evolving field of ATTR cardiomyopathy. Last month, as you all know, the top line results for Cardiotransform studying of Plontersen in ATTR cardiomyopathy read out and the study did not meet its primary efficacy endpoint with no benefit observed with combination therapy. At this point, we mostly want to acknowledge that this is a blow to the patients who participated in the trial and their families and the investigators, and we feel for them as part of the ATTR cardiomyopathy community. The case for combination therapy seems today null from a trial data perspective. Given the similar degrees of knockdown between Oplontersen and Vitriceran, we'll be interested to see how the knockdown performs in two settings. Number one, does the monotherapy relative risk reduction continue to underperform what we observed from Atruvi at 30 months? And two, does monotherapy knockdown actually not outperform a partial stabilizer in defamitis as we actually observed in Helios B? Recall, of course, that in addition to the real-world evidence I just cited, everywhere we looked in our attribute trial, acaramidase outperformed defamidase. The conclusions of this important study run by AstraZeneca and Ionis, we believe, will likely reinforce the case for stabilizers first. And if the monotherapy benefit, again, lags in time, as is observed with vitriceran and in magnitude of effect as compared with Atruvi, we believe this begins to make an even stronger case for using Atruvi first in the second line setting. Now I'd like to discuss the three pipeline programs that have moved into regulatory review this quarter and which we are preparing to launch. For BBP418, our LGMD2I program, the FDA accepted our NDA on May 27th with priority review. The PDUFA date is November 27th, 2026, and there is no advisory committee planned. We continue to have positive interactions with the agency. This is in line to be the next approval in our portfolio, and it would be the first approved therapy for LGMD2I, a devastating condition affecting a little more than 1,000 patients in the U.S. alone with significant unmet need. There's really no displacing credible competition in this space with gene therapy really the only other pipeline approach, and it suffers from safety and efficacy issues coupled with the fact that too much FKRP is toxic, so dosing might well be an issue. I'll remind everyone as well that the data generated by our program are easily the most profound ever in the LGMD space and perhaps the broader muscular dystrophy space, given that biochemical improvements tied strongly to functional and statistically significant improvements in ambulation, breathing, and other outcomes, and that the drug promoted improvements as opposed to the ever worsening observations on placebo. From a clinical perspective, our goals are number one, to educate broadly on already established data, and two, to reinforce our observations in the non-ambulatory and severe patient population that may initially be reluctant to try anything. Recall, we observed remarkably consistent benefit in our trial across ages, degree of severity, and the homozygous and compound heterozygous populations. Building on that, we'll be analyzing whether our established functional impacts also marry with some cardiovascular benefit, which affects many patients on the severe end of the spectrum. Our plan is to cut that data and present the results at World Muscle Society in late September, early October, so we are hopeful for a good outcome for the patients we serve there. As we prepare for launch, our neuromuscular commercial and medical field teams are hired, trained, and in the field, and Market Access is engaging with payers in a pre-approval information exchange. There are approximately 500 genetically confirmed patients today in the United States, with many who remain unidentified and misclassified within the broader LGMD or Becker muscular dystrophy space. Our goal is to find every patient who can benefit and be ready the moment we're able to reach them. Turning to a calorie for EH1, the FDA accepted our NDA on July 22nd with a PDUFA target action date of May 8, 2027, and no advisory committee planned. At the end of July, The agency granted priority review, and we have announced that today. We have also submitted our MAA to the EMA on July 27th, and it is under review. Incalerate would be the first therapy approved for ADH1 in both the United States and EU, and we are excited to serve this patient population. Speaking of that population, our patient finding efforts continue. and more than 2,200 patients have been identified in the ICD-10 claims between October 2023 and June 2026. That is an increase of about 300 since the first quarter and it's been driven by genetic testing, awareness education, use of the ICD-10 code and BridgeBio supported family testing events. We have also completed enrollment in the first of four cohorts in our pediatric ADH1 study and are preparing to open cohort two. But ADH-1 approval is the beginning of incalorant potential, not the end. Chronic hyperparathyroidism affects some 200,000 patients in the US and EU, a blockbuster opportunity in and of itself, whereas discussed last quarter, we see a real appetite for an oral option that corrects both hypocalcemia and hypercalciuria. I want to spend a minute on this opportunity because I think it's been overlooked significantly by investors. There may be a belief that PTH replacement is the beginning and end of the game here, with advances around dosing, for instance, going from daily to weekly, being the only salient dynamic for patients. But that overlooks a couple key facts. First, that benefits of existing therapy do not importantly extend to normalization of urine calcium, with some 40% of patients not normalizing and some 50% of CHP patients actually being hypercalciuric. There's a well-documented decrease in efficacy of PTH replacement over time, suggesting that other approaches may be important here. Third, perhaps most importantly, there is a need for a drug that spares the impact of PTH-mediated bone issues, especially considering that in a recent survey of 160 patients, 48% of them had osteoporosis or osteopenia. And fourth, that many individuals would prefer an oral medicine. I think some may have discounted this opportunity based on likely probability of technical success. That, I believe, is a mistake. First, the pathomechanism here is well described. Recall first that the hypercalciuria in chronic HP arises from three independent contributors. One, loss of calcium reabsorption at the distal nephron, that's PTH driven. Second, decreased calcium reabsorption in the thick ascending limb, that's calcium-sensing receptor driven. And third, obviously, exacerbation by conventional therapy. Analogous to PTH activity in the kidney to mediate reabsorption of calcium, Encalerate's action on the calcium sensing receptor has been shown to increase paracellular reabsorption of calcium in the thick ascending limb by reducing Claudin-14 expression, which in turn decreases the amount that integrates into the Claudin-16-19 complex, which acts as a calciuria-promoting pore blocking component. This mechanistic rationale helps to explain the observation from our proof of concept phase two, where 80% of post-surgical hyperthyroid patients administered within Calerate achieve both normal blood and urine calcium within five days. Okay, so we understand how negative allosteric modulation of the calcium tending receptor can mechanistically raise serum and lower urine calcium, even in a wild-type setting. But for those of you who don't want to bet on mechanism, recall also there's clinical evidence in the wild-time setting that exists for these drugs. Namely, the extensive data from the legacy clinical development program of Encallerate in osteoporosis participants expressing wild-type calcium-sensitive receptor like the chronic hyperparathyroidism population that we intend to study in the RECLAIM HP trial. And recall that in that osteoporosis study, The drug demonstrated dose-proportional increases in serum calcium at daily doses of 15 milligrams or above. So, we believe, given the endpoints of serum and urine calcium normalization, with all that we've seen and know, and the stability of those endpoints statistically, that we have a high probability of technical success trial on our hands. Secondly, investors may believe that the opportunity is not near term. But this is a relatively quick trial given the aforementioned endpoints and the rapidity of onset of our drug. And as mentioned in our press release, we have already activated our first sites for the reclaimed trial, our global phase three, and have begun screening with SPI imminent and a trial readout expected in the next 18 months. Okay, finally, I'll come to infogratinib, our oral treatment for achondroplasia, where we presented our phase three Propel 3 results at the International Conference on Children's Bone Health on June 30th. and simultaneously published them in the New England Journal of Medicine, the only achondroplasia program with phase three results in the New England Journal. Following that publication, I'm excited to announce we've submitted our NDA and we are targeting an M&A submission in Q4 of this year. We hope to see NDA acceptance and ideally priority review in Q4 of 2026 with approval following in mid 2027. Approval would make infogratinib the first FGFR3 targeted are all therapeutic for achondroplasia. And on top of its oral dosing advantage, it remains the only therapy with efficacy measures beyond annualized high velocity demonstrated in a placebo controlled setting at 52 weeks, including proportionality. Adding to this, we demonstrated a clear functional differentiator in our phase three results with a statistically significant 0.37 Standard Deviation Improvement on arm span with a p-value of less than 0.0001. This is the first-ever placebo-controlled arm span benefit in an achondroplasia trial. We look forward to presenting more data in the second half of this year and continuing to build infragratinib's scientific story through the pre-approval period. On the commercial front, our regional sales directors and medical affairs personnel are onboarded, and the field medical team is fully built. Our RSDs are building teams for meaningful share of voice in a market where two competitors are already present and where we see a real gap, especially in the U.S., between kids confirmed to have achondroplasia and those on treatment. We continue to think our peak achievable share in this space is above 65%. Finally, I also want to make mention of the critical work occurring off our balance sheet at Gondola Bio, where Bridge Bio shareholders retain exposure via our ownership stake and ongoing operational support. Our program in EPP announced positive Phase 2a data in June, and following a productive EOP2 meeting with the agency, we will be initiating a Phase 2b3 study in Q3 of this year. Critically, given the 80 plus percent magnitude of PP9 reduction, coupled with the quick onset of action and safe profile, the agency suggested that the 2b could form the basis of registration if PP9 lowering was met statistically and other functional trends lined up with it from the point estimate standpoint. Meanwhile, the rest of the pipeline continues to progress with some 17 programs and indications, including ADPKD, Alpha-1A trypsin, neurofibrotosis type 1 and CMT1A. In total, the activity has potential to yield five additional INDs by the end of this year, with some eight clinical proof of concept readouts to come in the 2027-2028 timeframe. Of course, despite all of this, we continue to stay focused on delivering our important medicines to patients in the commercial setting. And for more information on that, I'll pass it over to Matt.