All right, good evening, everybody. Welcome to this evening's session. We're really excited about it. It's titled The Amyloid Files, Cracking the Case, Evolving Strategies in Cardiac Amyloidosis. My name is Ron Witellis from Stanford University School of Medicine, and we really have a terrific panel here tonight who are all on stage. I'm Ruden Bardekar from University of Colorado, Justin Grodin from UT Southwestern, Margo Davis from University of British Columbia, and Robert Page from University of Colorado. I want to acknowledge the contributors for the educational grants for tonight's spotlight session from Alnilam, AstraZeneca, and BridgeBio. Really important to claim credit for this course. Make sure you've opted in through the ACC25's credit system by visiting accscientificsession.org slash credit, and then within 24 hours, you'll receive a link to the course evaluation via email, and you must complete the evaluation to claim credit, and that completion information will be transmitted to ACCME on your behalf. All right, also really important, so we're gonna have a lot of audience response systems tonight, and so if you haven't already, please scan this QR code, and we're gonna have this in a couple of different forms. We have pre-assessment questions, which will be, again, part of the whole credit part for this, for that, we're gonna show you the questions, but you're not gonna get the answers now, because that's part of hopefully showing what you all will learn tonight, and then if you see this right here, one lucky winner is gonna be, apparently you don't get to take it home, because it won't fit through TSA, but you do get to take a picture with this trophy for the competitive portion of tonight, where three of the sessions will be having, well, I'll explain it in just a moment. So first things first, let's do the pre-assessment questions. This has nothing to do with the trophy, I'm afraid. So these are the pre-assessment questions, and I'll read it for you, and then the choices will come on the next slide. So question one, you're referred a 72-year-old man for evaluation of new exercise intolerance. He was diagnosed with atrial fibrillation last year and prescribed metoprolol, but felt extremely fatigued, so he underwent a successful cardioversion. Since then, he has had gradually progressive NYHA class II or III symptoms, but has not been hospitalized. He has no history of hypertension, has well-controlled diabetes, and a 50-pack-a-year smoking history. He has a history of bilateral carpal tunnel surgery 15 years ago, and lumbar spinal stenosis. Exam is unremarkable, NC ProBNP is 2,800, high-sensitivity troponin I is 42, and his EGFR is 52. ECG shows sinus rhythm with first-degree AV block and antireceptal Q waves, and his echo shows normal LV size with an ejection fraction of 40%, concentric LVH, a septum measuring 14 millimeters, and a CCTA shows a grade III stenosis in the mid-RCA only. So which of the following are red flags for cardiac amyloidosis? Coronary artery disease on CCTA, intolerance of beta blockers, the history of smoking, reduced EF on echo, or reduced EGFR? All right. Let's do this. »» Okay. Question by the way, results intentionally hidden by me. It's not because I don't want to tell you, but I'm not allowed to tell you. So that will be for the end. Assessment question number two. A 70-year-old man is being evaluated for potential ATTR amyloidosis. Which of the following patient characteristics and testing results would confirm a diagnosis of ATTR amyloid cardiomyopathy? A, severe left ventricular hypertrophy and a confirmed V122I mutation. B, normal serum and urine protein electrophoresis with grade 3 uptake on a PYP scan with SPECT imaging. C, normal serum and urine protein electrophoresis, a serum-free light chain ratio of 1.95, you see the normal range. Stage 4 CKD and grade 3 uptake on a PYP scan with SPECT imaging. D, normal serum and urine protein electrophoresis with immunofixation, a serum-free light chain ratio of 1.2 and a grade 3 uptake on a PYP scan by planar imaging. Or E, increased ventricular wall thickness and an endomyocardial biopsy showing Congo red positive amyloid deposits. Okay. Next question, a 66-year-old man with wild type ATTR cardiomyopathy returns to your office for follow-up. He's euvolemic on exam after starting a low-dose loop diuretic SGLT2 inhibitor and mineral corticoid receptor antagonist. He currently has NYJ class 2 symptoms and his laboratory evaluation shows a normal potassium renal function and an NC probian P value of 1,100. What is the most appropriate next step in the management of this patient's wild type ATTR cardiomyopathy? A, add a GLP-1 agonist. B, start the patient on an approved trans-thyroid amyloidosis disease-specific treatment to prevent progression. C, refer to hem-onc for chemotherapy. Or D, refer for consideration of cardiac transplantation. Okay. Next question, a 51-year-old man with a history of hypertension, carpal tunnel, a family history of heart failure, neuropathy, recently had a genetic test revealing that he's heterozygous carrier of the pathogenic, we'll talk about the nomenclature a little later, PTADA TTR allele. His mother had a heart and liver transplant for trans-thyroid amyloidosis at age 58. His cardiac and neurologic exam is normal, EKG shows normal voltage, and he's in normal sinus rhythm. His echo demonstrates an interventricular septal thickness of nine millimeters with a normal LV strain pattern. High sensitivity cardiac troponin is 12, and his antiprobian P is 142. He only has a bilateral median neuropathy on EMG NCS and no other abnormalities, and he's asking about strategies to prevent ATTR. What's the most appropriate next step? Initiate diflunasol, 250 milligrams twice a day. Initiate dietary supplementation with green tea extract. Initiate treatment with doxycycline and, I'm not even, Tudco, we'll say. Initiate treatment with Vutri Saran and vitamin A supplementation, or discuss clinical trials testing CTR-targeted therapies for prevention of ATTR, such as ACT-Early. All right, and our final question, a 67-year-old male with past medical history of biventricular heart failure is admitted to the inpatient service with shock. Cardiac amyloidosis is suspected via cardiac MRI, so a formal amyloidosis workup is ordered showing the following, a PYP scan positive, a bone marrow biopsy showing marked serous degeneration, plasma cells are slightly increased with no light chain restriction and no amyloid deposits. The heart failure multidisciplinary team wants to begin the tefamidus, the tefamidus, and the patient currently has Colorado Medicaid for his prescription coverage. Using the expertise of your multidisciplinary team, which of the following would be your best first approach to minimizing barriers? A, have the nurse practitioner send the tefamidus prescription to his community pharmacy to determine an exact out-of-pocket cost. B, have the clinical pharmacist evaluate the Colorado Medicaid preferred drug list, obtain exact prior authorization requirements needed, and potential out-of-pocket co-pays. C, have the social worker meet with the family for possible fundraising to pay for the high cost of tefamidus. Or D, have the clinical dietitian determine if there are any food-drug interactions with tefamidus. Okay. That's all the pretest questions. So now let's get on to the trivia competition. Okay. So the way this is going to work, and we hope you all will join, is on your phone, click the button to join the trivia battle. Or you can use the QR code if you haven't already done it. So click that joint trivia battle button. And then put in your name and pick your icon. Keep in mind, everybody, your name may end up on the screen, so choose wisely. When you hear the sound clip on the questions, get your phone out, get ready to answer the questions. Points will be generated based not only on the number of correct answers, but how quickly you respond. And the top 10 trivia winners claim the trophy. I'm not sure how 10 people are going to claim it, but I guess you'll all get to take a picture with it. And there will be three rounds. So let's get started. Again, this is the code if you don't have it. And with that, I'm going to turn the floor over to our first presenter, Dr. Margo Davis from UBC.

Cardiac Amyloidosis

Educational Grants

Interactive Session

Audience Response System

Trivia Competition