Pulmonary Hypertension Virtual Patient Encounters_ A Patient Simulation Experience.PDF

CME/CNE/CPE Pulmonary Hypertension Virtual Patient Encounters A Patient Simulation Experience Supported by an independent educational grant from Actelion, A Janss...

CME/CNE/CPE Pulmonary Hypertension Virtual Patient Encounters A Patient Simulation Experience Supported by an independent educational grant from Actelion, A Janssen Pharmaceutical Company of Johnson and Johnson; Merck & Co., Inc.; United Therapeutics, a public benefit company SPEAKERS Vallerie V. McLaughlin, MD Chairperson Kim A. Eagle, MD Endowed Professor of Cardiovascular Medicine Director, Pulmonary Hypertension Program University of Michigan Ann Arbor, Michigan Murali Chakinala, MD, FCCP Josanna M. Rodriguez-Lopez, MD Professor of Medicine Assistant Professor Director, Pulmonary Hypertension Care Center Harvard Medical School Washington University School of Medicine in St Louis Massachusetts General Hospital St. Louis, Missouri Boston, Massachusetts  Agenda Welcome and Introductions Chairperson, Vallerie McLaughlin, MD Patient With PAH Case Presentation ▪ Patient assessment and evaluation of risk status ▪ Use of appropriate guideline-directed therapy to achieve treatment goal ▪ Use of appropriate strategies for patient monitoring and follow-up ▪ Use of appropriate strategies to improve treatment adherence to reach treatment goals Panel Discussion and Live Q&A PAH, pulmonary arterial hypertension. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Patient: Max “Patient quote here. Highlight part of sentence. More text” “ I’ve been short of breath for a while and can’t seem to get better ” Patient Profile Gender: Male Age: 65 year(s) Height: 172.7 cm Allergies: None Family and Social History ▪ Parents were smokers, both deceased in their 60s ▪ Max has 1 sister who has hypertension and “mild kidney problems.” ▪ Max works as an auto mechanic and is married, with 2 healthy children. ▪ Cardiac ROS: denies CP, PND, orthopnea, palpitations, LE edema These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. 6 Months Ago: Primary Care Office Max is here today to check in with symptoms of shortness of breath 6 months ago Weight, kg (lbs) 85 (187.4) BMI 28.5 RR 12 Pulse 76 Temp, F 98.6 BP, mm Hg 146/72 These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. 6 Months Ago: Primary Care Office Notes ▪ Max’s cardiopulmonary exam is unremarkable; clear breath sounds with no W/R/R and trace bilateral LE edema ▪ BP is not controlled ▪ Not using his CPAP at night ▪ Complete metabolic panel and complete blood count ordered to check kidney function and for anemia: within normal limits ▪ NT-proBNP: 800 pg/mL These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. 6 Months Ago: Primary Care Office Orders ▪ Use CPAP for at least 7 h a night consistently, take BP medications every day and record the results ▪ Losartan 20 mg is initiated for improved BP control ▪ Reinforced the importance of low-sodium foods and wearing elastic stockings during the day ▪ Return in 6 wk for follow-up These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. 4 Months Ago: Primary Care Follow-Up Max is here for follow-up. He states that he feels short of breath now when climbing a flight of stairs. He is using his CPAP for at least 7 hours a night consistently, taking BP medications every day, and recording the results (SBP range, 130-low 140 mm Hg). 4 months ago 6 months ago Weight, kg (lbs) 83 (183.0) 85 (187.4) BMI 27.8 28.5 RR 12 12 Pulse 76 76 Temp, F 98.6 98.6 BP, mm Hg 136/76 146/72 These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. 4 Months Ago: Primary Care Follow-Up Test Results Basic Metabolic Panel Within normal limits +CXR RA/RV/PA size increase, heart enlargement – lungs clear, no Chest X-Ray pulmonary edema, no effusions. No signs of left heart failure. Normal LV function, moderate TR with increased sPAP, and enlarged dilated Echocardiogram RA, RV and moderate RV dysfunction. TAPSE (1.3) cm/mm Hg dilated IVC Normal sinus rhythm, no ischemic changes, axis is neutral, and R1 seen in Electrocardiogram leads V1 and V2, indicating RV enlargement. NT-proBNP 500 pg/mL These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. 4 Months Ago: Primary Care Follow-Up Orders ▪ Continue losartan 20 mg daily ▪ Start furosemide 20 mg daily ▪ Low-sodium diet and include potassium-rich foods ▪ Record daily weights and BP ▪ Recommend referral to the PH center These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. 3 Months Ago: PH Center Max’s weight is down. His breathing is easier but not normalized. Previous echocardiogram results are highly suspicious for PH. 3 months ago 4 months ago 6 months ago Weight, kg (lbs) 79 (174.2) 83 (183.0) 85 (187.4) BMI 26.5 27.8 28.5 RR 12 12 12 Pulse 76 76 76 Temp, F 98.6 98.6 98.6 BP, mm Hg 135/76 136/76 146/72 These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Question 1 What of the following tests is used to confirm a diagnosis of PAH? 1. Right heart catheterization 2. V/Q perfusion scanning 3. NT-proBNP 4. Pulmonary function tests 5. 6-minute walk test These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Diagnostic Algorithm of Patients With Suspected PH Kovacs G, et al. Eur Respir J. 2024. doi:10.1183/13993003.01324-2024 [Epub ahead of print] These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. 3 Months Ago: PH Center Test Orders Test Results V/Q Lung Scan Negative HIV Test Negative 6MWT, m 390 NT-proBNP, pg/mL 500 These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. 3 Months Ago: PH Center Test Orders Test Results Basic Metabolic Panel RHC ▪ BUN 16 mg/dL ▪ PAPs 60/30 (mean, 40) mm Hg ▪ Chloride 104 mmol/L ▪ RAP 10 mm Hg ▪ Creatinine 1.0 mg/dL ▪ PAWP 12 mm Hg ▪ Glucose 85 mg/dL ▪ CI 2.8 L/min/m2 ▪ Potassium 4.5 mmol/L ▪ PVR 5.5 WU ▪ Sodium 140 mmol/L ▪ Bicarbonate 27 mEq/L Pulmonary Vasoreactivity Test: Negative These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Treatment Goals in PAH Dardi F, et al. Eur Respir J. 2024. doi:10.1183/13993003.01323-2024 [Epub ahead of print] These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Question 2 Which tool should be used to assess risk at diagnosis? 1. REVEAL 2.0 2. ESC/ERS 4-strata model 3. French registry 3 noninvasive criteria These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Assessment of Risk of Disease Progression in PAH International Survey Concordance Between Gestalt and Calculated Risk (dots indicate agreement) ▪ Gestalt risk: “In your opinion, how would you describe the patient’s current level of risk in terms of clinical worsening or death?” ▪ Calculated risk: 6 variables used to calculate risk of 1-y mortality: WHO FC, 6MWD, BNP/NT-proBNP, RAP, CI, and SvO2 Simons JE, et al. Adv Ther. 2019;36:2351-2363. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Risk Assessment Parameters Biochemical Markers ▪ NT-proBNP Clinical Assessment ▪ Progression of symptoms ▪ FC ▪ Syncope ▪ Signs of right HF Exercise Tests ▪ 6MWD ▪ CPET Hemodynamic Evaluations Echo Evaluations ▪ RAP ▪ RA area ▪ CI ▪ Pericardial effusion ▪ Mixed venous saturation. Galiè N, et al. Eur Heart J. 2016;37:67-119. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  REVEAL Risk Score Calculator 2.0 ▪ Low risk: ≤ 6 ▪ Intermediate risk: 7-8 ▪ High risk: ≥ 9 Benza RL, et al. Chest. 2019;156:323-337. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  REVEAL Risk Score Calculator 2.0 ▪ Low risk: ≤ 6 +2 ▪ Intermediate risk: 7-8 ▪ High risk: ≥ 9 +1 -1 8 Benza RL, et al. Chest. 2019;156:323-337. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  REVEAL-ECHO REVEAL-ECHO added to REVEAL Lite 2 supported further granularity of the risk assessment and further separation into 4 groups with differing survival. Survival curves according to 4 risk groups REVEAL-ECHO Parameter Risk Scoring System El-Kersh K, et al. Chest. 2023;163:1232-1244. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  COMPERA 3-Strata Risk Model 9 = 1.8 5 +2 +2 +2 +2 +1 Hoeper MM, et al. Eur Respir J. 2017;50:1700740. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  2022 ESC/ERS PH Guidelines Comprehensive PAH Risk Assessment: Three-Strata Model Humbert M, et al. Eur Heart J. 2022;43:3618-3731. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  2022 ESC/ERS PH Guidelines Comprehensive PAH Risk Assessment: Three-Strata Model Max’s Score: Intermediate Humbert M, et al. Eur Heart J. 2022;43:3618-3731. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Treatment Algorithm for PAH Chin KM, et al. Eur Respir J. 2024. doi:10.1183/13993003.01325-2024 [Epub ahead of print] These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Treatment Algorithm for PAH Max started on 10 mg macitentan daily + 40 mg tadalafil daily Chin KM, et al. Eur Respir J. 2024. doi:10.1183/13993003.01325-2024 [Epub ahead of print] These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Survival Based on Initial Treatment Strategy and Baseline Risk 1611 patients with PAH initiated therapy: ▪ Low risk (n = 234, mono or dual combo) ▪ Intermediate risk (n = 1134, mono, dual, or triple combo) ▪ High risk (n = 243, mono, dual, or triple combo) High Risk at Baseline Intermediate Risk at Baseline Boucly A, et al. Am J Respir Crit Care Med. 2021;204:842-854. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Follow-Up Assessment Suggested Assessment and Timing Green = is indicated; yellow = should be considered; orange = may be considered. Humbert M, et al. Eur Heart J. 2022;43:3618-3731. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Today: PH Center Follow-Up These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Today: PH Center Follow-Up These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Today - PH Center Follow-Up Vitals Vitals Today 3 months ago 4 months ago 6 months ago Weight, kg (lbs) 76 (167.6) 79 (174.2) 83 (183.0) 85 (187.4) BMI 25.5 26.5 27.8 28.5 RR 12 12 12 12 Pulse 76 76 76 76 Temp, F 98.6 98.6 98.6 98.6 BP, mm Hg 125/76 135/76 136/76 146/72 These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Today - PH Center Follow-Up Current Medications Current Medications Started Therapy Frequency 4 months ago Furosemide 20 mg Daily 4 months ago Losartan 40 mg Daily 3 months ago Macitentan 10 mg Daily 3 months ago Tadalafil 40 mg Daily These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Tests These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Tests Guidance These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Tests Guidance These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Tests Guidance These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Tests Guidance These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Tests Guidance These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Today - PH Center Follow-Up Summary of Tests WHO FC II Metabolic Panel Cr = 1.6 mg/dL, otherwise within normal limits eGFR 55 mL/min (CKD Stage 3) NT-proBNP 400 pg/mL 6MWT 420 m ▪ RVEF: 35% Chest MRI ▪ Stroke volume index, 30 mL/m2; RV end-systolic volume index, 52 mL/m2 High VE/VCO2 slope, with low PETCO2, decreasing during exercise with no EOV CPET (peak VO2, 11-15 nL/min/kg, 35%-65% predicted); VE/VCO2 slope, 36-44 These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Risk Scoring Tools at Follow-Up Dardi F, et al. Eur Respir J. 2024. doi:10.1183/13993003.01323-2024 [Epub ahead of print] These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Four-Strata Risk Assessment iPAH, HPAH, DPAH, PAH Associated With CTD (including the SSc subgroup), and PoPH Low risk Intermediate-low risk Intermediate-high risk High risk 1-y mortality rates in the 4 risk strata: 0% to 3% 2% to 7% 9% to 19% > 20% *Include RV assessment by Echo or cMRI and hemodynamics on an individual basis. Hoeper MM, et al. Eur Respir J. 2022;60:2102311; Humbert M, et al. Eur Heart J. 2022;43:3618-3731. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Today: Follow-Up Risk Assessment REVEAL Lite 2 Guidance These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Today: Follow-Up Risk Assessment ESC/ERS 4-Strata Risk Model Guidance These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Survival According to Changes in Risk Strata Between Baseline and First Reassessment Boucly A, et al. Eur Respir J. 2022;59:2102419. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Question 3 What would you do next? 1. No change to current treatment (macitentan + tadalafil) 2. Add a prostacyclin pathway agent 3. Add sotatercept (BMP/activin pathway) 4. Switch tadalafil for riociguat BMP, bone morphogenetic protein. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Treatment Algorithm for PAH Chin KM, et al. Eur Respir J. 2024. doi:10.1183/13993003.01325-2024 [Epub ahead of print] These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  PAH Therapies: 4 Major Pathways Chin KM, et al. Eur Respir J. 2024. doi:10.1183/13993003.01325-2024 [Epub ahead of print] These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  STELLAR Phase 3 Trial of Sotatercept for PAH Design: Multicenter, randomized, double-blind trial Subjects: adults with PAH (WHO FC II or III) on stable background therapy Treatment groups: ▪ SC sotatercept every 3 wk (starting dose, 0.3 mg/kg; target dose, 0.7 mg/kg) ▪ Placebo every 3 wk Primary outcome: change from baseline to 24 wk in 6MWD Baseline characteristics: ▪ 35% on double therapy ▪ 61% on triple therapy ▪ 40% on PCAs ▪ PVR: 9.5 WU on average Hoeper MM, et al. N Engl J Med. 2023;388:1478-1490. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  STELLAR: Sotatercept for PAH Outcomes Primary Outcome: 6MWD KM Curve: Time to Worsening/Death Event Sotatercept Mean Imputed median Imputed median (prespecified analysis) (post hoc analysis) Placebo Mean Imputed median Imputed median (prespecified analysis) (post hoc analysis) 60 1.0 Probability of Event-Free Survival 0.9 50 Sotatercept Change From Baseline, m 0.8 40 0.7 30 0.6 Placebo 6MWD - 40.8 m difference 20 0.5 (95% CI: 27.5, 54.1 m; P <.001) 10 0.4 84% reduction 0.3 P <.001 by log-rank test 0 0.2 -10 0.1 -20 0.0 0 3 6 9 12 15 18 21 24 0 5 10 15 20 25 30 35 40 45 50 55 50 65 70 75 Weeks After Randomization Weeks to First Occurrence of Either Death or Nonfatal Clinical Worsening Event. Hoeper MM, et al. N Engl J Med. 2023;388:1478-1490. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  STELLAR Adverse Events Through Week 24 AEs that occurred more frequently with sotatercept than placebo Sotatercept Placebo Difference Variable, No. (%) (n = 163) (n = 160) (95% CI) Increased hemoglobin level 9 (5.5) 0 5.5 (2.9, 10.2) Thrombocytopenia 10 (6.1) 4 (2.5) 3.6 (−0.9, 8.8) Bleeding events 35 (21.5) 20 (12.5) 9.0 (0.8, 17.2) Increased BP 6 (3.7) 1 (0.6) 3.1 (−0.2, 7.3) Telangiectasia 17 (10.4) 5 (3.1) 7.3 (2.0, 13.3) Epistaxis 20 (12.3) 3 (1.9) 10.4 (5.2, 16.6) Dizziness 17 (10.4) 3 (1.9) 8.6 (3.6, 14.4) Hoeper MM, et al. N Engl J Med. 2023;388:1478-1490. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Sotatercept SC Injection FDA Approved March 2024 Indications ▪ Treatment of adults with PAH (WHO Group 1) to increase exercise capacity, improve WHO FC, and reduce the risk of clinical worsening events Sotatercept-csrk [PI]. Approved 2024. Revised March 2024. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Macitentan/Tadalafil Single-Tablet Combination Therapy for PAH A DUE Study Ekkehard Grünig E, et al. J Am Coll Cardiol. 2024;83:473-484. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Oral Combination Macitentan and Tadalafil FDA Approved March 2024 Indications ▪ Chronic treatment of PAH (WHO Group 1) in adult patients of WHO FC II to III ▪ Individually, macitentan reduces the risk of clinical worsening events and hospitalization, and tadalafil improves exercise ability Macitentan and tadalafil [PI]. Approved 2024. Revised March 2024. These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited. Nonmedication Orders These materials are provided to you solely as an educational resource for your personal use. Any commercial use or distribution of these materials or any portion thereof is strictly prohibited.  Q&A To earn your credit, enter the URL below on your personal device: www.medscape.org/symposium/pah-simulation-credit OR Scan the QR code with your mobile device  THANK YOU FOR PARTICIPATING! To earn your CME/CNE/CPE credit: Click on the “Earn Credit” icon, OR Visit www.medscape.org/symposium/pah-simulation-credit OR Scan the QR code with your mobile device