1b) @AlbertaPHdoc from #Edmonton will be discussing Serial, Multidimensional #RiskAssessment in #PAH: How, Why, and Then What to Individualize Goal-Directed Therapy. It's all about 🫁 !
— pulmmed_ce (@Pulmmed_CE) April 25, 2023
👋@bmprii @SandeepSahayMD @LucillaPiccari @PVRI @OSitbon @jeanlucvachiery @PGeorgeMD
3) Let’s start with a case: 33♂️ presents to #emergencymedicine w/ dyspnea x 6m + two episodes exertional syncope.
— pulmmed_ce (@Pulmmed_CE) April 25, 2023
Hx: regular methamphetamine use >6 years ago, smoking; active #HIV infection, intermittently taking anti-retroviral Rx, but not always adherent. No other meds.
5) Initial labs ➡️NT-proBNP = 12,000 ng/L, #lymphopenia with a #CD4+ count of 130. Viral load is elevated & liver enzymes are very high – ALT 1200, AST 1200, Bilirubin 27 IU/L. He has acute #kidney injury with Cr 300 and eGFR of 32. His chest X-ray is below: pic.twitter.com/7Weii5tuPq
— pulmmed_ce (@Pulmmed_CE) April 25, 2023
7) Answer: A. He has 2 #riskfactors for pulmonary arterial hypertension (Group 1 #PH): both methamphetamine & HIV are assoc'd w/ #PAH. The clear lungs argue against #LV failure & no signs of #PJP. #DVT+#PE could also explain presentation, but less likely to ➡️ bilateral edema.
— pulmmed_ce (@Pulmmed_CE) April 25, 2023
9) You arrange an urgent right heart catheterization #RHC: RA 12, RV 65/7, EDP 19, PA 61/21 with mean 39, PAWP 12, CO 2.43, CI 1.28, SVI 12, PVR 11 Wood units. MvO2 44%. There is no response to inhaled nitric oxide #iNO.
— pulmmed_ce (@Pulmmed_CE) April 25, 2023
11) How would you assess this patient’s risk to determine his treatment?
— pulmmed_ce (@Pulmmed_CE) April 25, 2023
13) There are several approaches to risk assessment in #PAH. The REVEAL 2.0 score is an updated version of the original #REVEAL score derived from a large 🇺🇸 registry.
— pulmmed_ce (@Pulmmed_CE) April 25, 2023
REVEAL original: 🔓 https://t.co/6V4fgj7ffb
REVEAL 2.0 🔓 https://t.co/KhrefYMdcl pic.twitter.com/LlACTGMYIg
15) REVEAL Lite 2 is an abbreviated version of #REVEAL 2.0, uses 6 variables (#BNP/NT-proBNP, #6MWD, #NYHA Functional Class, SBP < 110 mm Hg, HR >96 bpm, renal insuff) & retains ability to discriminate pts into Low, Intermediate, & High risk groups.
— pulmmed_ce (@Pulmmed_CE) April 25, 2023
🔓 https://t.co/EQIEaTWb7X pic.twitter.com/3oKXOrcNeU
17) Using the 4-strata approach during follow-up nicely identifies groups with distinct survival trajectories.
— pulmmed_ce (@Pulmmed_CE) April 25, 2023
🔓 https://t.co/9Z5hsf1rQA pic.twitter.com/qdHxkPYLmJ
19) This study showed that for patients in functional class II, expert clinicians frequently categorized patients as low risk when, in fact, many were at intermediate or high-risk using objective risk assessment tools. pic.twitter.com/Qk1rXSDKfq
— pulmmed_ce (@Pulmmed_CE) April 25, 2023
21) Answer: D. No matter which way we slice it, this pt is high risk. Using the ESC/ERS table, REVEAL 2.0 & 2.0 Lite, he is hi risk. He is NYHA class III but has syncope, severe hemodynamics w/⬆️ RAP, ⬇️ CI & SVI, very ⬆️ NT-proBNP. Even w/o other data like 6MWD, he is hi risk. pic.twitter.com/H4lHu3rebB
— pulmmed_ce (@Pulmmed_CE) April 25, 2023
23) Answer: C, but it depends on the individual patient. Many factors to consider in addition to risk level, particularly when considering parenteral tx. Also, disease-specific considerations, preferences, & patient-specific factors can influence tx selection. More on this later.
— pulmmed_ce (@Pulmmed_CE) April 25, 2023
25) Welcome back!! We are talking Multidimensional, Serial Risk Assessment in #PAH with expert author @AlbertaPHdoc. 🆓CE/#CME at the end of this 🧵!
— pulmmed_ce (@Pulmmed_CE) April 26, 2023
👋@TamCardio @docroham @SudarRajagopal @heresi_gustavo @RyanTedfordMD @rjbernardoMD @kurt_prins @SashaPrisco
27) In this algorithm, the distinction is made for patients w/ & w/o #cardiopulmonary #comorbidities. Pts with significant CP issues were often excluded from #RCTs, & several studies show that such pts do not respond to, or do not tolerate, more aggressive tx. pic.twitter.com/BvXEDddxSW
— pulmmed_ce (@Pulmmed_CE) April 26, 2023
29) For high-risk #PAH, guidelines suggest upfront triple tx, tho strength of evidence for this rec is lower (Class IIa). Non-randomized studies suggested triple therapy may improve long-term survival vs 1 or 2 drugs, especially in high-risk patients.
— pulmmed_ce (@Pulmmed_CE) April 26, 2023
🔓 https://t.co/69y8zHGRQt pic.twitter.com/CJMWMHxkKn
31a) Answer: There is no right answer here, but there are several things to consider: 1) can this patient manage the complexity of parenteral drug long-term?
— pulmmed_ce (@Pulmmed_CE) April 26, 2023
31c) Answer Continued….
— pulmmed_ce (@Pulmmed_CE) April 26, 2023
3) oral #PAH drugs might not be absorbed and metabolized normally with acute #RV failure, 4) #ERAs, especially #bosentan, have a risk of #hepatotoxicity (recall his liver enzymes were >1000).
31e) In patients with #methamphetamine-associated #PAH, parenteral #prostanoids are used less frequently, despite patients having a worse overall prognosis than idiopathic PAH patients. See 🔓 https://t.co/V47Pg5Dxyn pic.twitter.com/3alvQJHRll
— pulmmed_ce (@Pulmmed_CE) April 26, 2023
33) In this case, pt started on IV #epoprostenol in 🏥 but couldn't tolerate typical side effects (headache, flushing, diarrhea, myalgias) so it was d/c'd. He declined a trial of SQ #prostanoid. He was tx'd w/ oral #tadalafil. #Macitentan started 1wk later after LFTs improved.
— pulmmed_ce (@Pulmmed_CE) April 26, 2023
35) Answer: C. All of his variables line up in the intermediate-high risk column, with an average score of 3. Therefore, he is still intermediate-high risk.
— pulmmed_ce (@Pulmmed_CE) April 26, 2023
37a) Adding #selexipag is based on the #GRIPHON trial, which remains the largest #PAH clinical trial to date. About 1/3 of patients in GRIPHON were receiving background #PDE5i & an ERA.
— pulmmed_ce (@Pulmmed_CE) April 26, 2023
38) The option to switch a #PDE5i to #riociguat was studied in the #REPLACE trial. In this open label RCT, patients switching to riociguat more often achieved the ‘multi-component improvement’ endpoint (improvement in 2/3 of NYHA, 6MWD & NT-pro-BNP)
— pulmmed_ce (@Pulmmed_CE) April 26, 2023
See https://t.co/bzkbeBnzNM. pic.twitter.com/rHH7N8Lal3
40) You decide to switch your patient to #riociguat for two reasons: 1. the patient reports experiencing episodes of priapism since starting tadalafil and 2. the patient refused a trial of #selexipag due to his experience with prostanoid side effects in hospital.
— pulmmed_ce (@Pulmmed_CE) April 26, 2023
42) #PAH therapies carry side effects and risk, and an individualized approach that considers comorbidities, concomitant medications, tolerability, social determinants of health and the availability of support also inform initial therapy and future changes.
— pulmmed_ce (@Pulmmed_CE) April 26, 2023
43) Speaking of support, YOU just earned 0.75hr 🆓CE/#CME! 🖱️to https://t.co/DUSgYbMfeE and grab your certificate. I am @AlbertaPHdoc and I thank you for joining me. FOLLOW US here on @Pulmmed_CE for more 🫁 #pulmtwitter #MedEd #FOAMed #FOAMcc delivered wholly on Twitter!
— pulmmed_ce (@Pulmmed_CE) April 26, 2023