2) This program is supported by educational grants from Actelion, AstraZeneca, and Bayer, and is intended for healthcare professionals. Statement of accreditation & disclosures https://t.co/0EkFYk5rP5. Follow this 🧵for credit. And here is a case of a pt with #scleroderma . . .
— pulmmed_ce (@Pulmmed_CE) October 17, 2022
4) In general, how often should patients with #scleroderma be screened for #PAH?
— pulmmed_ce (@Pulmmed_CE) October 17, 2022
6) 2015 @ESCardio/@EuroRespSoc guidelines: Screening with resting #echocardiography is recommended in asymptomatic patients, followed by annual screening with echo, diffusion capacity for carbon monoxide (#DLco), and biomarkers. See 🔓 https://t.co/yTWClv0Num
— pulmmed_ce (@Pulmmed_CE) October 17, 2022
8) 6th World Symposium: Annual screening with #PFTs and echocardiography should be considered in patients with the #SSc spectrum of diseases with uncorrected #DLco <80%. See 🔓https://t.co/i60dfAAUco
— pulmmed_ce (@Pulmmed_CE) October 17, 2022
10) Different thresholds for FVC%/DLCO% ratio ranging from ≥ 1.6 to ≥ 2.0. The sensitivity (91%) and NPV (93%) for detecting #PAH are improved with the lower cut-off while specificity is improved with the higher cutoff.
— pulmmed_ce (@Pulmmed_CE) October 17, 2022
See 🔓https://t.co/1gAJbPLG7R
12) She underwent #TTE that demonstrated a moderately enlarged right ventricle with mild dysfunction. Minimal TR – #TRjet 2.9 m/s with an estimated RVSP 44 mmHg. #TAPSE 1.8 cm. IVC dilated with abnormal respiratory collapse.
— pulmmed_ce (@Pulmmed_CE) October 17, 2022
14) The answer is A: #Scleroderma. #PAH associated with scleroderma is associated with higher risk. Male sex, especially over the age of 60, is associated with worse outcomes in PAH.
— pulmmed_ce (@Pulmmed_CE) October 17, 2022
16) The answer is C: VQ scan. All patients undergoing an evaluation for #PH should have a VQ scan to rule out chronic pulmonary embolism as a potential cause for their PH. Noncontrast CT chest is recommended to evaluate for #parenchymal #lungdisease in certain populations.
— pulmmed_ce (@Pulmmed_CE) October 17, 2022
18) Here’s a “by the way” for you. Know about vasodilator testing in patients with possible PAH? Check out https://t.co/S5AK7N3kRN. Shout out to @PHAssociation
— pulmmed_ce (@Pulmmed_CE) October 17, 2022
20) Answer is B: Intermediate risk. French registry invasive risk assessment has 4 criteria for low-risk status: WHO functional class I or II, 6MWD > 440 m, RA pressure <8 and CI ≥ 2.5. She meets 2/4 criteria. pic.twitter.com/Ggwo54A8Fb
— pulmmed_ce (@Pulmmed_CE) October 17, 2022
22) What therapy would you start? (and BTW this initial decision is best made by a #PAH specialist)
— pulmmed_ce (@Pulmmed_CE) October 17, 2022
A)Phosphodiesterase 5 inhibitor (PDE5i)
B)Endothelin Receptor Antagonist (ERA)
C)PDE5i + ERA
D)PDE5i + ERA + Prostacyclin
24a) Welcome back! I am @SudarRajagopal & you are earning 🆓CE/#CME about #PulmonaryHypertension. Thank you for joining us on this 🆕#accredited #tweetorial feed! Nods to @kurt_prins @jeanlucvachiery @RyanTedfordMD @SashaPrisco @ImadAlGhouleh @zeenatsafdarmd @rjbernardoMD
— pulmmed_ce (@Pulmmed_CE) October 18, 2022
25) The answer is C: PDE5i + ERA. The AMBITION trial demonstrated that initial combination therapy with a PDE5i + ERA was superior to PDE5i or ERA monotherapy. (D) is also reasonable and depends on risk profile/patient preference. See https://t.co/zR7VQ81u7m pic.twitter.com/ycWKTbrt3T
— pulmmed_ce (@Pulmmed_CE) October 18, 2022
27) Answer is B: Intermediate risk. Despite her improvement in #6MWD, her #NTproBNP is still elevated so she is still intermediate risk. Some would consider scleroderma as being an additional risk factor that would also contribute to her being relatively higher risk.
— pulmmed_ce (@Pulmmed_CE) October 18, 2022
29) Answer is C: Achievement of low risk status. Data from the French registry and others show that patients who achieve low risk status have better outcomes than those who do not improve in risk class.https://t.co/AZwFWcyluS pic.twitter.com/4vnQuqvSLZ
— pulmmed_ce (@Pulmmed_CE) October 18, 2022
31) The answer is D: All of the above. All of these are reasonable strategies for treating patients who are not reaching their treatment goals on therapy with an #PDE5i + ERA. If the patient were FC 4, a class I recommendation for IV #epoprostenol would be recommended.
— pulmmed_ce (@Pulmmed_CE) October 18, 2022
33) The GRIPHON trial was a study of oral #selexipag (a #prostacyclin receptor agonist) compared to placebo in patients on stable #PDE5i, ERA or both that showed an improvement in time to clinical worsening.
— pulmmed_ce (@Pulmmed_CE) October 18, 2022
See 🔓https://t.co/GSjg1jyDIS pic.twitter.com/8wJFynjAgh
35) Inhaled #prostacyclins options include inhaled #iloprost (given 6 to 9 times daily) and inhaled treprostinil (given 4 times daily). Both of these drugs have been shown to improve six minute walk distance. See 🔓https://t.co/GQkT6wAL4s and 🔓https://t.co/TpJ06S2PXd
— pulmmed_ce (@Pulmmed_CE) October 18, 2022
37) The REPLACE trial considered the strategy of replacing a #PDE5i w/ an sGC stimulator (#riociguat). This study found that transitioning to riociguat was assoc'd w/significant improvement in 6MWD, tho no benefit was seen in the #tadalafil subgroup. See https://t.co/bzkbeBFaFk pic.twitter.com/vmOcFnnsw5
— pulmmed_ce (@Pulmmed_CE) October 18, 2022
39) After discussion with the patient, an oral #prostacyclin was added. Three months later, she returned for follow-up with an improvement in her #6MWD 460 m and #NTproBNP 250 ng/ml. She has achieved low-risk status!
— pulmmed_ce (@Pulmmed_CE) October 18, 2022
41) To summarize, Pulmonary arterial hypertension (#PAH) is a disease w/ obstruction of the small pulmonary blood vessels, resulting in right heart failure & death if untreated. Patients with high-risk features & those needing advanced tx should be referred to expert centers.
— pulmmed_ce (@Pulmmed_CE) October 18, 2022
43) That’s it! You made it! 🆓CE/#CME! #physicians #nurses #pharmacists #PAs #NPs make your way to https://t.co/RFPhY7YVp5 for 0.5h credit, applicable 🇺🇸🇨🇦🇬🇧🇪🇺. I am @SudarRajagopal and I 🙏you for joining us . . . PLEASE follow @pulmmed_ce for the best in 🫁 med CE/#CME!
— pulmmed_ce (@Pulmmed_CE) October 18, 2022