2) I am very excited to be among the founding faculty in this new educational initiative. FOLLOW US for the latest & greatest in expert-led education #pulmtwitter!
— pulmmed_ce (@Pulmmed_CE) October 12, 2022
👍@SashaPrisco @mark_toshner @charifa_PVRI @SteveMathaiMD @rjbernardoMD @heresi_gustavo @PGeorgeMD @RyanTedfordMD
4) Let’s start with a case! A 34F non-smoker presents with progressive dyspnea over 4 wks & a syncopal episode while carrying groceries to her car. PMHx is unremarkable & no FamHx of 🫀disease. CT scan shows no acute PE, normal lung parenchyma, & pulmonary artery dilation.
— pulmmed_ce (@Pulmmed_CE) October 12, 2022
6) Answer D: All of the above. DLCO can be low from ⬇️ alveolar capillary membrane conductivity (⬇️surface area from emphysema, interstitial thickening from ILD, ⬇️ vessels in pulmonary vascular disease) and those that affect binding of CO to Hb (⬇️ Hb, ⬆️CO-Hb)
— pulmmed_ce (@Pulmmed_CE) October 12, 2022
8) Answer: B. The presence of #dyspnea, isolated low DLCO, syncope and enlarged pulmonary artery suggests pulmonary hypertension (#PAH). An #echocardiogram is recommended to assess the probability of #PAH, which guides additional testing and referral. 🔓https://t.co/2HKtYOqJsY pic.twitter.com/vwpYZRdERL
— pulmmed_ce (@Pulmmed_CE) October 12, 2022
10) What is the likelihood of pulmonary hypertension based on these echo findings?
— pulmmed_ce (@Pulmmed_CE) October 12, 2022
12) A right heart catheterization is performed. RAP 18, RV 89/5 EDP 10, PA 92/36 (mean 55), PAWP 5, CO 3.23, CI 1.7. PVR 15.5. HR 112 SVI 15. BP 98/65 mmHg Mixed venous O2 60%. Vasodilator testing is performed with inhaled nitric oxide with no significant change in hemodynamics.
— pulmmed_ce (@Pulmmed_CE) October 12, 2022
14) Her 6-minute walk distance was 300 m and NT-proBNP is 1285 ng/L. Ventilation-perfusion scan and abdominal ultrasound normal. HIV and connective tissue disease serologies are negative.
— pulmmed_ce (@Pulmmed_CE) October 12, 2022
16) Mark your answer now and return TOMORROW for more on this case and more expert #pulmonaryhypertension education! Nods to @anjalivaidyaMD @RogerAlvarezDO @kurt_prins @SudarRajagopal @JimWhiteCurePAH @OStibon @ATS_PC @PVRI @docroham @Viniciodjperez @Montanidavid
— pulmmed_ce (@Pulmmed_CE) October 12, 2022
18) So let’s recap. We have a 35♀️ with pre-capillary PH and severe RV dysfunction. Lung parenchyma and PFTs are normal other than low DLCO. Her V/Q was normal and there is no sign of chronic liver disease or connective tissue diseases. We ended up yesterday with a poll. pic.twitter.com/FckmS92Run
— pulmmed_ce (@Pulmmed_CE) October 13, 2022
20) She has advanced symptoms in NYHA functional class III, syncope, 6MWD 320m, HR 112, BP 98/65 RAP 18, CI 1.7 and NT-proBNP 1285. What is her risk category?
— pulmmed_ce (@Pulmmed_CE) October 13, 2022
22) What initial therapy would you start her on?
— pulmmed_ce (@Pulmmed_CE) October 13, 2022
A. Phosphodiesterase type-5 inhibitor (PDE5i)
B. Endothelin receptor antagonist (ERA)
C. PDE5i + ERA
D. PDE5i + ERA + IV prostacyclin
24) Initial combination therapy w/ a PDE5i+ERA is the preferred strategy for treatment naïve PAH patients who are low or intermediate risk. Combination therapy w/ 2 oral drugs is superior to monotherapy w/ a PDE5i or ERA, based on the AMBITION trial.
— pulmmed_ce (@Pulmmed_CE) October 13, 2022
🔓 https://t.co/zR7VQ7Kr5m pic.twitter.com/Dto00n3kwS
26) There are no RCTs in #PAH of triple therapy including IV prostacyclin versus two drugs. However, this strategy is used for high-risk patients who are younger and able to handle the complexities of a continuous IV therapy (more to come about this…)
— pulmmed_ce (@Pulmmed_CE) October 13, 2022
28) Intravenous #prostacyclins include #epoprostenol & #treprostinil. These meds are complicated. They require an indwelling central venous catheter, small battery-powered pump, & frequent mixing of vials by the patient or caregiver.
— pulmmed_ce (@Pulmmed_CE) October 13, 2022
30) #Epoprostenol remains the only PAH therapy with a demonstrated mortality benefit in an RCT. The initial epoprostenol trial in 1996 observed a short-term reduction in death in 81 patients, at a time when few Rx options were available. 🔓https://t.co/JoWUyPdWmt pic.twitter.com/JvvNAt63al
— pulmmed_ce (@Pulmmed_CE) October 13, 2022
32) Please mark your answer and come back tomorrow for the correct answer, more education, and a link to grab your FREE CE/#CME! @pulmmed_ce is new and is da 💣 –follow us before everyone does!@SashaPrisco @charifa_PVRI @SteveMathaiMD @rjbernardoMD @heresi_gustavo @jeffminMD
— pulmmed_ce (@Pulmmed_CE) October 13, 2022
34) So there was a poll (tweet # 31) yesterday. Scroll back up and vote, if you haven't already!! @RyanTedfordMD @SandeepSahay @docroham @anjalivaidyaMD @kurt_prins @JimWhiteCurePAH @OSitbon @ATS_PC @PVRI @Viniciodjperez @Montanidavid @jeanlucvachiery
— pulmmed_ce (@Pulmmed_CE) October 14, 2022
36) After 6 months, she wants to discuss becoming pregnant. What should you recommend to her about pregnancy and contraception?
— pulmmed_ce (@Pulmmed_CE) October 14, 2022
A. Pregnancy is safe in PAH.
B. Pregnancy is high risk and should be avoided.
C. ERAs are teratogenic.
D. B and C.
38) Because ERAs are teratogenic, two forms of contraception are recommended for females of childbearing age with PAH. There is no consensus on the best methods of contraception. Importantly, the ERA #bosentan can decrease the effectiveness of oral contraceptive pills.
— pulmmed_ce (@Pulmmed_CE) October 14, 2022
40) After 6 months of treatment, the patient is in NYHA class II, has a 6MWD of 480m, NT-proBNP 250 ng/L. Repeat RHC demonstrates:
— pulmmed_ce (@Pulmmed_CE) October 14, 2022
RAP 7
mPAP 35
PAWP 6
CO 5.7
CI 2.9
PVR 5.1
HR 69
SVI 42 mL/m2.
BP 95/60
42) Answer: A. She has achieved a low-risk profile. Using the French method (NYHA I/II, 6MWD >440, RAP <8, CI >2.5) she has 4/4 low-risk criteria and her REVEAL 2.0 score is 3, which is low-risk.
— pulmmed_ce (@Pulmmed_CE) October 14, 2022
44) One year later your PAH patient is feeling great but her BMI has ⬆️ to 32. She wants to start exercising more. What do you tell her?
— pulmmed_ce (@Pulmmed_CE) October 14, 2022
A. Exercise training improves quality of life in PAH
B. Exercise is not safe in PAH
C. Exercise training improves 6MWD by >30m
D. A and C.
46) Many patients with PAH are deconditioned and obesity is a common comorbidity. #Metabolic_syndrome and #insulin_resistance are also frequent in PAH. Exercise can empower patients, improve conditioning and metabolic function. 🔓 https://t.co/yhTx4bWkUz
— pulmmed_ce (@Pulmmed_CE) October 14, 2022
48) #PAH is a rare, severe, life-threatening disease and management of severe PAH should be done in expert centres. Patients and families can access additional resources and support through advocacy organizations such as @PHAssociation @PHA_UK and @PHACanada.
— pulmmed_ce (@Pulmmed_CE) October 14, 2022
49) And that's it–you made it! Free CE/#CME! Just go to https://t.co/a2QXnvOOIO to claim your credit. And please FOLLOW @pulmmed_CE more many new expert-authored! I am @AlbertaPHdoc.@HelpMyBreathing @jeanlucvachiery @SandeepSahayMD @wginsing @LucillaPiccari #FOAMed
— pulmmed_ce (@Pulmmed_CE) October 14, 2022