2) Be sure to check out our prior #tweetorial on #PH by @SudarRajagopal, still available for credit, at https://t.co/gBs1y4NlNF. Hello to @SashaPrisco @mark_toshner @charifa_PVRI @SteveMathaiMD @rjbernardoMD @heresi_gustavo @PGeorgeMD @RyanTedfordMD @AthenaisBoucly @SarahMedrek
— cardio-met (@cardiomet_CE) December 20, 2021
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.
— cardio-met (@cardiomet_CE) December 20, 2021
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)
— cardio-met (@cardiomet_CE) December 20, 2021
8) Answer: B. The presence of dyspnea, isolated low DLCO, syncope and enlarged pulmonary artery suggests pulmonary hypertension (PH). An echocardiogram is recommended to assess the probability of PH, which guides additional testing and referral. https://t.co/bCm15HGCff pic.twitter.com/hNupaT7RMu
— cardio-met (@cardiomet_CE) December 20, 2021
10) What is the likelihood of pulmonary hypertension based on these echo findings?
— cardio-met (@cardiomet_CE) December 20, 2021
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.
— cardio-met (@cardiomet_CE) December 20, 2021
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.
— cardio-met (@cardiomet_CE) December 20, 2021
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
— cardio-met (@cardiomet_CE) December 20, 2021
18) So let’s recap. We have a 35 F 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/2sIRusat0u
— cardio-met (@cardiomet_CE) December 21, 2021
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?
— cardio-met (@cardiomet_CE) December 21, 2021
22) What initial therapy would you start her on?
— cardio-met (@cardiomet_CE) December 21, 2021
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.https://t.co/MF6M6lEiXB pic.twitter.com/2yu9f2pU3S
— cardio-met (@cardiomet_CE) December 21, 2021
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…)
— cardio-met (@cardiomet_CE) December 21, 2021
28) Intravenous #prostacyclins include epoprostenol (Flolan®, Veletri® or Caripul®) & treprostinil (Remodulin®). These meds are complicated. They require an indwelling central venous catheter, small battery-powered pump, & frequent mixing of vials by the patient or caregiver.
— cardio-met (@cardiomet_CE) December 21, 2021
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/bsJZ8jrVwB pic.twitter.com/mVIphbpLbR
— cardio-met (@cardiomet_CE) December 21, 2021
32) Please mark your answer and come back tomorrow for the correct answer, more education, and a link to grab your FREE CE/#CME! The holidays are upon us–get your 2021 credit NOW! @SashaPrisco @mark_toshner @charifa_PVRI @SteveMathaiMD @rjbernardoMD @heresi_gustavo @jeffminMD
— cardio-met (@cardiomet_CE) December 21, 2021
34) So there was a poll (tweet # 31) yesterday. Scroll back up and vote, if you haven't already!! @RyanTedfordMD @SandeepSahay @docroham @anjalivaidyaMD @RogerAlvarezDO @kurt_prins @JimWhiteCurePAH @OSitbon @ATS_PC @PVRI @Viniciodjperez @Montanidavid @jeanlucvachiery
— cardio-met (@cardiomet_CE) December 22, 2021
36) After 6 months, she wants to discuss becoming pregnant. What should you recommend to her about pregnancy and contraception?
— cardio-met (@cardiomet_CE) December 22, 2021
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.
— cardio-met (@cardiomet_CE) December 22, 2021
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:
— cardio-met (@cardiomet_CE) December 22, 2021
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.
— cardio-met (@cardiomet_CE) December 22, 2021
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?
— cardio-met (@cardiomet_CE) December 22, 2021
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. #Metabolicsyndrome and #insulinresistance are also frequent in PAH. Exercise can empower patients, improve conditioning and metabolic function. https://t.co/4gGerxQkUp
— cardio-met (@cardiomet_CE) December 22, 2021
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.
— cardio-met (@cardiomet_CE) December 22, 2021
49) And that's it–you made it! Free CE/#CME! Just go to https://t.co/ENjVvnCxPG to claim your credit. And please FOLLOW @cardiomet_CE more many new expert-authored programs in 2022! I am @CalgaryPHdoc.@HelpMyBreathing @jeanlucvachiery @SandeepSahayMD @wginsing @LucillaPiccari
— cardio-met (@cardiomet_CE) December 22, 2021