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PAH GUIDELINES AND RIGHT VENTRICULAR FUNCTION
Recent guideline updates place a greater emphasis on the heart1-3
Recent updates to risk assessment parameters indicate a rising importance of right ventricular function to help evaluate risk more accurately from diagnosis. REVEAL Lite 2, 2022 ESC/ERS Guidelines, and 2024 WSPH Consensus Statements emphasize the need for and use of echo and/or RHC as effective tools for assessing RV function and its effect on PAH progression.1-3
Patients can present signs of progression in unique ways, and incomplete assessments can lead to an underestimation of mortality risk. For some patients, risk level can be masked by how physically active they are, so frequent clinical assessments, particularly of the right heart, are crucial to identify disease progression early.1,4
Explore the 2024 WSPH Consensus Statements.
Take a closer look at the updated clinical parameters in the 2022 ESC/ERS Guidelines.
*Article may be restricted by a paywall for nonsubscribers.
Recognizing signs of RV dysfunction with RHC and echocardiography1,5-9
RHC is required for a definitive PAH diagnosis. By directly measuring essential hemodynamic markers, RHC provides an accurate assessment of disease severity and informs appropriate patient management. Echocardiography can be a useful tool for monitoring the RV and indicators of progression.
Parameters to diagnose or manage PAH1,10,11
| Parameter | Normal range | Suggestive/ Diagnostic of PAH |
|---|---|---|
| PVR | 0.3–2.0 WU | >2 WU |
| mPAP | 8–20 mm Hg | >20 mm Hg |
| RAP | 2–6 mm Hg | >6 mm Hg |
| PCWP | ≤15 mm Hg | ≤15 mm Hg |
| CI | 2.5–4.0 L/min·m2 | <2.0 L/min·m2 |
| TAPSE | >1.7 cm | ≤1.7 cm |
| TAPSE/sPAP | >0.32 mm/mm Hg | <0.32 mm/mm Hg |
| RA Area | <18 cm2 | >18 cm2 |
RV changes occur before other indicators of progression12
Detrimental changes in right ventricular function can happen in as little as 12 weeks and occur before other indicators of progression, such as FC and 6MWD. Changes in RV function are also the first warning sign on the path to potential hospitalization.12,13
LEADING INDICATOR
Right ventricular function
LAGGING INDICATORS
Functional class and 6-minute walk distance
Echocardiograms can be a useful tool for helping assess RV function1
Echocardiograms can help you assess your patient’s RV function noninvasively and may help you identify a worsening prognosis.14 This is true even if parameters such as FC and 6MWD show signs of improvement.15
RIGHT HEART ASSESSMENT MILESTONES
Guidelines recommend echocardiography and RHC at least every 6 months and as early as 3 months after a change in therapy.1†
†Intervals may need to be adjusted according to patient needs.1
6MWD=6-minute walk distance; CI=cardiac index; ERS=European Respiratory Society; ESC=European Society of Cardiology; FC=functional class; mPAP=mean pulmonary arterial pressure; NT-proBNP=N-terminal pro–B-type natriuretic peptide; PAH=pulmonary arterial hypertension; PCWP=pulmonary capillary wedge pressure; PVR=pulmonary vascular resistance; RA=right atrial; RAP=right atrial pressure; REVEAL=Registry to EValuate Early And Long-term PAH disease management; RHC=right heart catheterization; RV=right ventricle/right ventricular; sPAP=systolic pulmonary arterial pressure; TAPSE=tricuspid annular plane systolic excursion; WSPH=World Symposium on Pulmonary Hypertension; WU=Wood unit.
Remodulin® (treprostinil) Injection
Important Safety Information for Remodulin
Warnings and Precautions
- Chronic intravenous (IV) infusions of Remodulin delivered using an external infusion pump with an indwelling central venous catheter are associated with the risk of blood stream infections (BSIs) and sepsis, which may be fatal. Therefore, continuous subcutaneous (SC) infusion is the preferred mode of administration.
- Avoid abrupt withdrawal or sudden large reductions in dosage of Remodulin, which may result in worsening of PAH symptoms.
- Titrate slowly in patients with hepatic insufficiency, because such patients will likely be exposed to greater systemic concentrations relative to patients with normal hepatic function.
- Remodulin is a pulmonary and systemic vasodilator. In patients with low systemic arterial pressure, treatment with Remodulin may produce symptomatic hypotension.
- Remodulin inhibits platelet aggregation and increases the risk of bleeding.
Adverse Reactions
- In clinical studies of SC Remodulin infusion, the most common adverse events reported were infusion site pain and infusion site reaction (redness, swelling, and rash). These symptoms were sometimes severe and sometimes required treatment with narcotics or discontinuation of Remodulin. The IV infusion of Remodulin with an external infusion pump has been associated with a risk of blood stream infections, arm swelling, paresthesias, hematoma, and pain. Other common adverse events (≥3% more than placebo) seen with either SC or IV Remodulin were headache (27% vs. 23%), diarrhea (25% vs. 16%), nausea (22% vs. 18%), rash (14% vs. 11%), jaw pain (13% vs. 5%), vasodilatation (11% vs. 5%), edema (9% vs. 3%), and hypotension (4% vs. 2%).
Drug Interactions
- Remodulin dosage adjustment may be necessary if inhibitors or inducers of CYP2C8 are added or withdrawn.
Specific Populations
- In patients with mild or moderate hepatic insufficiency, decrease the initial dose of Remodulin to 0.625 ng/kg/min of ideal body weight, and monitor closely. Remodulin has not been studied in patients with severe hepatic insufficiency.
- Safety and effectiveness of Remodulin in pediatric patients have not been established.
- It is unknown if geriatric patients respond differently than younger patients. Caution should be used when selecting a dose for geriatric patients.
- There are no adequate and well-controlled studies with Remodulin in pregnant women. It is not known whether treprostinil is excreted in human milk or if it affects the breastfed infant or milk production.
Indication
Remodulin is a prostacyclin vasodilator indicated for the treatment of pulmonary arterial hypertension (PAH; WHO Group 1) to diminish symptoms associated with exercise. Studies establishing effectiveness included patients with NYHA Functional Class II-IV symptoms and etiologies of idiopathic or heritable PAH (58%), PAH associated with congenital systemic-to-pulmonary shunts (23%), or PAH associated with connective tissue diseases (19%).
In patients with PAH requiring transition from epoprostenol, Remodulin is indicated to diminish the rate of clinical deterioration. Consider the risks and benefits of each drug prior to transition.
REMISIhcpFEB2025
Please see accompanying Full Prescribing Information for Remodulin.
For additional information, visit www.RemodulinPro.com or call Customer Service at 1-844-UNITHER (1-844-864-8437).
For additional information, visit www.RemodulinPro.com or call Customer Service at 1-844-UNITHER (1-844-864-8437).
Remodulin® (treprostinil) Injection
Important Safety Information for Remodulin
Warnings and Precautions
- Chronic intravenous (IV) infusions of Remodulin delivered using an external infusion pump with an indwelling central venous catheter are associated with the risk of blood stream infections (BSIs) and sepsis, which may be fatal. Therefore, continuous subcutaneous (SC) infusion is the preferred mode of administration.
- Avoid abrupt withdrawal or sudden large reductions in dosage of Remodulin, which may result in worsening of PAH symptoms.
- Titrate slowly in patients with hepatic insufficiency, because such patients will likely be exposed to greater systemic concentrations relative to patients with normal hepatic function.
- Remodulin is a pulmonary and systemic vasodilator. In patients with low systemic arterial pressure, treatment with Remodulin may produce symptomatic hypotension.
- Remodulin inhibits platelet aggregation and increases the risk of bleeding.
Adverse Reactions
- In clinical studies of SC Remodulin infusion, the most common adverse events reported were infusion site pain and infusion site reaction (redness, swelling, and rash). These symptoms were sometimes severe and sometimes required treatment with narcotics or discontinuation of Remodulin. The IV infusion of Remodulin with an external infusion pump has been associated with a risk of blood stream infections, arm swelling, paresthesias, hematoma, and pain. Other common adverse events (≥3% more than placebo) seen with either SC or IV Remodulin were headache (27% vs. 23%), diarrhea (25% vs. 16%), nausea (22% vs. 18%), rash (14% vs. 11%), jaw pain (13% vs. 5%), vasodilatation (11% vs. 5%), edema (9% vs. 3%), and hypotension (4% vs. 2%).
Drug Interactions
- Remodulin dosage adjustment may be necessary if inhibitors or inducers of CYP2C8 are added or withdrawn.
Specific Populations
- In patients with mild or moderate hepatic insufficiency, decrease the initial dose of Remodulin to 0.625 ng/kg/min of ideal body weight, and monitor closely. Remodulin has not been studied in patients with severe hepatic insufficiency.
- Safety and effectiveness of Remodulin in pediatric patients have not been established.
- It is unknown if geriatric patients respond differently than younger patients. Caution should be used when selecting a dose for geriatric patients.
- There are no adequate and well-controlled studies with Remodulin in pregnant women. It is not known whether treprostinil is excreted in human milk or if it affects the breastfed infant or milk production.
Indication
Remodulin is a prostacyclin vasodilator indicated for the treatment of pulmonary arterial hypertension (PAH; WHO Group 1) to diminish symptoms associated with exercise. Studies establishing effectiveness included patients with NYHA Functional Class II-IV symptoms and etiologies of idiopathic or heritable PAH (58%), PAH associated with congenital systemic-to-pulmonary shunts (23%), or PAH associated with connective tissue diseases (19%).
In patients with PAH requiring transition from epoprostenol, Remodulin is indicated to diminish the rate of clinical deterioration. Consider the risks and benefits of each drug prior to transition.
REMISIhcpFEB2025
Please see accompanying Full Prescribing Information for Remodulin.
For additional information, visit www.RemodulinPro.com or call Customer Service at 1-844-UNITHER (1-844-864-8437).
References: 1. Humbert M, Kovacs G, Hoeper MM, et al; ESC/ERS Scientific Document Group. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3618-3731. doi:10.1093/eurheartj/ehac237. 2. Sahay S, Bhatt J, Beshay S, et al. E‐REVEAL Lite 2.0 scoring for early prediction of disease progression in pulmonary arterial hypertension. Pulm Circ. 2022;12(1):e12026. doi:10.1002/pul2.12026. 3. Chin KM, Gaine SP, Gerges C, et al. Treatment algorithm for pulmonary arterial hypertension. Eur Respir J. 2024;64(4):2401325. Published 2024 Oct 31. doi:10.1183/13993003.01325-2024. 4. Sahay S, Tonelli AR, Selej M, et al. Risk assessment in patients with functional class II pulmonary arterial hypertension: comparison of physician gestalt with ESC/ERS and the REVEAL 2.0 risk score. PLoS One. 2020;15(11):e0241504. doi:10.1371/journal.pone.0241504. 5. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension. Rev Esp Cardiol (Engl Ed). 2016;69(2):177. doi:10.1016/j.rec.2016.01.002. 6. Kovacs G, Bartolome S, Denton CP, et al. Definition, classification and diagnosis of pulmonary hypertension. Eur Respir J. 2024;64(4):2401324. Published 2024 Oct 31. doi:10.1183/13993003.01324-2024. 7. Barst RJ, McGoon M, Torbicki A, et al. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43(12 Suppl S):40S-47S. doi:10.1016/j.jacc.2004.02.032. 8. Lang RM, Badano LP, Mor-Avi V, et al. Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2015;28(1):1-39.e14. doi:10.1016/j.echo.2014.10.003. 9. Topyła-Putowska W, Tomaszewski M, Wysokiński A, Tomaszewski A. Echocardiography in Pulmonary Arterial Hypertension: Comprehensive Evaluation and Technical Considerations. J Clin Med. 2021;10(15):3229. Published 2021 Jul 22. doi:10.3390/jcm10153229. 10. Pulmonary Hypertension. Thoracic Key website. https://thoracickey.com/pulmonary-hypertension-37/. Published February 15, 2025. Accessed May 2, 2025. 11. Mukherjee M, Rudski LG, Addetia K, et al. Guidelines for the Echocardiographic Assessment of the Right Heart in Adults and Special Considerations in Pulmonary Hypertension: Recommendations from the American Society of Echocardiography. J Am Soc Echocardiogr. 2025;38(3):141-186. doi:10.1016/j.echo.2025.01.006. 12. Milks MW, Sahay S, Benza RL, et al. Risk assessment in patients with pulmonary arterial hypertension in the era of COVID 19 pandemic and the telehealth revolution: state of the art review. J Heart Lung Transplant. 2021;40(3):172-182. doi:10.1016/j.healun.2020.12.005. 13. Simonneau G, Barst RJ, Galie N, et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med. 2002;165(6):800-804. doi:10.1164/ajrccm.165.6.2106079. 14. Truong U, Meinel K, Haddad F, et al. Update on noninvasive imaging of right ventricle dysfunction in pulmonary hypertension. Cardiovasc Diagn Ther. 2020;10(5):1604-1624. doi:10.21037/cdt-20-272. 15. van de Veerdonk MC, Marcus JT, Westerhof N, et al. Signs of right ventricular deterioration in clinically stable patients with pulmonary arterial hypertension. Chest. 2015;147(4):1063-1071. doi:10.1378/chest.14-0701.
