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PAH Disease Progression & Risk

PAH Disease Progression Makes It
Essential to Evaluate Risk Regularly1-3

Risk at Diagnosis

Most Patients Are at Intermediate Risk at Diagnosis1

In an analysis of the prospective COMPERA registry, 3-strata risk assessment was evaluated at baseline and 4-strata at follow-up for newly diagnosed patients with PAH.1

THE FIRST YEAR AFTER DIAGNOSIS IS CRITICAL.

Risk Status at Diagnosis (N=1588)1

%

Intermediate
Risk

15% High Risk (n=256)
76% Intermediate Risk (n=1257)
9% Low Risk (n=142)

Regular Risk, Dose, and Right Heart Assessments Are Essential to Stay Ahead of PAH Progression

PAH Progression is Not Always Visible and Right Heart Function Can Offer Early Clues2,3

While symptoms may appear stable, many patients experience underlying changes in right heart function that may go undetected without regular assessment.3

The ESC/ERS Guidelines recommend regular, multi-parametric assessments including right heart imaging and hemodynamic evaluation every 3-6 months or after any change in therapy.2,4,5

Prioritize Regular Risk and Dose Assessments to Get Ahead of Unpredictable Deterioration5

Key recommendations include:

Frequent risk assessment
(every 3-4 months)

Define clinical time-based goals

Regular dose assessments and therapy adjustments

Regular Right Heart Assessment is Essential5

PAH guidelines recommend:

Set hemodynamic goals and assess them at follow-up

Respond to changes in the right heart

Consistent use of risk assessment tools matters.6–9 Patients within the vast intermediate risk category may benefit from 4-strata assessments at follow-up, which allow for more granular decision-making.3

4-Strata Model Enables Earlier Identification of Patients Who Are Not at Goal or Are Declining4

Refined Cutoff Levels for 3- to 6-Month Follow-Up4

Make Timely Treatment Decisions for Your Intermediate Risk Patients to Achieve Low Risk 1,4

Evaluate risk levels to drive informed clinical decisions.

Risk status at first follow-up (median 4.1 months) from COMPERA registry (N=1414)1

~
%

were at intermediate risk at first follow up

17% High Risk (n=245)
28% Intermediate-Low Risk (n=395)
38% Intermediate-High Risk (n=534)
17% Low Risk (n=240)
“The intermediate risk group of PAH can be tricky because many of our intermediate risk patients will, on the surface, look relatively stable.”
—A PAH expert
Visit the PAH Initiative website to access risk score calculators and to download the app.
Visit Now

Start With the Heart

Discover how using right heart imaging and RHC every 3 to 6 months may help better define the needs of your intermediate risk patients sooner than other indicators.3,5

Learn about Moderate RV Dysfunction

6MWD=6-minute walk distance; BNP=B-type natriuretic peptide; CI=cardiac index; FC=functional class; mPAP=mean pulmonary arterial pressure; NT-proBNP=N-terminal pro–B-type natriuretic peptide; PAH=pulmonary arterial hypertension; PAWP=pulmonary arterial wedge pressure; PVR=pulmonary vascular resistance; RAP=right atrial pressure; RHC=right heart catheterization; RV=right ventricle/right ventricular; SvO2=mixed venous oxygen saturation; WHO=World Health Organization; WU=Wood units.

IMPORTANT SAFETY INFORMATION

Contraindications

  • Avoid use of Orenitram in patients with severe hepatic impairment (Child Pugh Class C) due to increases in systemic exposure.

IMPORTANT SAFETY INFORMATION FOR ORENITRAM

Contraindications

  • Avoid use of Orenitram in patients with severe hepatic impairment (Child Pugh Class C) due to increases in systemic exposure.

Adverse Reactions

  • In the 12-week, placebo-controlled, monotherapy study, and an event-driven placebo-controlled, combination therapy study, adverse reactions that occurred at rates at least 5% higher on Orenitram than on placebo included headache, diarrhea, nausea, vomiting, flushing, pain in jaw, pain in extremity, hypokalemia, abdominal discomfort, and upper abdominal pain.

Warnings and Precautions

  • Abrupt discontinuation or sudden large reductions in dosage of Orenitram may result in worsening of PAH symptoms.
  • The Orenitram tablet shell does not dissolve. In patients with diverticulosis, Orenitram tablets can lodge in a diverticulum.

Drug Interactions

  • Co-administration of Orenitram and the CYP2C8 enzyme inhibitor gemfibrozil increases exposure to treprostinil; therefore, Orenitram dosage reduction may be necessary in these patients.

Specific Populations

  • Animal reproductive studies with Orenitram have shown an adverse effect on the fetus. There are no adequate and well-controlled studies with Orenitram in pregnant women.
  • It is not known whether treprostinil is excreted in human milk or if it affects the breastfed infant or milk production.
  • Safety and effectiveness of Orenitram in pediatric patients have not been established.
  • Use of Orenitram in patients aged 65 years and over demonstrated slightly higher absolute and relative adverse event rates compared to younger patients. Caution should be used when selecting a dose for geriatric patients.
  • There is a marked increase in the systemic exposure to treprostinil in hepatically impaired patients.

INDICATION

Orenitram is a prostacyclin mimetic indicated for treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to delay disease progression and to improve exercise capacity. The studies that established effectiveness included predominately patients with WHO functional class II-III symptoms and etiologies of idiopathic or heritable PAH (66%) or PAH associated with connective tissue disease (26%).

OREISIhcpOCT19

Please see Full Prescribing Information and Patient Information at www.orenitram.com or call 1-877-UNITHER (1-877-864-8437).

References: 1. Hoeper MM, Pausch C, Olsson KM, et al. COMPERA 2.0: a refined four-stratum risk assessment model for pulmonary arterial hypertension. Eur Respir J. 2022;60(1):2102311. 2. 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. 3. Champion HC, Michelakis ED, Hassoun PM. Comprehensive invasive and noninvasive approach to the right ventricle-pulmonary circulation unit: state of the art and clinical and research implications. Circulation. 2009;120(11):992-1007. doi:10.1161/CIRCULATIONAHA.106.674028 4. 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. 5. Chin KM, Gaine SP, Gerges C, et al. Treatment algorithm for pulmonary arterial hypertension. Eur Respir J. 2024 Oct 31;64(4):2401325. 6. Benza RL, Gomberg-Maitland M, Elliott CG, et al. Predicting survival in patients with pulmonary arterial hypertension: the REVEAL risk score calculator 2.0 and comparison with ESC/ERS-based risk assessment strategies. Chest. 2019;156(2):323-337. 7. Benza RL, Kanwar MK, Raina A, et al. Development and validation of an abridged version of the REVEAL 2.0 risk score calculator, REVEAL Lite 2, for use in patients with pulmonary arterial hypertension. Chest. 2021;159(1):337-346. 8. Boucly A, Savale L, Jaïs X, et al. Association between initial treatment strategy and longterm survival in pulmonary arterial hypertension. Am J Respir Crit Care Med. 2021;204(7):842-854. 9. Simons JE, Mann EB, Pierozynski A. Assessment of risk of disease progression in pulmonary arterial hypertension: insights from an international survey of clinical practice. Adv Ther. 2019;36(9):2351-2363.