Consider escalating or switching therapy if patients are not at low risk2,3
Risk status is a useful measure of2,4:
Your patient’s PAH at baseline, and what time their initial treatment should be
Whether their PAH is getting better, staying the same, or getting worse
Whether their treatment plan needs to be adjusted
If patients do not reach low-risk status within 1 year of diagnosis, they may not reach it5
An explorative study using a 3-strata model of risk assessment found that, of patients who were at intermediate-risk status 1 year after diagnosis, only 12% achieved low-risk status by 3 years.5*
| Risk Parameters | Low-risk treatment goals |
|---|---|
| WHO FC | FC I or II |
| 6MWD | >440m |
| NT-proBNP/BNP | <300 ng/L / <50 ng/L |
| Hemodynamics |
RAP <8 mm Hg CI ≥2.5 L/min/m2 SvO2 >65% |
| Echo |
RA area <18cm2 No pericardial effusion |
2024 WSPH recommendations support adding inhaled prostacyclin therapy for patients as early as intermediate-low risk status.3
*503 patients with PAH were stratified by risk status using the SPAHR method at baseline and follow-up yearly appointments. Risk assessment was based on WHO FC, 6MWD, NT-proBNP, RA area, mRAP, CI, SvO2, and pericardial effusion. Results are taken from the main cohort of patients aged ≤75 years with <3 comorbidities (n=340).5
6MWD=6-minute walk distance; CI=cardiac index; Echo=echocardiogram; FC=functional class; mRAP=mean right atrial pressure; NT-proBNP=N-terminal pro-B-type natriuretic peptide; RA=right atrial/right atrium; SPAHR=Swedish Pulmonary Arterial Hypertension Registry; SvO2=mixed venous oxygen saturation; WHO=World Health Organization; WSPH=World Symposium on Pulmonary Hypertension.