What is pulmonary arterial hypertension and why does risk assessment matter? Pulmonary arterial hypertension is a rare, serious disease where the blood vessels in the
lungs become too narrow. This makes the right side of the heart work harder, which over
time can lead to heart failure.
Because it can progress quickly, doctors use risk assessment (sometimes called “risk stratification”) to:
- Understand how severe the disease is
- Predict how it may develop
- Guide treatment decisions
- Plan for advanced options, such as transplant or palliative care
- The goal for every patient is to reach and stay in the low-risk group, which is linked to longer
- survival and better quality of life.
How does risk assessment work?
- Done at diagnosis and then every 3–6 months
- Looks at many factors: age, sex, cause, exercise ability, symptoms, blood tests, imaging, and heart catheter results
- Even early changes (after 2–3 months of treatment) can be important signals
Key risk factors:
- Age & sex: Older patients and men over 60 tend to do worse.
- Cause of pulmonary arterial hypertension:
- Systemic sclerosis and portal hypertension = poorer outcomes
- HIV-associated PAH depends on immune health
- Congenital heart disease varies by type and age
- Physical ability & symptoms: Better function = better outlook
Main tests:
- Exercise tests: 6-minute walk test is widely used; wearables and advanced tests are being explored
- Biomarkers (blood tests): BNP/NT-proBNP are most useful; higher levels mean the heart is under strain
- Imaging: Echocardiography (widely available) and MRI (very detailed but less available) show right heart function
- Hemodynamics (heart catheterization): Measures like right atrial pressure, cardiac output, and SvO₂ give detailed information about heart function
- Genetics: Inherited changes (like BMPR2 mutations) increase risk and often causeearlier, more severe disease
Modern risk scores – Doctors now combine different test results into risk scoring systems:
UNITED STATES
- REVEAL 2.0: very detailed, includes age, hospitalizations, kidney function, and more
- REVEAL Lite 2: simpler, uses 6 easy measures
- PHORA: uses artificial intelligence to improve predictions
EUROPE
- ESC/ERS (2015): 3 categories (low, intermediate, high)
- French model: counts how many low-risk features a patient has
- COMPERA: moved from 3 to 4 categories (intermediate-low and intermediate-high for more detail)
- SPAHR (Sweden): refined further with imaging
- 6-strata systems: newest models for even more precision
- The main goal across all systems: help patients reach low risk.
Why “intermediate risk” is tricky:
- Many patients fall into this group, which is too broad
- Newer tools now split it into intermediate-low and intermediate-high to guide better treatment decisions
Special topics:
- Right heart function matters most → Echo and MRI of the right ventricle are becoming central in risk scores
- Clinical trials: Risk scores may replace older trial endpoints (like just walk distance),
- helping new treatments get tested and approved faster
- Children: Pediatric pulmonary arterial hypertension is different. Growth, development, and causes like congenital heart disease must be included.
- New child-specific models are urgently needed.
Consensus highlights (what experts agree on):
- Risk assessment should be done at baseline and follow-up
- Doctors should combine risk tools + clinical judgment
- Some groups (men >60, systemic sclerosis, portal hypertension) have higher risk
- BNP/NT-proBNP and echocardiography are key routine tools
- Hemodynamics still play an important role, especially at follow-up
- French, COMPERA, and REVEAL scores are widely used, but must follow current
- guidelines
- Pediatric, genetic, and biomarker-based tools are future priorities
- Risk scores could speed up clinical trials and access to treatments
Key messages for patients and advocates:
- Risk assessment is central in pulmonary hypertension care: it guides treatment, predicts survival, and tracks progress
- Multiple tools exist, but all aim for the same thing: keeping patients in the low-risk group
- Blood tests, imaging, and heart catheter results are the main building blocks today
- Children need their own scoring system that reflects growth and development
- Research is moving fast: genetics, biomarkers, and AI may bring more accurate and personalized risk tools in the future
Bottom line
- Risk assessment helps doctors and patients make better, more timely decisions.
- The sooner patients reach and maintain low risk, the better their chances of living
- longer and with improved quality of life.
Summary by Louise Bouman, volunteer and Member of the Board with the Alliance for Pulmonary Hypertension
Citation
Sahay, Sandeep et al. International Society for Heart and Lung Transplantation (ISHLT)
Consensus Statement on Risk Stratification in Pulmonary Arterial Hypertension. The Journal of Heart and Lung Transplantation – September 05, 2025
https://www.jhltonline.org/article/S1053-2498(25)01951-5/fulltext
See also key takeways at this link on the LinkedIn page of the International Society for Heart and Lung Transplant
Download pdf with full text at this link