Pulmonary Disorders II

PAH risk stratification is a multiparametric risk assessment tool approach that is recommended for

quantifying the severity of disease, predicting patient outcomes, and guiding the treatment of PAH.

The most predictive prognostic factors are 3 noninvasive parameters (WHO functional class [Table 3],

6MWD, and natriuretic peptides) that are included in all currently validated risk stratification tools.

The European Society of Cardiology/European Respiratory Society (ECS/ERS) and REVEAL

methods are the 2 risk stratification models recommended by guidelines. Each model stratifies a

patient at diagnosis as low-, intermediate-, or high-risk status. On reassessment, a 4-stratification

system is used: low-, intermediate-low, intermediate-high, or high-risk status. Risk categories are

associated with 1-year mortality rates.

The ECS/ERS 3-strata (Table 4) or REVEAL 2.0 models are recommended to be used at diagnosis

at baseline.

The ECS/ERS 4-strata (Table 5) or REVEAL Lite models are recommended to be used at 3- to

4-month intervals to assess a patient’s response to therapy.

Class

Definition

I

No symptoms (dyspnea, fatigue, syncope, chest pain) with normal activities

II

Symptoms with strenuous normal daily activities that slightly limit functional status and

activity level; no symptoms at rest

III

Symptoms of dyspnea, fatigue, syncope, and chest pain with normal daily activities that

severely limit functional status and activity level; no symptoms at rest

IV

Symptoms at rest; cannot perform normal daily activities without symptoms

aHumbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022;43:3618-731.

Multivariable Assessment of Mortality Risk to Guide Initial Therapy

Assessment

3-Risk Strata

Estimation of 1-year

mortality

Low Risk < 5%

Intermediate Risk 5%–20%

High Risk > 20%

Points assigned per

variablea

1 point

2 points

3 points

Signs of right heart

failure

No

No

Yes

Progression of

symptoms and clini-

cal manifestations

No

Slow

Rapid

Syncope

No

Occasional during strenuous

exercise

Repeated with little or normal

physical activity

WHO-FC

I, II

III

IV

6-Minute walk

distance

> 440 m

165–440 m

< 165 m

Cardiopulmonary

exercise testing

Peak VO2 > 15

mL/min/kg (> 65%

predicted) VE/VCO2

slope < 36

Peak VO2 11–15

mL/min/kg (35%–65%

predicted) VE/VCO2

slope 36–44

Peak VO2 < 11 mL/min/kg

(< 35% predicted) VE/VCO2

slope > 44