2022 ESC/ERS Guidelines recommend risk assessments for initial diagnosis and follow-up¹

Determinates of initial prognosis used in the 3-strata risk assessment

Signs of right HF

Progression of symptoms and clinical manifestations

Syncope

WHO FC

6MWD*

CPET

Biomarkers:
BNP or NT-proBNP

Echocardiography

cMRI

Hemodynamics

Depending on the severity of the clinical parameters, patients are stratified into low-, intermediate-, and high-risk groups.

The 4-strata risk assessment should be used at follow-up for prognosis

Patients who have been diagnosed with PAH should be assessed every 3 to 6 months

4-strata risk assessment model in PAH

Determinants of prognosis	Low risk	Intermediate-low risk	Intermediate-high risk	High risk
Points assigned	1	2	3	4
WHO FC	I or II^†	-	III	IV
6MWD, m	>440	320-440	165-319	<165
BNP or NT-proBNP, ng/L	<50 <300	50-199 300-649	200-800 650-1100	>800 >1100

Determinants of prognosis	Low risk	Intermediate-low risk
Points assigned	1	2
WHO FC	I or II^†	-
6MWD, m	>440	320-440
BNP or NT-proBNP, ng/L	<50 <300	50-199 300-649

Determinants of prognosis	Intermediate-high risk	High risk
Points assigned	3	4
WHO FC	III	IV
6MWD, m	165-319	<165
BNP or NT-proBNP, ng/L	200-800 650-1100	>800 >1100

Adapted from 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. With permission from the European Society of Cardiology and European Respiratory Society.

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*6MWD is dependent upon age, height, and burden of comorbidities.

^†WHO FC I and II are assigned 1 point as both are associated with good long-term survival.

6MWD=6-minute walk distance; BNP=brain natriuretic peptide; cMRI=cardiac magnetic resonance imaging; CPET=cardiopulmonary exercise testing; ERS=European Respiratory Society; ESC=European Society of Cardiology; FC=Functional Class; HF=heart failure; NT-proBNP=N-terminal pro-brain natriuretic peptide; PAH=pulmonary arterial hypertension; WHO=World Health Organization.

Reference: 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: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Eur Heart J. 2022;43(38):3618-3731.

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2022 ESC/ERS Guidelines recommend risk assessments for initial diagnosis and follow-up1