TABLE 6

Evolution of pulmonary arterial hypertension (PAH) patients at last follow-up according to risk assessment from the REVEAL 2.0 score, the European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines risk stratification tool and the simplified French PAH risk assessment

	Patient 1	Patient 2^#	Patient 3	Patient 4	Patient 5
Delay between diagnosis and assessment months	71	69	5	6	5
PAH therapies	ERA, PDE5i	PDE5i	ERA, PDE5i	ERA, PDE5i	ERA, PDE5i
REVEAL 2.0 score [23]
Score at diagnosis	2	3	8	4	5
Score at last follow-up	4 (low risk)	4 (low risk)	5 (low risk)	2 (low risk)	3 (low risk)
ESC/ERS guidelines [2]^¶
Signs of right heart failure	No	No	No	No	No
Progression of symptoms	No	No	No	No	No
Syncope	No	No	No	No	No
NYHA FC	II	I	II	I	II
6MWD m	587	358	454	490	533
CPET
V′_O₂ at peak mL·min⁻¹·kg⁻¹ (% pred)	NA	NA	NA	15 (81)	25 (76)
V′_E/V′_CO₂ slope	NA	NA	NA	40	32
NT-proBNP ng·mL⁻¹	96	234	66	92	113
Echocardiography
RA area cm²	<18	<18	NA	<18	<18
Pericardial effusion	No	No	NA	No	No
Haemodynamics
RAP mmHg	6	7	5	4	11
Cardiac index L·min⁻¹·m⁻²	2.57	2.56	2.83	2.42	2.68
S_vO₂ %	71	68	73	71	71
Simplified risk assessment [22]
NYHA FC I/II	Yes	Yes	Yes	Yes	Yes
6MWD >440 m	Yes	No	Yes	Yes	Yes
RAP <8 mmHg	Yes	Yes	Yes	Yes	No
Cardiac index ≥2.5 L·min⁻¹·m⁻²	Yes	Yes	Yes	No	Yes
Low-risk criteria n	4	3	4	3	4

Data are presented as n. ERA: endothelin receptor antagonist; PDE5i: phosphodiesterase type 5 inhibitor; NYHA FC: New York Heart Association Functional Class; 6MWD: 6-min walk distance; CPET: cardiopulmonary exercise testing; V′_O₂: oxygen consumption; V′_E: minute ventilation; V′_CO₂: carbon dioxide production; NT-proBNP: N-terminal pro-brain natriuretic peptide; RA: right atrium; RAP: right atrial pressure; S_vO₂: mixed venous oxygen saturation; NA: not available. ^#: for patient 2, haemodynamic reassessment was performed at 6 months of follow-up; ^¶: green indicates “low risk” and yellow indicates “intermediate risk” according to the ESC/ERS guidelines.