WHO PH Group 1 subgroup | | | | | APAH-CTD | Heritable PoPH (+3) |
Demographics | | | | | | Male, age >60 years |
Clinical signs of right heart failure | Absent | Absent | Present | | | |
Comorbidities | | | | | eGFR<60 mL·min−1/1.73 m2 or renal insufficiency | |
Symptom progression | No | Slow | Rapid | | | |
Vital signs | | | | | SBP <110 mmHg HR >96 beats·min−1 | |
Syncope | No | Occasional syncope | Repeated syncope | | | |
All-cause hospitalisations ≤6 months | | | | | ≥1 | |
NYHA/WHO FC | I, II | III | IV | I | III | IV |
6MWD | >440 m | 165–440 m | <165 m | ≥440 m (−2) 320–<440 m | <165 m | |
CPET | Peak V′O2 >15 mL·min−1·kg−1 (>65% pred) V′E/V′CO2 slope <36 | Peak V′O2 11–15 mL·min−1·kg−1 (35–65% pred) V′E/V′CO2 slope 36–44.9 | Peak V′O2 <11 mL·min−1·kg−1 (<35% pred) V′E/V′CO2 slope ≥44.9 | | | |
BNP/NT-proBNP plasma levels | BNP <50 ng·L−1 NT-proBNP <300 ng·L−1 | BNP 50–300 ng·L−1 NT-proBNP 300–1400 ng·L−1 | BNP >500 ng·L−1 NT-proBNP >1400 ng·L−1 | BNP<50 ng·L−1 NT-proBNP <300 ng·L−1 | BNP 200–800 ng·L−1 | BNP ≥800 ng·L−1 NT-proBNP ≥1100 ng·L−1 |
Imaging/echocardiography | RA area <18 cm2 No pericardial effusion | RA area 18–26 cm2 No or minimal pericardial effusion | RA area >26 cm2 Pericardial effusion | | Pericardial effusion | |
Haemodynamics/right heart catheterisation | RAP <8 mmHg CI ≥2.5 L/min/m2 SvO2 >65% | RAP 8–14 mmHg CI 2.0–2.4 L/min/m2 SvO2 60–65% | RAP >14 mmHg CI <2.0 L/min/m2 SvO22 <60% | PVR <5 Wood units | Mean RAP >20 mmHg within 1 year | |
Pulmonary function test | | | | | DLCO <40% pred | |