Figures
- FIGURE 1
Patient selection flow chart. PAH: pulmonary arterial hypertension; 6MWD: 6-min walking distance; WHO: World Health Organization; NYHA: New York Heart Association; FC: functional class; BNP: brain natriuretic peptide; NT-proBNP: N-terminal pro-brain natriuretic peptide.
- FIGURE 2
Number of low-risk criteria (World Health Organization/New York Heart Association functional class I–II; 6-min walking distance >440 m; right atrial pressure <8 mmHg; cardiac index ≥2.5 L·min−1·m−2) present at baseline and first re-evaluation within the first year after diagnosis.
- FIGURE 3
Transplant-free survival according to the number of low-risk criteria (World Health Organization/New York Heart Association functional class I–II I; 6-min walking distance >440 m; right atrial pressure <8 mmHg; cardiac index ≥2.5 L·min−1·m−2) present at a) time of pulmonary arterial hypertension diagnosis; b) first re-evaluation within the first year after diagnosis.
- FIGURE 4
Transplant-free survival according to the number of noninvasive low-risk criteria (World Health Organization/New York Heart Association functional class I–II; 6-min walking distance >440 m; brain natriuretic peptide <50 ng·L−1 or N-terminal pro-brain natriuretic peptide <300 ng·mL−1) present at first re-evaluation (n=603).
Tables
- TABLE 1
Demographics and baseline characteristics of overall and study populations of patients with newly diagnosed idiopathic, heritable and drug-induced pulmonary arterial hypertension (PAH)
Overall population Study population Subjects 1591 1017 Female/male 924 (58)/667 (42) 598 (59)/419 (41) Age years 60±17 57±17 BMI kg·m−2 27.8±7.0 27.7±6.4 BMI >30 kg·m−2 474 (30) 291 (29) PAH diagnosis Idiopathic 1228 (77) 762 (75) Heritable 109 (7) 94 (9) Drug-induced 254 (16) 161 (16) Acute vasodilator responder 139 (8.7) 97 (9.5) WHO/NYHA functional class I–II 441 (28) 261 (26) III 925 (58) 624 (61) IV 225 (14) 132 (13) 6-min walking distance m 307±142 311±145 Haemodynamics RAP mmHg 8.8±5.2 8.6±5.0 mPAP mmHg 48±13 50±13 PAWP mmHg 10±4 9±4 Cardiac output L·min−1 4.4±1.5 4.4±1.4 Cardiac index L·min−1·m−2 2.4±0.8 2.4±0.7 PVR Wood units 9.9±5.6 10.5±5.9 Mean blood pressure mmHg 96±18 95±17 Heart rate beats·min-1 80±16 80±15 SvO2 % 63±10 63±9# Data are presented as n, n (%) or mean±sd. BMI: body mass index; WHO: World Health Organization; NYHA: New York Heart Association; RAP: right atrial pressure; mPAP: mean pulmonary artery pressure; PAWP: pulmonary artery wedge pressure; PVR: pulmonary vascular resistance; SvO2: mixed venous oxygen saturation. #: n=606.
- TABLE 2
Univariable and multivariable Cox regression analysis of low-risk criteria assessed at baseline and first re-evaluation
Univariable analysis Multivariable analysis Hazard ratio (95% CI) p-value Hazard ratio (95% CI) p-value At baseline WHO/NYHA FC I–II 0.59 (0.43–0.82) 0.001 6-min walking distance >440 m 0.29 (0.18–0.46) <0.001 0.31 (0.19–0.50) <0.001 Right atrial pressure <8 mmHg 0.71 (0.55–0.92) 0.009 Cardiac index ≥2.5 L·min−1·m−2 0.76 (0.59–0.97) 0.029 At first re-evaluation WHO/NYHA FC I–II 0.30 (0.23–0.39) <0.001 0.44 (0.34–0.58) <0.001 6-min walking distance >440 m 0.24 (0.17–0.34) <0.001 0.37 (0.26–0.55) <0.001 Right atrial pressure <8 mmHg 0.51 (0.40–0.64) <0.001 0.74 (0.57–0.94) 0.016 Cardiac index ≥2.5 L·min−1·m−2 0.51 (0.40–0.65) <0.001 0.64 (0.50–0.82) <0.001 WHO: World Health Organization; NYHA: New York Heart Association; FC: functional class.
- TABLE 3
Change in functional class (FC), exercise capacity and haemodynamics between baseline and first re-evaluation
Baseline First re-evaluation# p-value WHO/NYHA FC I–II 261 (26) 595 (59) <0.001 III 624 (61) 349 (34) IV 132 (13) 73 (7) 6-min walking distance m 311±145 354±145 <0.001 Haemodymamics RAP mmHg 8.6±5.0 7.5±4.8 <0.001 mPAP mmHg 50±13 44±13 <0.001 Cardiac output L·min−1 4.4±1.4 5.3±1.6 <0.001 Cardiac index L·min−1·m−2 2.4±0.7 2.9±0.8 <0.001 PVR Wood units 10.5±5.9 7.1±4.6 <0.001 SvO2 % 63±10 67±8 <0.001 Data are presented as n (%) or mean±sd, unless otherwise stated. n=1017. WHO: World Health Organization; NYHA: New York Heart Association; RAP: right atrial pressure; mPAP: mean pulmonary artery pressure; PVR: pulmonary vascular resistance; SvO2: mixed venous oxygen saturation. #: 5.6±3.4 months after baseline.
- TABLE 4
Univariable and multivariable Cox regression analysis of low-risk criteria assessed at first re-evaluation in the subset of patients with available brain natriuretic peptide (BNP) or N-terminal pro-brain natriuretic peptide (NT-proBNP) measurements
Univariable analysis Multivariable analysis Hazard ratio (95% CI) p-value Hazard ratio (95% CI) p-value WHO/NYHA FC I–II 0.28 (0.19–0.42) <0.001 0.47 (0.32–0.71) <0.001 6-min walking distance >440 m 0.17 (0.09–0.31) <0.001 0.32 (0.17–0.60) <0.001 BNP <50 ng·L−1 or
NT-proBNP <300 ng·mL−10.21 (0.13–0.34) <0.001 0.31 (0.19–0.52) <0.001 Right atrial pressure <8 mmHg 0.45 (0.31–0.65) <0.001 Cardiac index ≥2.5 L·min−1·m−2 0.44 (0.30–0.65) <0.001 WHO: World Health Organization; NYHA: New York Heart Association; FC: functional class.
Supplementary Material
Please note: supplementary material is not edited by the Editorial Office, and is uploaded as it has been supplied by the author.
Supplementary tables. ERJ-00889-2017_Supplement
Supplementary figure S1: Survival of the overall cohort (n=1591). ERJ-00889-2017_Figure_S1
Supplementary figure S2: Transplant-free survival according to the change in number of low-risk criteria between baseline and first follow-up. Abbreviation: F-up: follow-up. ERJ-00889-2017_Figure_S2
Supplementary figure S3: Transplant-free survival in patients with zero low-risk criteria at follow-up according to the number of high-risk criteria (WHO/NYHA functional class IV, 6-minute walk distance #60;165 m, right atrial pressure <14 mmHg, cardiac index #60;2.0 L/min/m2). ERJ-00889-2017_Figure_S3
Supplementary figure S4: Transplant-free survival according to the number of low-risk criteria (WHO/NYHA FC I-II; 6MWD <440 m; right atrial pressure #60;8 mmHg; cardiac index #8805;2.5 L/min/m2, SvO2 <65%) present at first re-evaluation (n=606). Abbreviations: WHO/NYHA FC: World Health Organization/New York Heart Association functional class; 6MWD: 6-minute walk distance; SvO2: mixed venous oxygen saturation. ERJ-00889-2017_Figure_S4
Supplementary Material
E. Bergot ERJ-00889-2017_Bergot
A. Boucly ERJ-00889-2017_Boucly
A. Bourdin ERJ-00889-2017_Bourdin
A. Chaouat ERJ-00889-2017_Chaouat
V. Cottin ERJ-00889-2017_Cottin
P. de Groote ERJ-00889-2017_De_Groote
M. Humbert ERJ-00889-2017_Humbert
X. Jaïs ERJ-00889-2017_Jais
D. Montani ERJ-00889-2017_Montani
F. Parent ERJ-00889-2017_Parent
F. Picard ERJ-00889-2017_Picard
G. Prevot ERJ-00889-2017_Prevot
L. Savale ERJ-00889-2017_Savale
G. Simonneau ERJ-00889-2017_Simonneau
O. Sitbon ERJ-00889-2017_Sitbon
J. Weatherald ERJ-00889-2017_Weatherald
