Figures
- FIGURE 1
STROBE (Strengthening the Reporting of Observational Studies in Epidemiology) diagram showing eligibility for analysis. PAH: pulmonary arterial hypertension; mPAP: mean pulmonary arterial pressure; PAWP: pulmonary arterial wedge pressure. #: more than one reason for exclusion could apply.
- FIGURE 2
Kaplan–Meier survival estimates overall and by pulmonary arterial hypertension (PAH) subtype. CHD: congenital heart disease; POPH: porto-pulmonary hypertension; I/H/D: idiopathic/heritable/drug-associated; CTD: connective tissue disease.
- FIGURE 3
Temporal trends in the use of initial combination therapy and combination therapy 1 year after pulmonary arterial hypertension diagnosis in a) the entire cohort, and in the subgroups of patients aged b) <65 years and c) ≥65 years.
- FIGURE 4
Annualised survival rates (95% CI) at 1 and 3 years after pulmonary arterial hypertension (PAH) diagnosis in a) the entire cohort, and in the subgroups of patients aged b) <65 years and c) ≥65 years. The black and red numbers indicate the numbers of patients available for the annualised 1- and 3-year survival rates, respectively.
Tables
- TABLE 1
Baseline characteristics of patients newly diagnosed with pulmonary arterial hypertension (PAH) between 2010 and 2019
All PAH I/H/D-PAH CTD-PAH CHD-PAH HIV-PAH POPH Patients 2531 (100) 1698 (67.1) 536 (21.2) 128 (5.1) 24 (0.9) 145 (5.7) Age, years 64.6±15.8 65.9±15.9 66.3±13.1 50.6±18.1 45.8±12.1 57.8±11.7 Female 1609 (63.6) 1016 (59.8) 434 (81.0) 84 (65.6) 11 (45.8) 64 (44.1) BMI, kg·m−2 28.0±6.1 28.4±6.1 26.7±5.4 26.3±6.5 27.6±8.2 29.6±6.2 WHO FC I 15 (0.6) 7 (0.4) 2 (0.4) 4 (3.5) 1 (4.2) 1 (0.7) II 373 (15.7) 244 (15.3) 78 (15.4) 26 (22.8) 6 (25.0) 19 (13.8) III 1675 (70.4) 1117 (69.9) 369 (73.1) 73 (64.0) 14 (58.3) 102 (73.9) IV 315 (13.2) 229 (14.3) 56 (11.1) 11 (9.6) 3 (12.5) 16 (11.6) 6MWD, m 297.1±127.1 297.4±127.3 278.4±124.2 344.6±112.9 366.0±146.7 310.7±129.1 Pulmonary function TLC, % pred 89.6±17.4 91.7±16.4 84.0±18.8 84.0±15.2 88.7±11.5 94.7±16.9 FVC, % pred 80.1±20.3 80.4±20.0 80.0±21.5 70.2±18.5 85.5±10.6 82.5±19.1 FEV1, % pred 76.4±19.8 76.5±20.0 76.9±19.6 66.0±16.9 85.0±15.3 78.8±19.6 DLCO, % pred 50.5±21.7 52.1±22.6 42.6±17.0 65.8±18.9 47.0±17.5 57.9±17.8 PaO2, mmHg 65.0±12.3 64.5±12.6 65.2±12.0 66.0±10.1 69.8±19.3 68.9±10.8 PaCO2, mmHg 35.1±6.5 35.6±6.4 34.3±6.8 35.9±6.0 35.2±1.3 33.0±5.9 Smoking history Current 96 (6.9) 76 (6.6) 5 (3.3) 3 (10.0) 0 (0.0) 12 (25.5) Former 594 (43.0) 490 (42.6) 66 (43.7) 13 (43.3) 2 (50.0) 23 (48.9) Never 693 (50.1) 585 (50.8) 80 (53.0) 14 (46.7) 2 (50.0) 12 (25.5) Pack-years# 30.0 (15.0–40.0) 30.0 (15.0–45.0) 20.0 (10.0–30.0) 12.5 (10.0–28.8) NA (NA–NA) 20.0 (20.0–37.5) Comorbid conditions Obesity¶ 810 (32.7) 589 (35.1) 120 (23.7) 34 (26.6) 6 (28.6) 61 (42.4) Hypertension 1329 (60.7) 1007 (65.6) 225 (56.0) 36 (32.4) 7 (46.7) 54 (43.2) Coronary heart disease 554 (26.1) 435 (29.0) 89 (23.2) 10 (9.3) 1 (6.7) 19 (15.8) Diabetes mellitus 590 (27.2) 489 (32.1) 61 (15.5) 18 (16.2) 0 (0.0) 22 (17.5) Haemodynamics RAP, mmHg 8.3±4.8 8.3±4.7 7.8±4.9 8.6±5.5 8.3±4.1 9.8±5.3 mPAP, mmHg 44.2±12.9 44.3±12.7 41.3±11.5 50.8±19.1 47.0±11.4 47.4±10.8 PAWP, mmHg 9.5±3.4 9.5±3.3 9.3±3.4 9.6±3.6 7.9±3.0 9.7±3.5 CI, L·min−1·m−2 2.3±0.8 2.2±0.8 2.4±0.8 2.8±1.2 2.3±1.0 2.7±1.1 PVR, dyn·s·cm−5 742.7±402.4 764.8±412.5 675.5±353.8 789.4±511.0 871.2±348.3 669.7±321.6 PVR, WU 9.3±5.0 9.6±5.2 8.4±4.4 9.9±6.4 10.9±4.4 8.4±4.0 SvO2, % 62.8±8.6 62.3±8.5 63.5±8.7 65.4±10.1 57.2±7.7 64.5±7.5 Laboratory findings Creatinine, µmol·L−1 101.7±55.3 106.1±60.2 95.3±41.9 86.5±50.9 83.0±24.6 89.1±33.9 Uric acid, µmol·L−1 436.6±151.6 443.1±146.5 430.2±154.1 393.6±188.6 383.3±123.1 424.8±161.6 Bilirubin, µmol·L−1 15.6±14.2 14.7±11.8 12.9±12.2 16.5±21.6 32.0±34.6 29.5±20.0 NT-proBNP, pg·mL−1 1454 (480–3341) 1574 (566–3498) 1504 (432–3430) 513 (262–1163) 1212 (209–2542) 848 (233–2128) BNP, pg·mL−1 206 (94–497) 200 (102–462) 231 (78–636) 154 (62–315) 175 (150–201) 240 (143–454) Risk Low 246 (9.8) 153 (9.1) 49 (9.2) 22 (17.6) 6 (25.0) 16 (11.0) Intermediate 1698 (67.6) 1144 (67.8) 349 (65.8) 91 (72.8) 15 (62.5) 99 (68.3) High 568 (22.6) 391 (23.2) 132 (24.9) 12 (9.6) 3 (12.5) 30 (20.7) Data are presented as n (%), mean±sd or median (interquartile range). I/D/H: idiopathic/drug-associated/heritable; CTD: connective tissue disease; CHD: congenital heart disease; POPH: porto-pulmonary hypertension; BMI: body mass index; WHO FC: World Health Organization Functional Class; 6MWD: 6-min walk distance; TLC: total lung capacity; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; DLCO: diffusing capacity of the lung for carbon monoxide; PaO2: arterial oxygen tension; PaCO2: arterial carbon dioxide tension; RAP: right atrial pressure; mPAP: mean pulmonary arterial pressure; PAWP: pulmonary arterial wedge pressure; CI: cardiac index; PVR: pulmonary vascular resistance; SvO2: mixed venous oxygen saturation; NT-proBNP: N-terminal fragment of pro-brain natriuretic peptide; BNP: brain natriuretic peptide. #: if current or former smoker; ¶: BMI ≥30 kg·m−2.
- TABLE 2
Use of drugs to treat pulmonary arterial hypertension (PAH) within 3 months and 1, 2 and 3 years (±6 months) after diagnosis
3 months (n=2375) 1 year (n=2136) 2 years (n=1727) 3 years (n=1299) No therapy 8 (0.3) 73 (3.4) 61 (3.5) 48 (3.7) Monotherapy 1901 (80.0) 1146 (53.7) 841 (48.7) 604 (46.5) ERA 401 (21.1) 223 (19.5) 145 (17.2) 99 (16.4) PDE5i 1375 (72.3) 851 (74.3) 642 (76.3) 467 (77.3) sGC 63 (3.3) 40 (3.5) 31 (3.7) 23 (3.8) PCA 6 (0.3) 3 (0.3) 1 (0.1) 1 (0.2) PCA oral or inhaled# 1 (16.7) 2 (66.7) 1 (100.0) 0 (0.0) PCA i.v. or s.c.¶ 5 (83.3) 1 (33.3) 0 (0.0) 1 (100.0) CCB 51 (2.7) 29 (2.5) 22 (2.6) 14 (2.3) Other PAH treatment 5 (0.3) 0 (0.0) 0 (0.0) 0 (0.0) Combination therapy 466 (19.6) 917 (42.9) 825 (47.8) 647 (49.8) ERA+PDE5i 320 (68.7) 619 (67.5) 539 (65.3) 400 (61.8) Other than ERA+PDE5i 146 (31.3) 298 (32.5) 286 (34.7) 247 (38.2) ERA+PDE5i+PCA 33 (22.6) 106 (35.6) 110 (38.5) 104 (42.1) Triple combination therapy including i.v. or s.c. PCA+ 21 (14.4) 44 (14.8) 47 (16.4) 45 (18.2) Data are presented as n (%); percentages refer to subgroups only, if applicable. ERA: endothelin receptor antagonist; PDE5i: phosphodiesterase-5 inhibitor; sGC: soluble guanylate cyclase stimulator; PCA: prostacyclin analogue; CCB: calcium channel blocker. #: selexipag, iloprost inhaled, treprostinil inhaled, beraprost; ¶: epoprostenol, iloprost i.v., treprostinil i.v./s.c.; +: all triple combination therapies that include i.v. or s.c. PCA therapy.
- TABLE 3
Estimated survival probability at 1 and 3 years in patients diagnosed with pulmonary arterial hypertension (PAH) between 2010 and 2014 and between 2015 and 2019
2010–2014 2015–2019 1-year survival 3-year survival 1-year survival 3-year survival Entire cohort 89.0 (87.2–90.9) 67.8 (65.0–70.8) 90.8 (89.3–92.4) 70.5 (67.8–73.4) Patients aged <65 years 93.3 (91.1–95.5) 80.0 (76.4–83.8) 96.0 (94.3–97.8) 83.4 (79.7–87.2) Patients aged ≥65 years 85.5 (82.8–88.4) 58.1 (54.2–62.3) 87.7 (85.5–90.0) 63.1 (59.5–66.9) I/H/D-PAH 90.2 (88.0–92.5) 69.4 (66.0–73.0) 90.8 (89.0–92.7) 70.4 (67.1–73.8) CTD-PAH 85.3 (81.0–89.9) 57.4 (51.3–64.3) 88.2 (84.3–92.2) 67.1 (60.9–74.0) I/H/D-PAH with no comorbidities and DLCO >45% predicted 90.7 (82.4–99.8) 86.0 (76.2–97.0) 97.5 (94.2–100.0) 90.1 (82.6–98.2) Data are presented as mean % (95% CI). I/H/D: idiopathic/heritable/drug-associated; CTD: connective tissue disease; DLCO: diffusing capacity of the lung for carbon monoxide.
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