Figures
- FIGURE 1
Overall survival. a) All-cause mortality and b) all-cause mortality by aetiology. PAH: pulmonary arterial hypertension; CHD: congenital heart disease; CTD: connective tissue disease; IPAH: idiopathic PAH; HPAH: heritable PAH. The percentages show the number of patients surviving.
- FIGURE 2
Non-elective hospitalisation- free survival for all-cause mortality. The percentages given are for patients alive and not requiring hospitalisation.
- FIGURE 3
All-cause mortality and triple combination therapy-free survival. Percentages shown are for patients alive and not requiring triple combination therapy.
- FIGURE 4
All-cause mortality for non-elective hospitalisation and triple combination therapy-free survival. Percentages shown are for patients alive and not requiring hospitalisation or triple combination therapy.
Tables
- TABLE 1
Baseline characteristics at initiation of combination therapy
All patients IPAH/HPAH PAH-CHD PAH-CTD Patients n 181 102 61 29 Male % 40 48 38 17 Age at initiation of combination therapy years 50 (36–62) 50 (36–62) 45 (31–54) 66 (58–72) Duration of monotherapy months 16 (5–39) 14 (4–35) 26 (8–51) 13 (6–27) New York Heart Association functional class III/IV % 52 54 39 72 6-min walking distance m 425 (307–491) 441 (271–516) 428 (383–494) 360 (219–425) Right atrial pressure mmHg 9 (7–13) 8 (7–12) 9 (7–12) 12 (7–16) Mean pulmonary arterial pressure mmHg 62 (51–75) 59 (49–69) 78 (65–90) 50 (44–56) Pulmonary wedge pressure mmHg 10 (8–12) 10 (8–12) 11 (9–12) 9 (6–12) Mean systemic arterial pressure mmHg 85 (77–93) 85 (77–91) 82 (75–94) 89 (82–97) Cardiac index L·min−1·m−2 2.2 (1.9–2.6) 2.3 (1.9–2.7) 2.1 (1.8–2.6) 2.1 (1.9–2.4) Pulmonary vascular resistance WU 13 (10–18) 12 (9–16) 17 (13–27) 13 (8–14) Systemic vascular resistance WU 20 (16–24) 18 (16–23) 21 (16–27) 20 (18–25) Arterial oxygen saturation % 93 (90–96) 95 (92–97) 90 (84–95) 94 (92–97) Pulmonary arterial oxygen saturation % 64 (57–69) 62 (56–67) 68 (61–73) 60 (51–64) Data are presented as median (interquartile range) unless otherwise stated. PAH: pulmonary arterial hypertension; IPAH: idiopathic PAH; HPAH: heritable PAH; CHD: congenital heart disease; CTD: connective tissue disease; WU: Wood units.
- TABLE 2
Symptomatic and haemodynamic parameters at baseline and after 3–4 months of combination therapy
All patients n=192 IPAH/HPAH n=93 PAH-CHD n=60 PAH-CTD n=28 Baseline 3–4 months p-value Baseline 3–4 months p-value Baseline 3–4 months p-value Baseline 3–4 months p-value NYHA FC III/IV 49 35 <0.001 51 32 <0.001 38 28 0.146 71 64 0.687 6MWD m 428 (339–496) 465 (380–545) <0.001 450 (302–531) 498 (376–570) <0.001 431 (394–498) 456 (425–546) <0.001 364 (214–426) 397 (310–434) 0.030 RAP mmHg 9 (7–12) 8 (6–11) 0.003 8 (6–11) 7 (6–10) 0.003 9 (7–12) 9 (7–11) 0.405 12 (7–16) 10 (6–15) 0.321 mPAP mmHg 62 (51–76) 57 (48–70) <0.001 59 (49–69) 54 (46–63) <0.001 78 (65–90) 73 (59–90) <0.001 50 (43–56) 49 (42–54) 0.011 CI L·min−1·m−2 2.3 (1.9–2.6) 2.5 (2.2–2.9) <0.001 2.3 (1.9–2.7) 2.7 (2.4–3.0) <0.001 2.1 (1.8–2.6) 2.4 (2.1–2.9) 0.003 2.2 (1.9–2.5) 2.5 (2.0–2.8) 0.010 PVR WU 13 (10–18) 11 (8–15) <0.001 12 (9–16) 10 (7–12) <0.001 17 (13–27) 14 (9–21) <0.001 13 (8–14) 9 (7–14) 0.003 Data are presented as % or median (interquartile range), unless otherwise stated. PAH: pulmonary arterial hypertension; IPAH: idiopathic PAH; HPAH: heritable PAH; CHD: congenital heart disease; CTD: connective tissue disease; NYHA FC: New York Heart Association functional class; 6MWD: 6-min walking distance; RAP: right atrial pressure; mPAP: mean pulmonary arterial pressure; CI: cardiac index; PVR: pulmonary vascular resistance; WU: Wood units. The p-values were determined using a non-parametric Wilcoxon–Mann–Whitney test for all comparisons, except for NYHA that was evaluated by McNemar test.
- TABLE 3
Cause of death
All patients IPAH/HPAH PAH-CHD PAH-CTD Patients 192 102 61 29 Death 66 34 17 15 Heart failure 40 17 11 12 Sudden death 9 4 4 1 Haemoptysis 4 3 1 0 Respiratory failure 3 3 0 0 Infection 3 1 1 1 Other 3 0 Neoplasia 1 Myocardial infarction 1 Renal failure 1 Unknown 4 4 0 0 Data are presented as n. PAH: pulmonary arterial hypertension; IPAH: idiopathic PAH; HPAH: heritable PAH; CHD: congenital heart disease; CTD: connective tissue disease.
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 figures and table - Figures S1-S3 and table S1
