Figures
- Figure 1–
Kaplan–Meier estimates of 5-yr survival from time of diagnosis in different pulmonary hypertension subtypes. IPAH: idiopathic pulmonary arterial hypertension; CTD: connective tissue disease; CTEPH: chronic thromboembolic pulmonary hypertension; PoPH: portopulmonary hypertension; PVOD: pulmonary veno-occlusive disease. The p-value for the overall comparison is <0.001. #: compared with IPAH.
- Figure 2–
a) Observed and estimated survival (National Institutes of Health (NIH) and Pulmonary Hypertension Connection (PHC) equations) of idiopathic pulmonary arterial hypertension (IPAH) nonresponders in the Spanish Registry of Pulmonary Arterial Hypertension (REHAP) registry. b) Observed and estimated survival (French equations) of IPAH cases diagnosed <48 months before June 30, 2008 in the REHAP registry.
Tables
- Table 1– Clinical and haemodynamic data at the time of diagnosis of pulmonary hypertension in pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH)
PAH CTEPH Subjects n 866 162 Age yrs 45±17 61±15* Males % 29 40* Time from symptoms to diagnosis yrs 3.5±6.1 2.7±4.3* WHO FC % I–II 31 23 III 58 68 IV 11 9 MeanPpa mmHg 54±16 47±13* Cardiac index L·min−1·m−2 2.6±0.9 2.3±0.5* PVR Wood units 12±6 10±5* Pra mmHg 9±5 8±6 6MWD m 363±120 317±112* Data are presented as mean±sd, unless otherwise stated. WHO: World Health Organization; FC: functional class; Ppa: pulmonary artery pressure; PVR: pulmonary vascular resistance; Pra: right atrial pressure; 6MWD: 6-min walk distance. *: p<0.05 compared with PAH.
- Table 2– 6-min walk distance (6MWD) and pulmonary haemodynamics in pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) according to functional class (FC)
PAH# ANOVA p-value CTEPH¶ ANOVA p-value WHO FC I–II III IV I–II III IV 6MWD m 442±97 343±111* 255±98* <0.001 379±111 309±10* 210±82* <0.001 PVR Wood units 9±5 13±6* 13±7* <0.001 9±5 10±5 12±6 0.083 Pra mmHg 8±5 9±5 11±6* 0.004 7±5 8±5 13±7* 0.001 Data are presented as mean±sd, unless otherwise stated. WHO: World Health Organization; PVR: pulmonary vascular resistance; Pra: right atrial pressure. #: n=866; ¶: n=162. *: p<0.05 for differences between FC I–II versus FC III and FC IV groups for both aetiologies.
- Table 3– Clinical and haemodynamic data at the time of diagnosis in pulmonary arterial hypertension (PAH) subtypes and chronic thromboembolic pulmonary hypertension (CTEPH)
PAH CTEPH ANOVA p-value IPAH CTD CHD HIV PoPH TOS PVOD Subjects n (%) 314 (30) 157 (15) 167 (16) 54 (5) 61 (6) 33 (3.2) 15 (1.5) 162 (16) Age yrs 46±18 54±15 * 36±17* 39±5* 52±10* 39±13* 51±15 61±15* <0.001 Males % 27 10* 27 42* 49 31 22 40* <0.001 6MWD m 382±117 309±115* 365±101 416±126 396±109 372±139 314±127 317±112* <0.001 WHO FC % I–II 29 21 38 40 53 13 22 23 <0.001 III 59 64 54 40 46 72 44 68 IV 11 15 8 19 2 16 33 9 Haemodynamics Mean Ppa mmHg 55±15 47±13* 68±19* 52±11 49±13* 67±17* 55±11 47±13* <0.001 Pra mmHg 8±5 9±6* 9±6 8±6 8±5 8±5 9±4 8±5 0.577 Cardiac index L·min−1·m−2 2.4±0.7 2.6±0.9 3.0±0.9* 2.4±0.6 3.2±0.9* 2.2±0.5 2.2±0.5 2.3±0.5 <0.001 PVR Wood units 12±6 11±6* 13±7 12±6 7±3* 17±8* 13±6 10±5* <0.001 Sv,O2 % 62±9 59±11 66±10* 60±10 69±7* 59±8 57±17 61±10 <0.001 Data are presented as mean±sd, unless otherwise stated. IPAH: idiopathic PAH; CTD: connective tissue disease; CHD: congenital heart disease; PoPH: portopulmonary hypertension; TOS: toxic oil syndrome; PVOD: veno-occlusive disease; 6MWD: 6-min walk distance; WHO: World Health Organization; FC: functional class; Ppa: pulmonary artery pressure; Pra: right atrial pressure; PVR: pulmonary vascular resistance; Sv,O2: mixed-venous oxygen saturation. *: p<0.05 compared with IPAH.
- Table 4– Survival rates in the Spanish Registry of Pulmonary Arterial Hypertension (REHAP) registry
Subjects n 1 yr 3 yrs 5 yrs All patients 1028 87 75 65 Diagnosed before July 2007 861 87 75 65 Diagnosed after July 2007 167 88 74 CTEPH 162 93 75 65 IPAH 314 89 77 68 Data are presented as %, unless otherwise stated. CTEPH: chronic thromboembolic pulmonary hypertension; IPAH: idiopathic pulmonary arterial hypertension.
- Table 5– Multivariate predictors of death in Cox's proportional hazards analysis
All patients Patients with IPAH HR (95% CI) p-value HR (95% CI) p-value Male sex 1.38 (1.03–1.83) 0.03 2.25 ( 1.31–3.72) 0.002 WHO FC III–IV 1.32 (0.95–1.84) 0.134 2.39 (1.64–4.92) 0.018 Pra 5 mmHg 1.30 (1.15–1.47) <0.001 1.46 (1.11–1.91) 0.007 Cardiac index 1 L·min−1·m−2 0.61 (0.51–0.75) <0.001 0.68 (0.44–1.05) 0.079 Subtype of PH 0.010 IPAH Reference CTD 1.78 (0.19–2.64) 0.005 CHD 0.86 (0.51–1.46) 0.583 CTEPH 1.08 (0.71–1.66) 0.720 HIV 1.05 (0.57–1.94) 0.883 PoPH 3.70 (2.28–6.03) <0.001 TOS 0.69 (0.37–1.28) 0.238 PVOD 3.11 (1.25–7.74) 0.015 ≥2 aetiologies 1.21 (0.44–2.80) 0.710 Others 1.49 (0.79–2.81) 0.213 IPAH: idiopathic pulmonary arterial hypertension; HR: hazard ratio; WHO: World Health Organization; FC: functional class; Pra: right atrial pressure; PH: pulmonary hypertension; CTD: connective tissue disease; CHD: congenital heart disease; CTEPH: chronic thromboembolic pulmonary hypertension; PoPH: portopulmonary hypertension; TOS: toxic oil syndrome; PVOD: pulmonary veno-occlusive disease.
Supplementary material
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Supplementary material
Please note: supplementary material is not edited by the Editorial Office, and is uploaded as it has been supplied by the author.
Files in this Data Supplement:
- Supplementary tables - S1-S3
