Table 1 Characteristics of PAH patients (n = 131)
From: Monotherapy in patients with pulmonary arterial hypertension at four German PH centres
Total n = 131 | Typical patients N = 86 | Atypical patients N = 45 | p value | |
|---|---|---|---|---|
Age (years) | 64 (± 13.8) | 59 (± 14.1) | 74 (± 5.3) | < 0.001 |
Male | 65 (49.6%) | 48 (55.8%) | 17 (37.8%) | < 0.050 |
BMI (kg/m2) | 28.1 (± 6.4) | 27.8 (± 7.0) | 28.9 (± 5.0) | 0.296 |
Diagnoses | ||||
IPAH | 48 (36.6%) | 28 (32.6%) | 20 (44.4%) | 0.180 |
PAH | 83 (63.4%) | 58 (67.4%) | 25 (55.6%) | |
Portopulmonary PAH | 11 (8.4%) | 10 (17.2%) | 1 (4.0%) | |
PAH due to connective tissue disease | 40 (30.5%) | 23 (39.7%) | 17 (68.0%) | |
PAH due to congenital heart disease | 3 (2.3%) | 3 (5.2%) | - | |
Further diagnoses | ||||
Cardiovascular disease | ||||
Diastolic dysfunction (by echocardiography) | 62 (47.3%) | 30 (34.9%) | 32 (71.1%) | < 0.001 |
Arterial hypertension | 95 (72.5%) | 54 (62.8%) | 41 (91.1%) | < 0.001 |
Coronary heart disease | 32 (24.4%) | 14 (16.3%) | 18 (40.0%) | 0.003 |
Atrial fibrillation | 37 (28.4%) | 12 (14.0%) | 25 (55.6%) | < 0.001 |
Peripheral arterial occlusive disease | 6 (4.6%) | 3 (3.5%) | 3 (6.7%) | 0.409 |
Thromboembolic disease | 19 (14.5%) | 9 (10.5%) | 10 (22.2%) | 0.070 |
Pulmonary embolism | 12 (63.2%) | 6 (66.6%) | 6 (60.0%) | |
Diabetes mellitus | 38 (29.0%) | 15 (17.4%) | 23 (51.1%) | < 0.001 |
Insulin dependence | 17 (44.7%) | 6 (40.0%) | 11 (47.8%) | |
Chronic kidney disease | 64 (48.9%) | 32 (37.2%) | 32 (71.1%) | < 0.001 |
Thyroid disease | 30 (22.9%) | 17 (19.8%) | 13 (28.9%) | 0.238 |
Pulmonary disease | ||||
COPD | 30 (22.9%) | 21 (24.4%) | 9 (20.0%) | 0.568 |
ILD | 21 (16.0%) | 13 (15.1%) | 8 (17.8%) | 0.693 |
Cancer | 11 (8.4%) | 5 (5.8%) | 6 (13.3%) | 0.186 |
Obstructive sleep apnoea | 12 (9.2%) | 7 (8.1%) | 5 (3.8%) | 0.751 |
Functional class | ||||
II | 18 (13.7%) | 15 (17.4%) | 3 (6.7%) | 0.107 |
III | 90 (68.7%) | 54 (62.8%) | 36 (80.0%) | |
IV | 23 (17.6%) | 17 (19.8%) | 6 (13.3%) | |
6-MWD (m) (n = 57) | 266 (± 129) | 266 (± 139) n = 38 | 267 (± 107) n = 19 | 0.959 |
Pulmonary function | ||||
FVC %pred | 83.4 (± 20.9) (n = 93) | 82.8 (± 20.4) (n = 65) | 84.7 (± 22.2) (n = 28) | 0.696 |
FVC %pred < 70% | 21 (22.6%) | 14 (21.5%) | 7 (25.0%) | |
FEV1%pred | 76.7 (± 19.9) (n = 94) | 75.3 (± 19.1) (n = 66) | 80.1 (± 21.6) (n = 28) | 0.284 |
FEV1%pred < 60% | 21 (22.3%) | 16 (24.2%) | 5 (17.9%) | |
FEV1/FVC | 74.4 (± 12.4) (n = 94) | 74.2 (± 13.4) (n = 66) | 75.0 (± 9.9) (n = 28) | 0.772 |
FEV1/FVC < 70% | 31 (33.0%) | 24 (36.4%) | 7 (25.0%) | |
DLCO %pred | 41.5 (± 17.2) (n = 82) | 42.1 (± 19.0) (n = 59) | 39.9 (± 11.9) (n = 23) | 0.534 |
DLCO %pred < 45% | 49 (59.8%) | 34 (57.6%) | 15 (65.2%) | |
paO2 (mmHg) | 62.0 (± 17.8) (n = 93) | 63.8 (± 17.1) (n = 65) | 57.8 (± 18.9) (n = 28) | 0.138 |
paCO2 (mmHg) | 33.5 (± 6.8) (n = 93) | 33.2 (± 6.0) (n = 65) | 34.2 (± 8.6) (n = 28) | 0.571 |
CPET | ||||
VO2 peak %pred | 49.9 (± 16.1) (n = 73) | 50.0 (± 16.7) (n = 48) | 49.6 (± 15.2) (n = 25) | 0.917 |
VO2 peak (ml/kg/min) | 11.8 (± 4.2) (n = 73) | 12.6 (± 4.6) (n = 48) | 10.1 (± 2.7) (n = 25) | 0.004 |
VE/VCO2 slope | 52.7 (± 17.8) (n = 71) | 51.4 (± 17.6) (n = 48) | 55.3 (± 18.3) (n = 23) | 0.396 |
Haemodynamics | ||||
PAPm (mmHg) | 48 (± 12.8) | 48.9 (± 13.7) (n = 86) | 45.9 (± 10.5) (n = 45) | 0.197 |
RAPm (mmHg) | 10 (± 5.1) (n = 126) | 9.5 (± 5.4) (n = 82) | 10.3 (± 4.5) (n = 44) | 0.416 |
PAOPm (mmHg) | 12 (± 4.9) (n = 126) | 11.5 (± 4.6) (n = 84) | 12.7 (± 4.3) (n = 42) | 0.162 |
CI (l/min/m2) | 2.4 (± 0.8) (n = 115) | 2.4 (± 0.9) (n = 76) | 2.4 (± 0.7) (n = 39) | 0.701 |
PVR (Wood Unit) | 9.2 (± 4.9) (n = 119) | 9.7 (± 5.4) (n = 78) | 8.4 (± 3.7) (n = 41) | 0.156 |
Echocardiography | ||||
TAPSE (mm) | 18 (± 5.3) (n = 100) | 17.4 (± 4.8) (n = 63) | 17.6 (± 6.2) (n = 37) | 0.893 |
PAP syst. (mmHg) | 72 (± 21.9) (n = 104) | 77.8 (± 23.8) (n = 62) | 74.9 (± 18.9) (n = 42) | 0.502 |
RA surface (cm2) | 27 (± 6.8) (n = 82) | 26.2 (± 6.2) (n = 50) | 27.9 (± 7.7) (n = 32) | 0.256 |
Pericardial effusion | 89 | 60 | 29 | |
Yes | 12 (13.5%) | 9 (15.0%) | 3 (10.3%) |