Figures
- Fig. 1—
Distribution of World Health Organization functional class I pulmonary arterial hypertension patients by age and sex. □: female; ▓: male.
- Fig. 2—
Kaplan–Meier survival curve for World Health Organization functional class I pulmonary arterial hypertension patients. The number of patients at risk at 0, 5, 10, 15 and 20 yrs was 568, 240, 71, 19 and 3, respectively.
Tables
- Table 1—
Clinical and haemodynamic data at the time of diagnosis of pulmonary arterial hypertension
Clinical data All cases Incident cases Prevalent cases p-value# Subjects n 578 82 496 Female 77 76 77 0.75 Age yrs 48±14 (21–83) 51±14 (21–79) 48±14 (21–83) 0.063 WHO functional class III–IV 80 83 76 0.19 Exercise capacity METs 3.5±3 3.6±1.8 3.5±2.0 0.87 Haemodynamic data mRAP mmHg 11±7 10±7 11±7 0.037 mPAP mmHg 52±14 51±12 52±14 0.30 PCWP mmHg 10±4 10±4 10±4 0.89 Cardiac index L·min−1·m−2 2.3±0.9 2.4±0.8 2.2±0.9 0.07 PA,O2 saturation 58±12 61±10 57±12 0.015 PVR Woods unit 12.5±7.3 11±5 13±7 0.027 Acute vasodilator responders 4.6 4.5 4.6 0.99 Aetiology 0.12 Idiopathic/familial 48 34 50 Connective tissue disease 30 40 28 Congenital heart disease 11 13 10 Portal hypertension 7 9 7 Anorexigens 3 2 3 HIV 1 1 1 Data are presented as %, mean±sd or mean±sd (range), unless otherwise stated. WHO: World Health Organization; METs: metabolic equivalents; mRAP: mean right atrial pressure; mPAP: mean pulmonary artery pressure; PCWP: pulmonary capillary wedge pressure; PA,O2: alveolar oxygen partial pressure; PVR: pulmonary vascular resistance. #: comparisons are for incident versus prevalent cases of pulmonary arterial hypertension.
- Table 2—
Medications at the time of referral
Medication n (%) Calcium channel blockers 182 (32) Digoxin 102 (18) Warfarin 164 (28) Prostacyclins 14 (2) Endothelin antagonists 18 (3) Phosphodiesterase inhibitors 5 (1) Aspirin 77 (13) Angiotensin-converting enzyme inhibitors 70 (12) Angiotensin receptor blockers 23 (4) β-blockers 50 (9) Diuretics 272 (47) Selective serotonin re-uptake inhibitors 16 (6) - Table 3—
Baseline clinical characteristics of patients by aetiology of pulmonary arterial hypertension(PAH)
Clinical Data IPAH/FPAH CTD Congenital heart disease Anorexigen HIV Portal hypertension p-value Age yrs 45±14 55±15 42±12 52±11 40±4 49±9 <0.0001 Female 75 87 68 100 13 70 <0.0001 WHO functional class Mean 3.0±0.9 3.2±0.7 2.8±0.9 3.1±0.7 2.7±0.8 2.9±0.9 <0.007 Class I 7 3 10 0 0 7 Class II 13 9 21 19 43 22 Class III 49 49 45 56 43 49 Class IV 31 39 24 25 14 22 Exercise capacity METs 3.7±3.1 3.1±2.7 3.9±2.9 3.6±3.2 3.3±3.7 3.5±3.0 0.199 Data are presented as mean±sd or %, unless otherwise stated. IPAH: idiopathic PAH; FPAH: familial PAH; CTD: connective tissue disease; WHO: World Health Organization; METs: metabolic equivalents.
- Table 4—
Haemodynamics at the time of diagnosis for patients in each subtype of pulmonary arterial hypertension(PAH)
IPAH/FPAH CTD Congenital heart disease Anorexigen HIV Portal hypertension p-value mRAP mmHg 11±7 11±6 10±7 13±5 14±5 12±7 0.17 mPAP mmHg 56±13 48±11 54±22 52±12 50±5 46±14 <0.0001 PCWP mmHg 10±4 10±4 13±8 9±3 10±2 12±5 <0.0001 Cardiac index L·min−1·m−2 2±0.7 2.3±0.8 2.8±0.7 1.9±0.7 2.3±0.8 3.2±1.5 <0.0001 PA,O2 saturation 57±11 57±11 66±14 54±13 54±9 62±13 <0.0001 PVR Woods unit 14±6.8 11.1±5.9 13.2±11.4 13.5±7.1 10.3±3.3 7.4±5.3 <0.0001 Acute vasodilator responders 5.4 2.3 12.5 0 0 0 0.12 Data are presented as mean±sd or %, unless otherwise stated. IPAH: idiopathic PAH; FPAH: familial PAH; CTD: connective tissue disease; mRAP: mean right atrial pressure; mPAP: mean pulmonary artery pressure; PCWP: pulmonary capillary wedge pressure; PA,O2: alveolar oxygen partial pressure; PVR: pulmonary vascular resistance.
- Table 5—
Comparison of clinical and haemodynamic data for patients diagnosed in three different treatment eras
Year p-value 1982–1996 1996–2002 2002–2006 Subjects n 103 328 147 Age yr 41±12 49±14 52±14 <0.0001 Female 77 (75) 253 (77) 115 (78) 0.81 WHO functional class 3.2±0.9 2.9±0.8 3.2±0.8 0.003 Exercise capacity METs 3.6±3.05 3.6±3.05 3.4±2.9 0.81 Prostacyclins# 3 (2.9) 11 (3.4) 0 0.44 Endothelin antagonists# 0 1 (0.3) 17 (11.6) <0.001 Phosphodiesterase inhibitors# 0 0 5 (3.4) <0.001 Idiopathic/familial PAH 77 (75) 150 (46) 49 (33) <0.0001 CTD 12 (11) 100 (30) 61 (42) <0.0001 Congenital heart disease 10 (10) 34 (10) 18 (12) 0.78 Portal hypertension 4 (4) 25 (8) 14 (10) 0.241 Anorexigens 0 13 (4) 3 (2) 0.065 HIV 0 6 (2) 2 (1) 0.164 RAP mmHg 12±7 11±6 10±6 0.10 mPAP mmHg 56±14 51±14 51±12 0.005 PCWP mmHg 10±4 10±4 10±5 0.40 Cardiac index L−1·min·m−2 2.1±0.8 2.3±0.9 2.3±0.8 0.37 PA,O2 saturation 56 58 60 0.07 PVR Wood units 13.4±6.4 12.7±7.9 11.5±6.1 0.15 Acute vasodilator responders 3.7 4.2 5.8 0.78 Data are presented as mean±sd, % or n (%), unless otherwise stated. WHO: World Health Organization; METs: metabolic equivalents; PAH: pulmonary arterial hypertension; CTD: connective tissue disease; RAP: right atrial pressure; mPAP: mean pulmonary artery pressure; PCWP: pulmonary capillary wedge pressure; PA,O2: alveolar oxygen partial pressure; PVR: pulmonary vascular resistance. #: PAH-specific medications taken by patients at the time of referral to the authors’ centre.
