Table 5—

Comparison of clinical and haemodynamic data for patients diagnosed in three different treatment eras

Yearp-value
1982–19961996–20022002–2006
Subjects n103328147
Age yr41±1249±1452±14<0.0001
Female77 (75)253 (77)115 (78)0.81
WHO functional class3.2±0.92.9±0.83.2±0.80.003
Exercise capacity METs3.6±3.053.6±3.053.4±2.90.81
Prostacyclins#3 (2.9)11 (3.4)00.44
Endothelin antagonists#01 (0.3)17 (11.6)<0.001
Phosphodiesterase inhibitors#005 (3.4)<0.001
Idiopathic/familial PAH77 (75)150 (46)49 (33)<0.0001
CTD12 (11)100 (30)61 (42)<0.0001
Congenital heart disease10 (10)34 (10)18 (12)0.78
Portal hypertension4 (4)25 (8)14 (10)0.241
Anorexigens013 (4)3 (2)0.065
HIV06 (2)2 (1)0.164
RAP mmHg12±711±610±60.10
mPAP mmHg56±1451±1451±120.005
PCWP mmHg10±410±410±50.40
Cardiac index L−1·min·m−22.1±0.82.3±0.92.3±0.80.37
PA,O2 saturation5658600.07
PVR Wood units13.4±6.412.7±7.911.5±6.10.15
Acute vasodilator responders3.74.25.80.78
  • 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.