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Table 1 Characteristics of patients with IPAH and HPAH who underwent a genetic testing

From: Genetic analyses in a cohort of 191 pulmonary arterial hypertension patients

Characteristic IPAH (n = 179) HPAH (n = 12)
Age at diagnosis, y 30.7 ± 10.6 24.8 ± 7.2
Female sex 135 (75.4%) 7 (58.3%)
NYHA I-II 82 (45.8%) 6 (50.0%)
NYHA III 91 (50.8%) 5 (41.7%)
NYHA IV 6 (3.4%) 1 (8.3%)
Hemodynamics at diagnosis
RAP, mmHg 5.2 ± 4.2 2.8 ± 4.5
mPAP, mmHg 56.1 ± 14.7 61.6 ± 16.4
PVR, dyn*s*cm− 5 1155.6 ± 523.6 1052.8 ± 422.4
PAWP, mmHg 7.1 ± 3.5 7.1 ± 3.1
CI, L/min/m2 2.8 ± 0.9 3.1 ± 1.2
SvO2, % 67.8 ± 7.5 71.4 ± 6.3
Peak VO2, ml/min/Kg 13.1 ± 3.6 12.2 ± 2.2
6-min walk distance, m 415.6 ± 101.3 435.5 ± 100.3
NT-proBNP, pg/ml 1403.6 ± 1327.4 970.1 ± 881.8
  1. Values are mean ± SD or n (%). NYHA, New York Heart Association functional class; RAP right atrial pressure, mPAP mean pulmonary artery pressure, PVR pulmonary vascular resistance, PAWP pulmonary artery wedge pressure, CI cardiac index, SvO2 mixed venous oxygen saturation, Peak VO2 peak oxygen consumption, NT-proBNP N-terminal pro–B-type natriuretic peptide