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Table 2 Clinical, functional and echocardiographic variables, and data obtained from right heart catheterisation: comparison between PH and non-PH groups

From: Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method

Clinical, functional and echocardiographic variables PH (n = 8) Non-PH (n = 97) P
Age (years) 44 ± 6 41 ± 13 0.60
Time from LAM diagnosis (years, IQR) 2 (1.5–11.5) 5 (1–9) 0.75
Use of sirolimus (n, %) 7 (87%) 27 (28%) 0.002
Duration of use of sirolimus (months) 23 ± 7 28 ± 20 0.57
FEV1 (% predicted) 33 ± 18 76 ± 21 <0.001
DLCO (% predicted) 24 ± 14 72 ± 26 < 0.001
6MWD (m) 371 ± 113 488 ± 110 0.01
Minimum SpO2 82 ± 6 91 ± 8 0.01
Final Borg dyspnea score 5 (5–7) 2 (0–5) 0.048
Estimated systolic PAP 38 ± 7 26 ± 5 < 0.001
Right heart catheterisation PH (n = 8) Non-PH (n = 8)  
mPAP (mmHg) 29 ± 5 21 ± 2 <0.001
PAOP (mmHg) 14 ± 4 10 ± 5 0.11
CO (L/min) 4.8 ± 1.2 5.0 ± 0.6 0.70
PVR (IU) 3.4 ± 1.2 2.3 ± 0.8 0.06
  1. Values are the mean ± SD or median (interquartile range)
  2. Definition of abbreviations: 6MWD six-minute walk distance, CO cardiac output, DL CO lung diffusing capacity for carbon monoxide, FEV 1 forced expiratory volume in the first second, LAM lymphangioleiomyomatosis, mPAP mean pulmonary arterial pressure, PH pulmonary hypertension, PAOP pulmonary artery occlusion pressure, PAP pulmonary arterial pressure, PVR pulmonary vascular resistance, SpO 2 oxygen saturation