Table 2– Pulmonary functions and 6-min walking test (6MWT) in patients with lymphangioleiomyomatosis (LAM) and pulmonary hypertension (PH) compared with 72 patients with LAM without PH

Variable	Patients	LAM with PH	Control LAM without PH	p-value
FVC % pred	18	76±28 (27–121)	88±25 (30–145)	0.08
FEV₁ % pred
Prebronchodilator	19	42±24 (13–96)	63±25 (16–129)	0.002
Postbronchodilator	19	46±26 (13–96)	NA	NA
FEV_₁/FVC % pred	18	47±15 (22–75)	60±16 (24–95)	0.003
TLC % pred	20	104±16 (77–143)	109 ±23 (51–169)	0.404
RV % pred	20	162±52 (63–243)	143±58 (65–309)	0.461
D_L,CO % pred	18	29±13 (14–57)	50±25 (15–111)	0.002
K_CO % pred	13	35±14 (19–69)	57±22 (15–93)	0.002
P_a,O₂ at rest kPa	17	7.4±1.1 (5.5–9.5)	10.1±1.9 (6.7–14.5)	<0.001
P_a,CO₂ at rest kPa	18	4.8±0.5 (3.9–5.9)	4.7±0.6 (3.7–6.7)	0.188
6MWD m	18	340±84 (200–475)	474±144 (110–770)	0.001
S_p,O₂ % at end of 6MWT	18	81±9 (57–91)	88±8 (62–99)	0.009
S_p,O₂ % decrease during 6MWT	18	-10±8 (-28–0)	-8±8 (-36–2)	0.189

Data are presented as n or mean±sd (range) unless otherwise stated. FVC: forced vital capacity; % pred: % predicted; FEV₁: forced expiratory volume in 1 s; TLC: total lung capacity; RV: residual volume; D_L,CO: diffusing capacity of the lungs for carbon monoxide; K_CO: transfer coefficient for the lung for carbon monoxide; P_a,O₂: arterial oxygen tension; P_a,CO₂: arterial carbon dioxide tension; 6MWD: 6-min walking distance; S_p,O₂: arterial oxygen saturation measured by pulse oximetry; NA: not available.