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

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

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
DL_CO (% 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

Values are the mean ± SD or median (interquartile range)
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

ISSN: 1750-1172