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Table 1 Clinical, functional and echocardiographic characteristics (n = 105)

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

Clinical variables
 Age (years) 41 ± 13
 Time from LAM diagnosis (years, IQR) 5 (1 – 9)
 Smoker (former or current) (n, %) 10 (9.5%)
 BMI > 35 kg.m−2 (n, %) 2 (2%)
 Tuberous sclerosis (n, %) 18 (17%)
 Renal angiomyolipoma (n, %) 48 (45%)
 Dyspnea (n, %) 74 (70%)
 Pneumothorax (n, %) 48 (46%)
 Hemoptysis (n, %) 14 (13%)
 Use of sirolimus (n, %) 34 (32%)
 Duration of use of sirolimus (months) 27 ± 18
Pulmonary function tests
 FEV1 (L; % predicted) 2.08 ± 0.72 L; 73 ± 24%
 FVC (L; % predicted) 3.01 ± 0.73 L; 86 ± 19%
 FEV1/FVC 0.68 ± 0.17
 RV (L; % predicted) 138 ± 57%
 TLC (L; % predicted) 5.08 ± 1.02 L; 103 ± 18%
 RV/TLC 0.43 ± 0.15
 DLco (mL/min/mmHg; % predicted) 16.7 ± 7.1; 68 ± 28%
Six-minute walk test
 Distance (m; % predicted) 480 ± 114 m; 82 ± 19%
 Minimum SpO2 (%) 90 ± 8
 Change in SpO2 (%) 7 ± 5
 Peak HR, beats/min 115 ± 19
 Final Borg dyspnea score (IQR) 2 (0 – 5)
 Final Borg leg discomfort score (IQR) 1 (0 – 3)
Transthoracic echocardiography
 Estimated systolic PAP (mmHg) 27 ± 6
 Left ventricular ejection fraction (%) 67 ± 2
  1. Values are the mean ± SD, median (interquartile range) or percentage
  2. Definition of abbreviations: BMI body mass index, DL CO lung diffusing capacity for carbon monoxide, FEV 1 forced expiratory volume in the first second, FVC forced vital capacity, HR heart rate, LAM lymphangioleiomyomatosis, PAP pulmonary arterial pressure, RV residual volume, SpO 2 oxygen saturation, TLC total lung capacity