Table 1.
Clinical, functional and echocardiographic characteristics (n = 105)
| 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 |
Values are the mean ± SD, median (interquartile range) or percentage
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