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 |