Inclusion Criteria:

- Lymphangioleiomyomatosis (definite or probable), either sporadic or associated with
tuberous sclerosis

- Precapillary pulmonary hypertension, as defined by right-sided heart catheterization
(mean pulmonary artery pressure higher than 25 mmHg, wedge capillary pulmonary
pressure less than 15 mmHg, pulmonary vascular resistance higher than 3 Wood units)

Exclusion Criteria:

- Post capillary pulmonary hypertension

- Other identifiable causes of pulmonary hypertension (ex: thromboembolic disease)

- Not willing to participate