Pulmonary Hypertension in Lymphangioleiomyomatosis

Observational Study of Patients With Lymphangioleiomyomatosis and Pulmonary Hypertension

This is a descriptive study of patients with Lymphangioleiomyomatosis and precapillary pulmonary hypertension.

Descriptive study of patients with Lymphangioleiomyomatosis and precapillary pulmonary hypertension This will include modality of diagnosis of lLymphangioleiomyomatosis, results of right-sided heart catheterization, and pulmonary function tests

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