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Pulmonary Hypertension in Lymphangioleiomyomatosis

Completed
Conditions
Lymphangioleiomyomatosis
Pulmonary Hypertension
Registration Number
NCT00960895
Lead Sponsor
Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires
Brief Summary

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

Detailed Description

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

Recruitment & Eligibility

Status
COMPLETED
Sex
Female
Target Recruitment
20
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

Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Secondary Outcome Measures
NameTimeMethod

Trial Locations

Locations (1)

Louis Pradel Hospital

🇫🇷

Lyon, University of Lyon, France

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