Characterization of Pulmonary Artery Stenoses in Alagille Syndrome - a Medical Record Review

Completed

• Conditions: Alagille Syndrome

• Registration Number: NCT01515631
• Lead Sponsor: Stanford University

Brief Summary

Patients who have Alagille Syndrome (AGS) also frequently have blockages (or "stenoses") of their pulmonary arteries. Little is known about the degree or variability of these stenoses, or the effect of this disease on the right ventricle (the chamber of the heart which pumps blood to the lungs). This study will first quantify and describe pulmonary artery stenosis in patients with Alagille Syndrome. The study will also assess the effect of these stenoses on the right ventricle. The investigators hope to learn the degree and characteristics of pulmonary artery stenosis in Alagille Syndrome. The investigators also hope to learn the effect of this pulmonary artery stenosis on the right ventricle in patients with Alagille Syndrome. This information is critical in the management of patients with Alagille syndrome, as there is currently no data to guide clinicians on the management of pulmonary artery stenosis. Furthermore, the information from this study may help physicians manage pulmonary artery stenosis in other patients as well.

Detailed Description

The investigators hope to learn the degree and characteristics of pulmonary artery stenosis in Alagille Syndrome. We also hope to learn the effect of this pulmonary artery stenosis on the right ventricle in patients with Alagille Syndrome. This information is critical in the management of patients with Alagille syndrome, as there is currently no data to guide clinicians on the management of pulmonary artery stenosis. Furthermore, the information from this study may help physicians manage pulmonary artery stenosis in other patients as well. For the third aim, we hope to learn about the different blood vessel abnormalities in the brain, and which abnormalities are most likely to lead to stroke.

Study Timeline

• Study Start: 2007-04
• Study First Submitted: 2012-01-18
• Study First Submitted QC: 2012-01-23
• Study First Posted: 2012-01-24
• Primary Completion: 2018-02
• Study Completion: 2018-02
• Status Verified: 2018-09
• Last Update Submitted: 2018-09-27
• Last Update Posted: 2018-09-28

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 25

Inclusion Criteria

• Diagnosis of Alagille Syndrome

Read More

Exclusion Criteria

• None

Read More

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Assessment of PA in Alagille Patient	Open

Trial Locations

Locations (1)

Lucile Packard Children's Hospital at Stanford; 🇺🇸 Palo Alto, California, United States