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

This study is currently recruiting participants. (see Contacts and Locations)
Verified January 2014 by Stanford University
Information provided by (Responsible Party):
Stanford University Identifier:
First received: January 18, 2012
Last updated: January 7, 2014
Last verified: January 2014

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.

Alagille Syndrome

Study Type: Observational
Study Design: Observational Model: Case-Only
Time Perspective: Retrospective

Resource links provided by NLM:

Further study details as provided by Stanford University:

Primary Outcome Measures:
  • Assessment of PA in Alagille Patient [ Time Frame: Open ] [ Designated as safety issue: No ]

Estimated Enrollment: 25
Study Start Date: April 2007
Estimated Primary Completion Date: December 2017 (Final data collection date for primary outcome measure)
Patients with alagille syndrome


Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population

Patients with Alagille Syndrome. The age range will be from birth through adulthood. There will be no gender or ethnic limitations to study.


Inclusion Criteria:

  • Diagnosis of Alagille Syndrome

Exclusion Criteria:

  • None
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its identifier: NCT01515631

Contact: Jeffrey Feinstein, MD, MPH 650 723 7913

United States, California
Lucile Packard Children's Hospital at Stanford Recruiting
Palo Alto, California, United States, 94304
Principal Investigator: Jeffrey Feinstein, MD, MPH         
Sponsors and Collaborators
Stanford University
  More Information

No publications provided

Responsible Party: Stanford University Identifier: NCT01515631     History of Changes
Other Study ID Numbers: AG9031
Study First Received: January 18, 2012
Last Updated: January 7, 2014
Health Authority: United States: Institutional Review Board

Additional relevant MeSH terms:
Alagille Syndrome
Abnormalities, Multiple
Bile Duct Diseases
Biliary Tract Diseases
Cardiovascular Abnormalities
Cardiovascular Diseases
Cholestasis, Intrahepatic
Congenital Abnormalities
Digestive System Diseases
Genetic Diseases, Inborn
Heart Defects, Congenital
Liver Diseases
Pathologic Processes processed this record on October 23, 2014