An interventional study of Assessment for bleeding disorder with tailored post-operative care in Alagille Syndrome, Pulmonary Artery Stenoses and Acquired Von Willebrand Disease, sponsored by Stanford University. Enrolling by invitation at 1 site in United States. Open to participants aged Up to 17 Years. Per ClinicalTrials.gov, last updated 2023-05-06.
Sponsored by Stanford University · Not applicable, Interventional, and Prevention
The goal of this interventional study is to test a hemostasis screening protocol and cardiac peri-procedural and post-operative hemostasis pathway to improving bleeding complications and improve patient survival for children with Alagille syndrome and complex cardiac conditions. The main questions it aims to answer are:
Participants will undergo additional hematology and bleeding disorder screening prior to cardiac surgery. They will additionally undergo a detailed family screening for a history of bleeding by a genetic counselor.
Researchers will compare these findings with children who have similar complex cardiac conditions requiring surgery, but who do not have Alagille syndrome to see if bleeding conditions and complications are more or less common in children with Alagille syndrome.
This study aims to develop and implement a pre-operative screening protocol prior to major procedures or surgeries for children with Alagille syndrome, including cardiac catheterization and cardiothoracic surgery (pulmonary artery reconstruction).
Secondary Objectives
This is a single-site study with the goal to enroll 40 patients over the course of the study period. All patients that are referred for Pulmonary Artery Reconstruction will be screened for eligibility. All patients will undergo standard of care pre-operative hematologic screening to evaluate for bleeding disorders (specifically platelet aggregation disorders and acquired von Willebrand Factor deficiency).
The study includes a pre-screening period of up to 4 weeks followed by a 12-months follow-up period as part of the standard of care following pulmonary artery reconstruction surgery for children with Alagille syndrome and a research related protocol for children without Alagille syndrome.
Patients would have blood drawn one time at least 2 weeks in advance of their cardiothoracic surgery. This is the screening protocol to understand if they have a bleeding disorder that would change their management in the operating room and after their surgery. For patients that are found to have a bleeding disorder requiring treatment, they would need post-operatively a blood test daily for up to 7 days total and another blood test weekly for 2 weeks and then once prior to discharge.
29 studies on the registry are indexed under Alagille Syndrome; 10 are open to participants now.
This study's planned enrollment of 40 is above the median of 29 across 14 interventional studies indexed under Alagille Syndrome.
Browse Alagille Syndrome studies →Stanford University is the lead sponsor of 2,117 studies on the registry; 425 are open to participants now.
Of its 259 completed or terminated interventional studies of FDA-regulated products, 197 (76%) have results posted.
Counted across the registry records on this site, refreshed daily.
Exclusion Criteria:
Patients with complex cardiac conditions requiring cardiothoracic surgery who also have a history of Alagille syndrome.
Other: Assessment for bleeding disorder with tailored post-operative care
Patients with complex cardiac conditions requiring cardiothoracic surgery who do not have a diagnosis of Alagille syndrome.
Other: Assessment for bleeding disorder with tailored post-operative care
Intervention will be expanded lab work-up for hematologic conditions, consultation by Hematology and tailored intra-operative and post-operative plan with aim to mitigate bleeding and hemorrhage risk, balancing thrombotic risk with this intervention.
Blood product volume
Time frame: Intra-operatively through 24 hours post-operatively
Chest tube output blood volume
Time frame: Intra-operatively through 24 hours post-operatively
Number of participants with pulmonary hemorrhage
Time frame: Intra-operatively through 24 hours post-operatively
Incidence of thromboembolism events in patients with hematologic condition
This outcome is to evaluate thrombosis risk for patients identified to have a hematologic condition who receive medication intra-operatively and post-operatively to decrease their risk of bleeding. Occurrence of thromboembolism (including blood vessel, intracardiac, stroke, mediastinal thrombosis events) will be assessed based on imaging.
Time frame: Intra-operatively through 30 days post-operatively
Plan to share: No
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