Tailored Hemostasis Management for Alagille Syndrome

Recruiting in Palo Alto (17 mi)

Overseen byNoelle Ebel, MD

Age: < 18

Sex: Any

Travel: May Be Covered

Time Reimbursement: Varies

Trial Phase: Academic

Waitlist Available

Sponsor: Stanford University

No Placebo Group

Trial Summary

What is the purpose of this trial?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: * Are children with Alagille syndrome with cardiac anomalies more likely to have acquired von Willebrand syndrome (a condition that causes increased bleeding) * Does implementation of a novel screening protocol to detect pre-operative bleeding conditions decrease intra-operative and/or post-operative bleeding complications and mortality risk? * Does implementation of a novel screening protocol to detect and treat bleeding conditions cause thrombotic complications? 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.

Eligibility Criteria

This trial is for children aged 0-17 with Alagille syndrome and complex heart conditions needing pulmonary artery reconstruction. It's not open to those who already have a known bleeding disorder or are 18 years or older.

Inclusion Criteria

I am 17 or younger with a heart condition needing surgery on my lung arteries.

Exclusion Criteria

I have a known bleeding disorder.

I am 18 years old or older.

Participant Groups

The study tests if a new screening protocol can spot bleeding disorders like von Willebrand syndrome before heart surgery, reducing bleeding risks and death in kids with Alagille syndrome compared to those without it.

2Treatment groups

Experimental Treatment

Group I: No history of Alagille syndromeExperimental Treatment1 Intervention

Patients with complex cardiac conditions requiring cardiothoracic surgery who do not have a diagnosis of Alagille syndrome.

Group II: Alagille syndromeExperimental Treatment1 Intervention

Patients with complex cardiac conditions requiring cardiothoracic surgery who also have a history of Alagille syndrome.