Scientists are currently conducting a study to assess the merits of a hemostatic screening protocol and cardiac peri- and postoperative hemostatic pathway to reduce bleeding complications and improve survival in children with Alagille syndrome (ALGS) who have complex cardiac conditions. 

Children with ALGS tend to be at a higher risk of developing cardiac disease, which may contribute to significant morbidity and early mortality. Thus, researchers are at pains to improve the prognosis of patients who are suffering from cardiac complications of ALGS. 

The authors of the study hypothesized that a better screening protocol and the development of a hemostatic pathway to treat periprocedural bleeding complications may improve clinical outcomes. These procedures may include major surgeries, such as cardiothoracic surgery for pulmonary artery reconstruction. 

Continue Reading

Forty patients will be enrolled from a pool of patients with ALGS currently being assessed for pulmonary artery reconstruction suitability. Patients must be 17 years of age or younger with a complex cardiac condition necessitating this surgical procedure, such as Tetralogy of Fallot or branch pulmonary artery stenosis. 

Read more about ALGS etiology 

There will be a prescreening period up to 4 weeks, followed by a 12-month follow-up program postoperatively. Two weeks prior to surgery, patients will have blood drawn at least once to assess if they have bleeding disorders that require additional attention. If they are found to have a bleeding disorder, blood-taking will be required postoperatively as well. 

The primary outcome measures of this trial are blood product volume, chest tube output blood volume, and the number of participants with pulmonary hemorrhage. The time frame for these outcomes is intraoperatively through 24 hours postsurgery. Secondary outcomes measures include the incidence of thromboembolism events in patients with a hematological condition within the same time frame. 

The trial started on April 18, 2023, and has an estimated completion date of November 2024. 


Decreasing hemorrhage risk in children with Alagille syndrome. May 6, 2023. Accessed May 18, 2023.