Combined cardiac and liver interventions enhance recovery in patients with Alagille syndrome

A multidisciplinary approach to managing Alagille syndrome, particularly in patients with advanced liver disease and peripheral pulmonary artery stenosis, can lead to successful surgical outcomes, according to a study that demonstrated that extensive pulmonary artery reconstruction and associated procedures can significantly improve cardiovascular health and facilitate future liver transplantation.

The retrospective review included 6 children who underwent extensive bilateral branch pulmonary artery reconstruction.

The study found that all patients received one-stage pulmonary artery rehabilitation, with additional procedures such as tetralogy of Fallot repair and supravalvar pulmonary artery stenosis correction. Of note, 1 patient had balloon angioplasty before the surgery. Post-operatively, there was a significant decrease in right ventricular systolic pressure for all patients, and 3 underwent liver transplantation due to pre-existing liver dysfunction.

At a median follow-up of 3 years, all patients remained alive with improved cardiovascular outcomes. However, 1 patient experienced recurrent left pulmonary artery stenosis 13 months after surgery and required balloon angioplasty.

Reference
Karim F, Hiremath G, Samayoa JC, et al. Complex Pulmonary Artery Rehabilitation in Children with Alagille Syndrome: An Early Single-Center Experience of a Successful Collaborative Work. J Cardiovasc Dev Dis. 2024;11(8):232. doi: 10.3390/jcdd11080232. PMID: 39195140.

Rare Disease 360® is the Official Media Partner and Official Publication of The Alagille Syndrome Alliance (Alagille.org).