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Allergy/Immunology

Study highlights effective management of pulmonary and liver complications in Alagille syndrome

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A multidisciplinary approach enables successful management of complex cases in Alagille syndrome, including pulmonary artery reconstruction in patients with advanced liver disease, improving their outcomes and facilitating liver transplantation, according to a study.

The retrospective review analyzed 6 children who underwent pulmonary artery reconstruction between 2021 and 2022.

All patients underwent one-stage extensive bilateral branch pulmonary artery rehabilitation. Additional procedures included tetralogy of Fallot repair in 1 case and supravalvar pulmonary artery stenosis repair in 2 others. Postoperative outcomes showed a significant reduction in right ventricular systolic pressure across all cases.

Three patients required liver transplantation due to pre-existing liver dysfunction. At a median follow-up of 3 years, all 6 patients were alive, with their right ventricular systolic pressure remaining below half of their systemic systolic pressure. One patient required balloon angioplasty for recurrent stenosis 13 months post-surgery.

The study concludes that complex pulmonary artery reconstruction is feasible and can improve outcomes for patients with Alagille syndrome, even in the presence of severe liver disease. The findings underscore the importance of a multidisciplinary team approach in managing this rare condition and advocate for specialized Alagille programs to optimize patient care.

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; PMCID: PMC11354506.

 

 

 

 

 

 

 

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

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