Liver dysfunction has ‘profound’ impact on outcomes in ALGS after heart surgery

The impact liver dysfunction has on the survival of children with Alagille syndrome (ALGS) undergoing congenital heart surgery is great, according to a study.

In this retrospective review, 69 patients with ALGS who underwent congenital heart surgery for peripheral pulmonary artery stenosis (PPAS; n = 29), tetralogy of Fallot with PPAS (n = 14), tetralogy with pulmonary valve atresia (PA; n = 3), PA with ventricular septal defect and major aortopulmonary collateral arteries (n = 21), D-transposition (n = 1) and supravalvar aortic stenosis (n =1), were included. Procedures performed included PPAS repair (n = 43), tetralogy with PA repair (n = 3), unifocalization procedures (n = 21), and other (n = 2). At the time of surgery, the median age was 16 months.

Mild or no liver dysfunction was noted in 42 (61%) patients and moderate or severe liver dysfunction in 26 (38%) of patients. The median cardiopulmonary bypass time was 345 minutes.

Overall, there were 8 operative (12%) deaths, with 6 occurring in patients with liver dysfunction and 2 in patients without liver dysfunction; there were 3 late (4%) deaths, 2 of which occurred in patients with liver dysfunction and 1 without liver dysfunction.

Reference
Mainwaring RD, Felmly ML, Collins RT, et al. Impact of liver dysfunction on outcomes in children with Alagille syndrome undergoing congenital heart surgery. Eur J Cardiothorac Surg. 2022;ezac553. doi: 10.1093/ejcts/ezac553. Epub ahead of print. PMID: 36458925.

Official Media Partner & Publisher of ALGSA