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

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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.

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.

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