Alagille Syndrome and Repeat Oxygenator Failure during Cardiopulmonary Bypass: A Word of Caution

Alagille syndrome is an autosomal dominant disorder that caused by heterozygous mutation of JAG1 or NOTCH2 gene impacts several multisystem organs including but may not be limited to the liver, heart, musculoskeletal, skin, and eyes. The most common congenital heart defect associated with multilevel right ventricular outflow tract obstruction multiple central peripheral branch pulmonary arterial stenoses occurring in up two-thirds these patients. We report two cases who underwent extensive rehabilitation experienced unusual oxygenator failure during cardiopulmonary bypass (CPB). present lessons learned from changes we implemented our practice facilitated smooth conduct CPB other performed subsequently.