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

Ashley C. Moore, Kyle N. Sieck, Sarah J. Lojovich, Roger P. Mueller, Jason E. Windle, Sameh M. Said

Research output: Contribution to journalArticlepeer-review

Abstract

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

Original languageEnglish (US)
Pages (from-to)338-342
Number of pages5
JournalJournal of Extra-Corporeal Technology
Volume54
Issue number4
DOIs
StatePublished - Dec 1 2022

Bibliographical note

Publisher Copyright:
© Copyright 2022 AMSECT.

Keywords

  • Alagille syndrome
  • Oxygenator failure.
  • Pulmonary arterioplasty
  • Pulmonary artery reconstruction

PubMed: MeSH publication types

  • Case Reports

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