Issue |
J Extra Corpor Technol
Volume 54, Number 4, December 2022
|
|
---|---|---|
Page(s) | 338 - 342 | |
DOI | https://doi.org/10.1051/ject/202254338 | |
Published online | 15 December 2022 |
Case Report
Alagille Syndrome and Repeat Oxygenator Failure during Cardiopulmonary Bypass: A Word of Caution
* Division of Pediatric Cardiovascular Surgery, Masonic Children’s Hospital, University of Minnesota, Minneapolis, MN 55454;
† Lecturer, Department of Cardiothoracic Surgery, Faculty of Medicine, Alexandria University, Alexandria, Egypt
Address correspondence to: Sameh M. Said, MBBCh, MD, FACC, FACS, Chief, Division of Pediatric and Adult Congenital Cardiac Surgery, Maria Fareri Children’s Hospital, Westchester Medical Center, 100 Woods Road, Valhalla, New York 10595. Lecturer, Department of Cardiothoracic Surgery, Faculty of Medicine, Alexandria University, Alexandria, Egypt. Email: sameh.said@wmchealth.org
Received:
31
August
2022
Accepted:
3
October
2022
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.
Key words: Alagille syndrome / pulmonary arterioplasty / pulmonary artery reconstruction / oxygenator failure.
© 2022 AMSECT
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