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Alagille Syndrome

Study highlights risk factors for ECMO after surgical repair of peripheral pulmonary artery stenoses

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Acute lung injury is a known complication that can arise after pulmonary artery reconstruction for peripheral pulmonary artery stenosis. In severe cases, patients may require support with extracorporeal membrane oxygenation (ECMO). A new study published in the Journal of Thoracic and Cardiovascular Surgery evaluated the characteristics of patients who needed ECMO support after pulmonary artery reconstruction.

Of the 150 patients undergoing pulmonary artery reconstruction, 11 (7.3%) needed postoperative extracorporeal membrane oxygenation support, including 10 for acute lung injury and 1 for cardiac insufficiency. These patients had Williams syndrome (n = 4), Alagille syndrome (n = 3), and tetralogy of Fallot (n = 4).

Characteristics of patients who required extracorporeal membrane oxygenation included higher preoperative right ventricle to aortic peak systolic pressure ratios, greater number of pulmonary artery ostial interventions, and longer duration of cardiopulmonary bypass.

Overall, there were 3 in-hospital deaths, of which 2 patients had undergone postoperative extracorporeal membrane oxygenation support.

Reference
Felmly LM, Mainwaring RD, Algaze C, et al. Analysis of risk factors associated with extracorporeal membrane oxygenation after surgical repair of peripheral pulmonary artery stenoses. JTCVS Open. 2023;13:344-356. doi: 10.1016/j.xjon.2023.01.011. PMID: 37063146; PMCID: PMC10091391.

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