Surgical repair of peripheral pulmonary artery stenosis (PPAS) may be followed by acute lung injury, with severe cases requiring support with extracorporeal membrane oxygenation (ECMO), according to findings from a retrospective study published in JTCVS Open.

It is presumed that such acute lung injury (ie, lung reperfusion injury) is caused by an acute postsurgical increase in pressure and/or flow to some portions of the lung that previously had been protected by the stenosis.

The majority of individuals with PPAS have syndromic disorders, such as Williams syndrome, Alagille syndrome (ALGS), elastin arteriopathy, and conotruncal abnormalities. The researchers sought to examine the characteristics of patients who required ECMO after pulmonary artery reconstruction. They conducted a review of 150 consecutive patients who had undergone surgical repair of PPAS at Lucile Packard Children’s Hospital in Stanford, California, between 2002 and 2022.

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Underlying diagnoses among the participants included Williams syndrome in 44 individuals, ALGS in 43 patients, elastin arteriopathy in 21 participants, tetralogy of Fallot in 21 individuals, and other diagnoses in 21 patients. The investigators compared the characteristics of patients who required ECMO following pulmonary artery reconstruction with those of patients who did not, recognizing that most individuals who undergo surgical repair of PPAS are able to recover without the development of acute lung complications or the need for ECMO.

Results of the study revealed that 7.3% (11 of 150) of the participants who underwent pulmonary artery reconstruction required postoperative ECMO—10 for acute lung injury and 1 for cardiac insufficiency. Of the individuals who required ECMO, 4 had Williams syndrome, 4 had tetralogy of Fallot, and 3 had ALGS.

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Compared with patients who did not require ECMO, those who did exhibited higher presurgical right ventricle to aortic peak systolic ratios (mean, 1.14 vs 0.95), a greater number of pulmonary arterial ostial interventions (median, 23 vs 17), and a longer duration of cardiopulmonary bypass (median, 597 minutes vs 400 minutes). Overall, 3 in-hospital deaths were reported; 2 of these patients had required postsurgical ECMO support.

According to the authors, “The data demonstrate multiple differences between patients who did and did not require ECMO after surgical repair of PPAS.” They concluded, “These results suggest that the preoperative extent of disease may predispose to development of acute lung injury requiring ECMO support.”


Felmly LM, Mainwaring RD, Algaze C, Martin E, Ma M, Hanley FL. Analysis of risk factors associated with extracorporeal membrane oxygenation after surgical repair of peripheral pulmonary artery stenoses. JTCVS Open. Published online January 31, 2023. doi:10.1016/j.xjon.2023.01.011