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Affected neonates are dependent on prostaglandin for adequate pulmonary blood flow. Depending on the severity of disease, treatment options range from transcatheter pulmonary valve perforation and ultimate biventricular repair to staged single-ventricle palliation. Cardiac transplantation is recommended in the most severe cases. This review will discuss the perioperative and anesthetic management of patients with PA\/IVS and highlight the challenges in management.<\/jats:p>","DOI":"10.1177\/1089253217737180","type":"journal-article","created":{"date-parts":[[2017,10,19]],"date-time":"2017-10-19T08:13:36Z","timestamp":1508400816000},"page":"256-264","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":15,"title":["Perioperative and Anesthetic Considerations in Pulmonary Atresia With Intact Ventricular Septum"],"prefix":"10.1177","volume":"22","author":[{"given":"Stephen","family":"Gleich","sequence":"first","affiliation":[{"name":"Seattle Children\u2019s Hospital, Seattle, WA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gregory J.","family":"Latham","sequence":"additional","affiliation":[{"name":"Seattle Children\u2019s Hospital, Seattle, WA, 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