{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,30]],"date-time":"2025-12-30T08:55:05Z","timestamp":1767084905512,"version":"build-2065373602"},"reference-count":23,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2022,5,5]],"date-time":"2022-05-05T00:00:00Z","timestamp":1651708800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Jordan\u2019s Guardian Angels"},{"name":"Sunderland Foundation"},{"name":"Brotman Baty Institute"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Pulmonary vein stenosis is a rare and frequently lethal childhood disease. There are few known genetic associations, and the pathophysiology is not well known. Current treatments include surgery, interventional cardiac catheterization, and more recently, medications targeting cell proliferation, which are not uniformly effective. We present a patient with PVS and a PIK3CA mutation, who demonstrated a good response to the targeted inhibitor, alpelisib.<\/jats:p>","DOI":"10.3390\/children9050671","type":"journal-article","created":{"date-parts":[[2022,5,5]],"date-time":"2022-05-05T13:10:26Z","timestamp":1651756226000},"page":"671","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Pulmonary Vein Stenosis Associated with Germline PIK3CA Mutation"],"prefix":"10.3390","volume":"9","author":[{"given":"Delphine","family":"Yung","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Division of Cardiology, University of Washington School of Medicine, Seattle, WA 98195, USA"},{"name":"Center for Integrative Brain Research, Seattle Children\u2019s Research Institute, Seattle, WA 98105, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5478-5016","authenticated-orcid":false,"given":"Kaitlyn","family":"Freeman","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Cardiology, University of Washington School of Medicine, Seattle, WA 98195, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2648-7657","authenticated-orcid":false,"given":"Ghayda","family":"Mirzaa","sequence":"additional","affiliation":[{"name":"Center for Integrative Brain Research, Seattle Children\u2019s Research Institute, Seattle, WA 98105, USA"},{"name":"Department of Pediatrics, University of Washington School of Medicine, Seattle, WA 98195, USA"},{"name":"Brotman Baty Institute for Precision Medicine, Seattle, WA 98195, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,5,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"e656","DOI":"10.1542\/peds.2008-0075","article-title":"Pulmonary vein stenosis: Prematurity and associated conditions","volume":"122","author":"Drossner","year":"2008","journal-title":"Pediatrics"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1016\/j.jpeds.2018.02.030","article-title":"Pulmonary Vein Stenosis in Infants: A Systematic Review, Meta-Analysis, and Meta-Regression","volume":"198","author":"Backes","year":"2018","journal-title":"J. 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