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Abdominal angiography at 6\u2009months demonstrated a large extrahepatic portosystemic shunt, which was percutaneously closed with a vascular plug. Kawashima operation was performed 2\u2009weeks after portosystemic shunt closure. Although cyanosis improved temporarily, the patient suffered from deteriorating cyanosis at 9\u2009months of age and underwent Fontan completion. Thereafter, her oxygen saturation gradually improved to 95% over the course of 3\u2009weeks. Both the congenital extrahepatic portosystemic shunt and Kawashima operation contributed to the development of pulmonary arteriovenous malformations.<\/jats:p>","DOI":"10.1136\/bcr-2019-229491","type":"journal-article","created":{"date-parts":[[2019,7,3]],"date-time":"2019-07-03T07:25:17Z","timestamp":1562138717000},"page":"e229491","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Pulmonary arteriovenous malformations in a patient with single ventricle and polysplenia syndrome"],"prefix":"10.1136","volume":"12","author":[{"given":"Shinichiro","family":"Sakaki","sequence":"first","affiliation":[{"name":"Division of Pediatric Cardiology, National Center for Child Health and Development, Tokyo, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3627-2799","authenticated-orcid":false,"given":"Taiyu","family":"Hayashi","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology, National Center for Child Health and Development, Tokyo, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroshi","family":"Ono","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology, National Center for Child Health and Development, Tokyo, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2019,7,2]]},"reference":[{"key":"2025082712125969000_12.7.e229491.1","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(00)00812-3"},{"key":"2025082712125969000_12.7.e229491.2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00796995"},{"key":"2025082712125969000_12.7.e229491.3","doi-asserted-by":"publisher","DOI":"10.1007\/s00247-009-1508-y"},{"key":"2025082712125969000_12.7.e229491.4","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1111\/j.1747-0803.2010.00478.x","article-title":"Congenital portosystemic venous connections and other abdominal venous abnormalities in patients with polysplenia and functionally univentricular heart disease: a case series and literature review","volume":"6","author":"McElhinney","year":"2011","journal-title":"Congenit Heart Dis"},{"key":"2025082712125969000_12.7.e229491.5","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.92.5.1217"},{"key":"2025082712125969000_12.7.e229491.6","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1007\/s00431-015-2623-4","article-title":"Congenital extrahepatic portosystemic shunt: an underdiagnosed but treatable cause of hepatopulmonary syndrome","volume":"175","author":"Fu","year":"2016","journal-title":"Eur J Pediatr"},{"key":"2025082712125969000_12.7.e229491.7","unstructured":"Kliegman R , St Geme J . 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