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A bubble echocardiogram showed a possible right-to-left shunt, CT aortogram displayed evidence of portal hypertension and cirrhosis, and liver biopsy findings were consistent with alpha-1 antitrypsin deficiency. The patient\u2019s increased oxygen demand was subsequently treated with continuous positive airway pressure before he was discharged with 8\u2009L home oxygen. With no current established medical therapy for HPS, the patient was assessed for liver transplantation and a decision was made in favour of this.<\/jats:p>","DOI":"10.1136\/bcr-2021-244712","type":"journal-article","created":{"date-parts":[[2021,9,20]],"date-time":"2021-09-20T12:08:20Z","timestamp":1632139700000},"page":"e244712","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["First presentation of portal hypertension complicated by hepatopulmonary syndrome"],"prefix":"10.1136","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0470-8845","authenticated-orcid":false,"given":"Nahima","family":"Miah","sequence":"first","affiliation":[{"name":"General Medicine, Medway Maritime Hospital, Gillingham, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0977-0688","authenticated-orcid":false,"given":"Aidan","family":"Ryan","sequence":"additional","affiliation":[{"name":"General Medicine, Medway Maritime Hospital, Gillingham, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5353-3375","authenticated-orcid":false,"given":"Ceyhun Aksel","family":"Oztumer","sequence":"additional","affiliation":[{"name":"Medicine, Brighton and Sussex Medical School, Brighton, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohamed","family":"Saleh","sequence":"additional","affiliation":[{"name":"Gastroenterology and Hepatology, Medway Maritime Hospital, Gillingham, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2021,9,20]]},"reference":[{"key":"2025082803151621000_14.9.e244712.1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.04.00010904"},{"key":"2025082803151621000_14.9.e244712.2","doi-asserted-by":"publisher","DOI":"10.1136\/gut.51.6.853"},{"key":"2025082803151621000_14.9.e244712.3","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1016\/j.rmed.2017.05.016","article-title":"The multiple dimensions of platypnea-orthodeoxia syndrome: a review","volume":"129","author":"Agrawal","year":"2017","journal-title":"Respir Med"},{"key":"2025082803151621000_14.9.e244712.4","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2018.03.024"},{"key":"2025082803151621000_14.9.e244712.5","doi-asserted-by":"publisher","DOI":"10.1016\/S0272-5231(05)70302-6"},{"key":"2025082803151621000_14.9.e244712.6","doi-asserted-by":"crossref","unstructured":"Than NN . 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