{"title":"通过吸入曲普瑞替尼和肝移植缓解严重的肺门高压症","authors":"Alex J Chang, Choon Hwa Goh","doi":"10.14503/THIJ-23-8209","DOIUrl":null,"url":null,"abstract":"<p><p>Portopulmonary hypertension is a rare condition with a poor prognosis. Prompt management is essential for liver transplantation eligibility, a potentially curative option. This report presents a case of severe portopulmonary hypertension that resolved with a conservative therapeutic regimen of tadalafil, macitentan, and inhaled treprostinil, which ultimately enabled successful liver transplantation. There was no recurrence of pulmonary hypertension after transplantation, and the patient was weaned off most pulmonary arterial hypertension therapies. This case report is the first to provide evidence that inhaled treprostinil is a safe and effective alternative to continuous intravenous prostacyclins in portopulmonary hypertension.</p>","PeriodicalId":48680,"journal":{"name":"Texas Heart Institute Journal","volume":"51 1","pages":""},"PeriodicalIF":0.9000,"publicationDate":"2024-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11075503/pdf/","citationCount":"0","resultStr":"{\"title\":\"Resolution of Severe Portopulmonary Hypertension With Inhaled Treprostinil and Liver Transplantation.\",\"authors\":\"Alex J Chang, Choon Hwa Goh\",\"doi\":\"10.14503/THIJ-23-8209\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Portopulmonary hypertension is a rare condition with a poor prognosis. Prompt management is essential for liver transplantation eligibility, a potentially curative option. This report presents a case of severe portopulmonary hypertension that resolved with a conservative therapeutic regimen of tadalafil, macitentan, and inhaled treprostinil, which ultimately enabled successful liver transplantation. There was no recurrence of pulmonary hypertension after transplantation, and the patient was weaned off most pulmonary arterial hypertension therapies. This case report is the first to provide evidence that inhaled treprostinil is a safe and effective alternative to continuous intravenous prostacyclins in portopulmonary hypertension.</p>\",\"PeriodicalId\":48680,\"journal\":{\"name\":\"Texas Heart Institute Journal\",\"volume\":\"51 1\",\"pages\":\"\"},\"PeriodicalIF\":0.9000,\"publicationDate\":\"2024-03-14\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11075503/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Texas Heart Institute Journal\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.14503/THIJ-23-8209\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Texas Heart Institute Journal","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.14503/THIJ-23-8209","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Resolution of Severe Portopulmonary Hypertension With Inhaled Treprostinil and Liver Transplantation.
Portopulmonary hypertension is a rare condition with a poor prognosis. Prompt management is essential for liver transplantation eligibility, a potentially curative option. This report presents a case of severe portopulmonary hypertension that resolved with a conservative therapeutic regimen of tadalafil, macitentan, and inhaled treprostinil, which ultimately enabled successful liver transplantation. There was no recurrence of pulmonary hypertension after transplantation, and the patient was weaned off most pulmonary arterial hypertension therapies. This case report is the first to provide evidence that inhaled treprostinil is a safe and effective alternative to continuous intravenous prostacyclins in portopulmonary hypertension.
期刊介绍:
For more than 45 years, the Texas Heart Institute Journal has been published by the Texas Heart Institute as part of its medical education program. Our bimonthly peer-reviewed journal enjoys a global audience of physicians, scientists, and healthcare professionals who are contributing to the prevention, diagnosis, and treatment of cardiovascular disease.
The Journal was printed under the name of Cardiovascular Diseases from 1974 through 1981 (ISSN 0093-3546). The name was changed to Texas Heart Institute Journal in 1982 and was printed through 2013 (ISSN 0730-2347). In 2014, the Journal moved to online-only publication. It is indexed by Index Medicus/MEDLINE and by other indexing and abstracting services worldwide. Our full archive is available at PubMed Central.
The Journal invites authors to submit these article types for review:
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