Current Therapy and Liver Transplantation for Portopulmonary Hypertension in Japan
Portopulmonary hypertension (PoPH) and hepatopulmonary syndrome are severe pulmonary complications associated with liver cirrhosis (LC) and portal hypertension. Three key pathways, involving endothelin, nitric oxide, and prostacyclin, have been identified in the development and progression of pulmon...
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-01-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/12/2/562 |
| _version_ | 1797440583696908288 |
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| author | Katsutoshi Tokushige Tomomi Kogiso Hiroto Egawa |
| author_facet | Katsutoshi Tokushige Tomomi Kogiso Hiroto Egawa |
| author_sort | Katsutoshi Tokushige |
| collection | DOAJ |
| description | Portopulmonary hypertension (PoPH) and hepatopulmonary syndrome are severe pulmonary complications associated with liver cirrhosis (LC) and portal hypertension. Three key pathways, involving endothelin, nitric oxide, and prostacyclin, have been identified in the development and progression of pulmonary arterial hypertension (PAH). To obtain a good effect with PAH-specific drugs in PoPH patients, it is important to diagnose PoPH at an early stage and promptly initiate therapy. The majority of therapeutic drugs are contraindicated for Child-Pugh grade C LC, and their effects decrease in the severe PAH stage. Among many LC patients, the measurement of serum brain natriuretic peptide levels might be useful for detecting PoPH. Previously, liver transplantation (LT) for PoPH was contraindicated; however, the indications for LT are changing and now take into account how well the PoPH is controlled by therapeutic drugs. In Japan, new registration criteria for deceased-donor LT have been established for PoPH patients. PoPH patients with a mean pulmonary arterial pressure <35 mmHg and pulmonary vascular resistance <400 dyn/s/cm<sup>−5</sup> are indicated for LT, regardless of whether they are using therapeutic drugs. Combined with PAH-specific drugs, LT may lead to excellent long-term outcomes in PoPH patients. We aimed to review current therapies for PoPH, including LT. |
| first_indexed | 2024-03-09T12:11:21Z |
| format | Article |
| id | doaj.art-0e975f041a364d098f832e3dcc3ab915 |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-09T12:11:21Z |
| publishDate | 2023-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-0e975f041a364d098f832e3dcc3ab9152023-11-30T22:51:39ZengMDPI AGJournal of Clinical Medicine2077-03832023-01-0112256210.3390/jcm12020562Current Therapy and Liver Transplantation for Portopulmonary Hypertension in JapanKatsutoshi Tokushige0Tomomi Kogiso1Hiroto Egawa2Department of Internal Medicine and Gastroenterology, Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, JapanDepartment of Internal Medicine and Gastroenterology, Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, JapanDepartment of Hepatopancreatic Surgery, Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, JapanPortopulmonary hypertension (PoPH) and hepatopulmonary syndrome are severe pulmonary complications associated with liver cirrhosis (LC) and portal hypertension. Three key pathways, involving endothelin, nitric oxide, and prostacyclin, have been identified in the development and progression of pulmonary arterial hypertension (PAH). To obtain a good effect with PAH-specific drugs in PoPH patients, it is important to diagnose PoPH at an early stage and promptly initiate therapy. The majority of therapeutic drugs are contraindicated for Child-Pugh grade C LC, and their effects decrease in the severe PAH stage. Among many LC patients, the measurement of serum brain natriuretic peptide levels might be useful for detecting PoPH. Previously, liver transplantation (LT) for PoPH was contraindicated; however, the indications for LT are changing and now take into account how well the PoPH is controlled by therapeutic drugs. In Japan, new registration criteria for deceased-donor LT have been established for PoPH patients. PoPH patients with a mean pulmonary arterial pressure <35 mmHg and pulmonary vascular resistance <400 dyn/s/cm<sup>−5</sup> are indicated for LT, regardless of whether they are using therapeutic drugs. Combined with PAH-specific drugs, LT may lead to excellent long-term outcomes in PoPH patients. We aimed to review current therapies for PoPH, including LT.https://www.mdpi.com/2077-0383/12/2/562portopulmonary hypertensionendothelin receptor antagonistsliver transplantation |
| spellingShingle | Katsutoshi Tokushige Tomomi Kogiso Hiroto Egawa Current Therapy and Liver Transplantation for Portopulmonary Hypertension in Japan Journal of Clinical Medicine portopulmonary hypertension endothelin receptor antagonists liver transplantation |
| title | Current Therapy and Liver Transplantation for Portopulmonary Hypertension in Japan |
| title_full | Current Therapy and Liver Transplantation for Portopulmonary Hypertension in Japan |
| title_fullStr | Current Therapy and Liver Transplantation for Portopulmonary Hypertension in Japan |
| title_full_unstemmed | Current Therapy and Liver Transplantation for Portopulmonary Hypertension in Japan |
| title_short | Current Therapy and Liver Transplantation for Portopulmonary Hypertension in Japan |
| title_sort | current therapy and liver transplantation for portopulmonary hypertension in japan |
| topic | portopulmonary hypertension endothelin receptor antagonists liver transplantation |
| url | https://www.mdpi.com/2077-0383/12/2/562 |
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