U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Hepatopulmonary syndrome

MedGen UID:
109071
Concept ID:
C0600452
Disease or Syndrome
Synonyms: Hepato Pulmonary Syndrome; Hepato-Pulmonary Syndrome; Hepato-Pulmonary Syndromes; Hepatopulmonary Syndrome; Hepatopulmonary Syndromes; Syndrome, Hepato-Pulmonary; Syndrome, Hepatopulmonary; Syndromes, Hepato-Pulmonary; Syndromes, Hepatopulmonary
SNOMED CT: Hepatopulmonary syndrome (371067004)
 
Monarch Initiative: MONDO:0004694

Definition

Hepatopulmonary syndrome (HPS) is a lung disease characterized by widening of arteries and veins (dilatation) in the lungs in people who have chronic liver disease. Because of the dilated vases, the workload of the heart increases and the blood pumped to the body does not have enough oxygen, leading to a decreased level of oxygen in the blood (hypoxemia). The normal diameter of the lung vessels ranges between 8 and 15 µm whereas when in HPS rises to between 15 and 500 µm. While many people with HPS don't have any obvious problems, the main reported symptom is shortness of breath (dyspnea) that is worse when standing or sitting up, and is relieved when lying down (platypnea). Symptoms related to chronic liver disease (generally cirrhosis) include small red spots on the skin (spider angiomas) and abnormal vascular dilatations. Some other symptoms that have been described are infections in the brain (brain abscesses), brain bleeding and an increased number of red blood cells in the blood (polycythemia). There is currently no effective medication for HPS. Oxygen therapy may improve the breathing in some cases. Liver transplant is an efficient treatment which improves the symptoms, even in severe cases. [from MONDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHepatopulmonary syndrome

Professional guidelines

PubMed

Yoshiji H, Nagoshi S, Akahane T, Asaoka Y, Ueno Y, Ogawa K, Kawaguchi T, Kurosaki M, Sakaida I, Shimizu M, Taniai M, Terai S, Nishikawa H, Hiasa Y, Hidaka H, Miwa H, Chayama K, Enomoto N, Shimosegawa T, Takehara T, Koike K
J Gastroenterol 2021 Jul;56(7):593-619. Epub 2021 Jul 7 doi: 10.1007/s00535-021-01788-x. PMID: 34231046Free PMC Article
Franchi-Abella S, Gonzales E, Ackermann O, Branchereau S, Pariente D, Guérin F; International Registry of Congenital Portosystemic Shunt members
Abdom Radiol (NY) 2018 Aug;43(8):2023-2036. doi: 10.1007/s00261-018-1619-8. PMID: 29730740
Khanna R, Sarin SK
J Hepatol 2014 Feb;60(2):421-41. Epub 2013 Aug 23 doi: 10.1016/j.jhep.2013.08.013. PMID: 23978714

Recent clinical studies

Etiology

Baiges A, Turon F, Simón-Talero M, Tasayco S, Bueno J, Zekrini K, Plessier A, Franchi-Abella S, Guerin F, Mukund A, Eapen CE, Goel A, Shyamkumar NK, Coenen S, De Gottardi A, Majumdar A, Onali S, Shukla A, Carrilho FJ, Nacif L, Primignani M, Tosetti G, La Mura V, Nevens F, Witters P, Tripathi D, Tellez L, Martínez J, Álvarez-Navascués C, Fraile López ML, Procopet B, Piscaglia F, de Koning B, Llop E, Romero-Cristobal M, Tjwa E, Monescillo-Francia A, Senzolo M, Perez-LaFuente M, Segarra A, Sarin SK, Hernández-Gea V, Patch D, Laleman W, Hartog H, Valla D, Genescà J, García-Pagán JC; REHEVASC, VALDIG an EASL consortium, Abernethy group
Hepatology 2020 Feb;71(2):658-669. Epub 2019 Aug 19 doi: 10.1002/hep.30817. PMID: 31211875
Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, Heimbach JK, Ramsay MA, Sitbon O, Sokol RJ
Transplantation 2016 Jul;100(7):1440-52. doi: 10.1097/TP.0000000000001229. PMID: 27326810
Nacif LS, Andraus W, Pinheiro RS, Ducatti L, Haddad LB, D'Albuquerque LC
Arq Bras Cir Dig 2014 Apr-Jun;27(2):145-7. doi: 10.1590/s0102-67202014000200012. PMID: 25004294Free PMC Article
Tumgor G
World J Gastroenterol 2014 Mar 14;20(10):2586-94. doi: 10.3748/wjg.v20.i10.2586. PMID: 24627594Free PMC Article
Khanna R, Sarin SK
J Hepatol 2014 Feb;60(2):421-41. Epub 2013 Aug 23 doi: 10.1016/j.jhep.2013.08.013. PMID: 23978714

Diagnosis

Yoshiji H, Nagoshi S, Akahane T, Asaoka Y, Ueno Y, Ogawa K, Kawaguchi T, Kurosaki M, Sakaida I, Shimizu M, Taniai M, Terai S, Nishikawa H, Hiasa Y, Hidaka H, Miwa H, Chayama K, Enomoto N, Shimosegawa T, Takehara T, Koike K
J Gastroenterol 2021 Jul;56(7):593-619. Epub 2021 Jul 7 doi: 10.1007/s00535-021-01788-x. PMID: 34231046Free PMC Article
Kumar P, Rao PN
N Engl J Med 2020 Mar 5;382(10):e14. doi: 10.1056/NEJMicm1901205. PMID: 32130817
Franchi-Abella S, Gonzales E, Ackermann O, Branchereau S, Pariente D, Guérin F; International Registry of Congenital Portosystemic Shunt members
Abdom Radiol (NY) 2018 Aug;43(8):2023-2036. doi: 10.1007/s00261-018-1619-8. PMID: 29730740
Bloom S, Kemp W, Lubel J
Intern Med J 2015 Jan;45(1):16-26. doi: 10.1111/imj.12590. PMID: 25230084
Khanna R, Sarin SK
J Hepatol 2014 Feb;60(2):421-41. Epub 2013 Aug 23 doi: 10.1016/j.jhep.2013.08.013. PMID: 23978714

Therapy

Carroll A, Boike JR
Curr Gastroenterol Rep 2023 Oct;25(10):232-241. Epub 2023 Aug 21 doi: 10.1007/s11894-023-00884-7. PMID: 37603109
Cartin-Ceba R, Krowka MJ
Clin Liver Dis 2019 Nov;23(4):683-711. Epub 2019 Aug 9 doi: 10.1016/j.cld.2019.06.003. PMID: 31563218
Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, Heimbach JK, Ramsay MA, Sitbon O, Sokol RJ
Transplantation 2016 Jul;100(7):1440-52. doi: 10.1097/TP.0000000000001229. PMID: 27326810
Machicao VI, Fallon MB
Semin Respir Crit Care Med 2012 Feb;33(1):11-6. Epub 2012 Mar 23 doi: 10.1055/s-0032-1301730. PMID: 22447256
Golbin JM, Krowka MJ
Clin Chest Med 2007 Mar;28(1):203-18, ix. doi: 10.1016/j.ccm.2006.11.004. PMID: 17338936

Prognosis

Yoshiji H, Nagoshi S, Akahane T, Asaoka Y, Ueno Y, Ogawa K, Kawaguchi T, Kurosaki M, Sakaida I, Shimizu M, Taniai M, Terai S, Nishikawa H, Hiasa Y, Hidaka H, Miwa H, Chayama K, Enomoto N, Shimosegawa T, Takehara T, Koike K
J Gastroenterol 2021 Jul;56(7):593-619. Epub 2021 Jul 7 doi: 10.1007/s00535-021-01788-x. PMID: 34231046Free PMC Article
Baiges A, Turon F, Simón-Talero M, Tasayco S, Bueno J, Zekrini K, Plessier A, Franchi-Abella S, Guerin F, Mukund A, Eapen CE, Goel A, Shyamkumar NK, Coenen S, De Gottardi A, Majumdar A, Onali S, Shukla A, Carrilho FJ, Nacif L, Primignani M, Tosetti G, La Mura V, Nevens F, Witters P, Tripathi D, Tellez L, Martínez J, Álvarez-Navascués C, Fraile López ML, Procopet B, Piscaglia F, de Koning B, Llop E, Romero-Cristobal M, Tjwa E, Monescillo-Francia A, Senzolo M, Perez-LaFuente M, Segarra A, Sarin SK, Hernández-Gea V, Patch D, Laleman W, Hartog H, Valla D, Genescà J, García-Pagán JC; REHEVASC, VALDIG an EASL consortium, Abernethy group
Hepatology 2020 Feb;71(2):658-669. Epub 2019 Aug 19 doi: 10.1002/hep.30817. PMID: 31211875
Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, Heimbach JK, Ramsay MA, Sitbon O, Sokol RJ
Transplantation 2016 Jul;100(7):1440-52. doi: 10.1097/TP.0000000000001229. PMID: 27326810
Koch DG, Fallon MB
Curr Opin Gastroenterol 2014 May;30(3):260-4. doi: 10.1097/MOG.0000000000000067. PMID: 24686436
Khanna R, Sarin SK
J Hepatol 2014 Feb;60(2):421-41. Epub 2013 Aug 23 doi: 10.1016/j.jhep.2013.08.013. PMID: 23978714

Clinical prediction guides

Yoshiji H, Nagoshi S, Akahane T, Asaoka Y, Ueno Y, Ogawa K, Kawaguchi T, Kurosaki M, Sakaida I, Shimizu M, Taniai M, Terai S, Nishikawa H, Hiasa Y, Hidaka H, Miwa H, Chayama K, Enomoto N, Shimosegawa T, Takehara T, Koike K
J Gastroenterol 2021 Jul;56(7):593-619. Epub 2021 Jul 7 doi: 10.1007/s00535-021-01788-x. PMID: 34231046Free PMC Article
Baiges A, Turon F, Simón-Talero M, Tasayco S, Bueno J, Zekrini K, Plessier A, Franchi-Abella S, Guerin F, Mukund A, Eapen CE, Goel A, Shyamkumar NK, Coenen S, De Gottardi A, Majumdar A, Onali S, Shukla A, Carrilho FJ, Nacif L, Primignani M, Tosetti G, La Mura V, Nevens F, Witters P, Tripathi D, Tellez L, Martínez J, Álvarez-Navascués C, Fraile López ML, Procopet B, Piscaglia F, de Koning B, Llop E, Romero-Cristobal M, Tjwa E, Monescillo-Francia A, Senzolo M, Perez-LaFuente M, Segarra A, Sarin SK, Hernández-Gea V, Patch D, Laleman W, Hartog H, Valla D, Genescà J, García-Pagán JC; REHEVASC, VALDIG an EASL consortium, Abernethy group
Hepatology 2020 Feb;71(2):658-669. Epub 2019 Aug 19 doi: 10.1002/hep.30817. PMID: 31211875
Lv Y, Fan D
Dig Dis Sci 2015 Jul;60(7):1914-23. Epub 2015 Mar 3 doi: 10.1007/s10620-015-3593-0. PMID: 25732713
Nacif LS, Andraus W, Pinheiro RS, Ducatti L, Haddad LB, D'Albuquerque LC
Arq Bras Cir Dig 2014 Apr-Jun;27(2):145-7. doi: 10.1590/s0102-67202014000200012. PMID: 25004294Free PMC Article
Tumgor G
World J Gastroenterol 2014 Mar 14;20(10):2586-94. doi: 10.3748/wjg.v20.i10.2586. PMID: 24627594Free PMC Article

Recent systematic reviews

Wu WK, Grogan WM, Ziogas IA, Patel YJ, Bacchetta M, Alexopoulos SP
Transplant Rev (Orlando) 2022 Apr;36(2):100693. Epub 2022 Apr 6 doi: 10.1016/j.trre.2022.100693. PMID: 35413506
Aragon Pinto C, Iyer VN, Albitar HAH, Anderson A, Cajigas H, Simonetto DA, Krowka MJ, DuBrock HM, Gallo de Moraes A
Respir Med Res 2021 Nov;80:100852. Epub 2021 Jul 30 doi: 10.1016/j.resmer.2021.100852. PMID: 34418867
Sokollik C, Bandsma RH, Gana JC, van den Heuvel M, Ling SC
J Pediatr Gastroenterol Nutr 2013 Jun;56(6):675-81. doi: 10.1097/MPG.0b013e31828b3750. PMID: 23412540

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...