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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

A disorder more commonly seen in patients with chronic liver disease and portal hypertension. Pulmonary manifestations include dyspnea and hypoxemia. [from NCI]

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
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

Recent clinical studies

Etiology

Nanchal R, Subramanian R, Karvellas CJ, Hollenberg SM, Peppard WJ, Singbartl K, Truwit J, Al-Khafaji AH, Killian AJ, Alquraini M, Alshammari K, Alshamsi F, Belley-Cote E, Cartin-Ceba R, Dionne JC, Galusca DM, Huang DT, Hyzy RC, Junek M, Kandiah P, Kumar G, Morgan RL, Morris PE, Olson JC, Sieracki R, Steadman R, Taylor B, Alhazzani W
Crit Care Med 2020 Mar;48(3):e173-e191. doi: 10.1097/CCM.0000000000004192. PMID: 32058387
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
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
Nanchal R, Subramanian R, Karvellas CJ, Hollenberg SM, Peppard WJ, Singbartl K, Truwit J, Al-Khafaji AH, Killian AJ, Alquraini M, Alshammari K, Alshamsi F, Belley-Cote E, Cartin-Ceba R, Dionne JC, Galusca DM, Huang DT, Hyzy RC, Junek M, Kandiah P, Kumar G, Morgan RL, Morris PE, Olson JC, Sieracki R, Steadman R, Taylor B, Alhazzani W
Crit Care Med 2020 Mar;48(3):e173-e191. doi: 10.1097/CCM.0000000000004192. PMID: 32058387
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

Choe Y, Lee YJ, Lee YA, Ko JS, Shin CH
Front Endocrinol (Lausanne) 2024;15:1407686. Epub 2024 Oct 22 doi: 10.3389/fendo.2024.1407686. PMID: 39502571Free PMC Article
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

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