Hepatopulmonary Syndrome

A syndrome characterized by the clinical triad of advanced chronic liver disease, pulmonary vascular dilatations, and reduced arterial oxygenation (HYPOXEMIA) in the absence of intrinsic cardiopulmonary disease. This syndrome is common in the patients with LIVER CIRRHOSIS or portal hypertension (HYPERTENSION, PORTAL).

Also Known As:

Networked: 542 relevant articles (25 outcomes, 34 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Liver Diseases (Liver Disease)
2.	Hypoxia (Hypoxemia)
3.	Liver Cirrhosis (Hepatic Cirrhosis)
4.	Hypertension (High Blood Pressure)
5.	End Stage Liver Disease

Experts

1.	Fallon, Michael B: 30 articles (01/2022 - 01/2003)
2.	Krowka, Michael J: 12 articles (01/2022 - 01/2003)
3.	Yi, Bin: 11 articles (12/2021 - 01/2013)
4.	Zhang, Junlan: 11 articles (11/2016 - 05/2003)
5.	Kawut, Steven M: 8 articles (01/2022 - 08/2008)
6.	Luo, Bao: 8 articles (04/2009 - 05/2003)
7.	Tang, Liping: 8 articles (04/2009 - 05/2003)
8.	Lee, Shou-Dong: 7 articles (01/2020 - 04/2013)
9.	Ling, Yiqun: 7 articles (04/2009 - 11/2003)
10.	Li, Xiao: 6 articles (03/2020 - 07/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hepatopulmonary Syndrome:

1.	QuercetinIBA 07/04/2016 - "Quercetin shows protective effects against hepatopulmonary syndrome (HPS), as demonstrated in a rat model. " 07/01/2007 - "Evaluation of the protective effects of quercetin in the hepatopulmonary syndrome." 07/04/2016 - "Quercetin alleviates pulmonary angiogenesis in a rat model of hepatopulmonary syndrome." 12/03/2022 - "Quercetin ameliorates the hepatopulmonary syndrome-induced lung injury through its anti-inflammatory, antioxidative and antifibrotic effects." 12/03/2022 - "Quercetin ameliorates acute lung injury in a rat model of hepatopulmonary syndrome."
2.	Nitric Oxide (Nitrogen Monoxide)FDA Link 11/01/2013 - "Our study aims to investigate the effects of Flavanoid on hepatopulmonary syndrome through its inhibition of nitric oxide. " 11/01/2021 - "Inhaled nitric oxide improves the hepatopulmonary syndrome: a physiologic analysis." 01/01/2020 - "A Role for Alveolar Exhaled Nitric Oxide Measurement in the Diagnosis of Hepatopulmonary Syndrome." 05/01/2010 - "[A case of hepatopulmonary syndrome with elevated exhaled nitric oxide]." 06/01/2009 - "The exact pathophysiology of hepatopulmonary syndrome remains unknown but nitric oxide is an important factor underlying hepatopulmonary syndrome. "
3.	Methadone (Dolophine)FDA LinkGeneric 07/01/2010 - "Improvement in hepatopulmonary syndrome after methadone withdrawal: a case report with implications for disease mechanism." 02/01/2011 - "Resolution of hepatopulmonary syndrome after methadone withdrawal as demonstrated by right to left shunt study." 02/01/2011 - "Follow-up images showed resolution of hepatopulmonary syndrome following methadone withdrawal although there had been no improvement in his liver disease. "
4.	EndotoxinsIBA 01/28/2005 - "Experimental study on the role of endotoxin in the development of hepatopulmonary syndrome." 01/01/2007 - "[Reduction of endotoxin may protect lung from injury in rats with hepatopulmonary syndrome]." 03/15/2012 - "Because constitutive and induced PIMs are pro-inflammatory in response to endotoxins and bacteria, there is a need to study their biology in inflammatory lung diseases such as sepsis, asthma, chronic obstructive pulmonary diseases, or hepato-pulmonary syndrome. " 01/01/2007 - "To study the protective effects of ofloxacin by reduction of endotoxin on lung in rats with hepatopulmonary syndrome (HPS). " 01/28/2005 - "Overproduction of TNF-alpha due to endotoxin stimulation of Kupffer cells via mitogen-activated protein kinase (MAPK) signal transduction pathway may be a major mechanism mediating the pathologic alterations of hepatopulmonary syndrome."
5.	Oxygen (Dioxygen)IBA 04/01/2008 - "The aim of this study was to assess oxygen desaturation during sleep in hepatopulmonary syndrome. " 04/21/2006 - "To study the effect of oral garlic on arterial oxygen pressure in children with hepatopulmonary syndrome. " 05/01/2019 - "Hand-agitated saline was used for CE and, by assessing left-chamber opacification, IPS was classified as grade 0 to 4. Grade 3/4 constituted significant IPS and hepatopulmonary syndrome (HPS) was defined as significant IPS with the arterial partial pressure of oxygen < 70 mmHg. " 03/01/2014 - "One plausible explanation is that the lower ambient oxygen found at higher elevation leads to pulmonary vasoconstriction, which mitigates the primary physiological lesion of hepatopulmonary syndrome, namely, pulmonary vasodilation." 03/01/2012 - "Among 26 patients of the positive group, nine had hypoxaemia (partial pressure of oxygen (PaO(2)) <70 mm Hg) and were diagnosed as having hepatopulmonary syndrome (HPS), the other 17 who had shown echocardiographic evidence of IPVDs but without hypoxaemia were diagnosed as having sub-clinical HPS. "
6.	Methylene Blue (Methylthioninium Chloride)FDA Link 09/04/2001 - "Methylene blue improves the hepatopulmonary syndrome." 11/07/2000 - "Methylene blue improves the hepatopulmonary syndrome." 10/20/1994 - "Methylene blue in the hepatopulmonary syndrome." 06/01/2010 - "Acute administration of methylene blue (MB) can reverse hypoxemia in patients with hepatopulmonary syndrome (HPS). " 11/07/2000 - "Intravenous methylene blue improved hypoxemia and hyperdynamic circulation in patients with liver cirrhosis and severe hepatopulmonary syndrome."
7.	Radioisotopes (Radionuclides)IBA 08/01/1998 - "Hepatopulmonary syndrome and radionuclide lung perfusion imaging." 03/01/1998 - "We report a patient with hepatopulmonary syndrome who showed a significant and durable (4 months') improvement in his symptoms, arterial oxygenation, and intrapulmonary shunts, as calculated by radionuclide studies after transjugular intrahepatic portosystemic shunt placement. "
8.	Ofloxacin (Ofloxacine)FDA LinkGeneric 01/01/2007 - "To study the protective effects of ofloxacin by reduction of endotoxin on lung in rats with hepatopulmonary syndrome (HPS). "
9.	Acetylcysteine (Siran)FDA LinkGeneric 04/01/2008 - "N-acetylcysteine effects on genotoxic and oxidative stress parameters in cirrhotic rats with hepatopulmonary syndrome." 04/01/2008 - "The results suggest protective effects after treatment with N-acetylcysteine in cirrhotic rats with hepatopulmonary syndrome." 04/01/2008 - "The aim of this study was to evaluate the potential antioxidant effects of N-acetylcysteine in hepatopulmonary syndrome, a complication of cirrhosis, using an experimental model of common bile duct ligation in rats. "
10.	GasesIBA 07/01/1997 - "The diagnosis of hepatopulmonary syndrome was based on clinical grounds, as well as abnormal arterial blood gases and positive contrast echocardiography. " 01/01/2014 - "Hepatopulmonary syndrome is formed by a triad of liver disease, intrapulmonary vascular dilatation and changes in blood gases. " 11/01/2013 - "Hepatopulmonary syndrome is formed by a triad of liver disease, intrapulmonary vascular dilatation and changes in blood gases. " 11/01/2003 - "Tin protoporphyrin treatment normalized carboxyhemoglobin and improved arterial blood gases and intrapulmonary vasodilatation, reflecting partial reversal of hepatopulmonary syndrome. "

Therapies and Procedures

1.	Liver Transplantation 02/01/2010 - "Improved survival after liver transplantation in patients with hepatopulmonary syndrome." 09/28/2006 - "To study the presence of sustained low diffusing capacity (DL(CO)) after liver transplantation (LT) in patients with hepatopulmonary syndrome (HPS). " 11/01/2000 - "The objective of this study was to rehabilitate a patient with hepatopulmonary syndrome (HPS) who underwent living-related orthotopic liver transplantation (LT). " 04/01/1995 - "In the context of persisting uncertainty about the cause and treatment of the hepatopulmonary syndrome, future studies must focus on better understanding the pathophysiology of the hepatopulmonary syndrome, predicting reversibility after liver transplantation, and identifying other treatment options." 10/28/2023 - "Outcomes of liver transplantation for hepatopulmonary syndrome in patients with concomitant respiratory disease."
2.	Transplantation 09/01/1998 - "Five patients met the criteria for the diagnosis of the hepatopulmonary syndrome before transplantation; the syndrome was cured by transplantation. " 10/01/2021 - "This suggests that patients with hepatopulmonary syndrome do benefit from transplantation." 01/01/2021 - "50 Years Ago in TheJournalofPediatrics: Hepatopulmonary Syndrome: Transplantation Is the Big Difference." 08/01/1995 - "This report confirms previous experience that the hepatopulmonary syndrome may be reversible after transplantation." 12/01/2000 - "About 80% of patients with the hepatopulmonary syndrome eventually have improved oxygenation after liver transplantation, thereby making worsening hypoxaemia the primary indication for transplantation in many instances. "
3.	Surgical Portasystemic Shunt (Portosystemic Shunt) 01/01/2019 - "Effect of Transjugular Intrahepatic Portosystemic Shunt Creation on Pulmonary Gas Exchange in Patients with Hepatopulmonary Syndrome: A Prospective Study." 04/01/2021 - "We present a case of a 53-year-old female with suspected NRH and hepatopulmonary syndrome with inconclusive liver biopsies and absent portosystemic shunts per abdominal imaging. " 12/01/2017 - "Clinical efficacy of transjugular intrahepatic portosystemic shunt in the treatment of hepatopulmonary syndrome." 02/01/2016 - "Congenital extrahepatic portosystemic shunt (CEPS) is a rare cause of hepatopulmonary syndrome (HPS). " 02/01/2016 - "Congenital extrahepatic portosystemic shunt: an underdiagnosed but treatable cause of hepatopulmonary syndrome."
4.	Therapeutics 06/01/2003 - "Furthermore, no medical therapy appears to be really useful in the treatment of hepatopulmonary syndrome. " 01/01/2013 - "A PubMED and Scopus search was conducted in January 2013 on the English literature published in any time period to find human and animal studies reporting pharmacological therapy of hepatopulmonary syndrome. " 04/01/2015 - "The main indications are portopulmonary or hepatopulmonary syndromes and other cardiorespiratory failure syndromes that are refractory to conventional therapy. " 09/01/2014 - "Anti COX-2 therapy could also be tested in human individuals, particularly in patients presenting a hepatopulmonary syndrome or HHT with small PAVM. " 06/01/2011 - "Hepatopulmonary syndrome (HPS) remains refractory to medical therapy. "
5.	Transjugular Intrahepatic Portasystemic Shunt 08/01/1997 - "Lack of efficacy of TIPS for hepatopulmonary syndrome." 12/07/2014 - "However, the role of TIPS in the treatment of hepatorenal and hepatopulmonary syndrome is not well defined. " 02/01/2002 - "[TIPSS for the treatment of hepatopulmonary syndrome]." 01/01/2019 - "To evaluate effect of transjugular intrahepatic portosystemic shunt (TIPS) creation on pulmonary gas exchange in patients with hepatopulmonary syndrome (HPS). " 09/01/2015 - "To evaluate the role of transjugular intrahepatic portosystemic shunt (TIPS) creation in the management of hepatopulmonary syndrome (HPS). "