Hepatopulmonary syndrome

Summary

Hepatopulmonary syndrome (MONDO:0004694) is a disease and 13 clinical trials. Top therapeutic interventions include bosentan, midodrine, and norfloxacin. A subtype of liver disorder — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Clinical trials: 13

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	hepatopulmonary syndrome
Mondo ID	MONDO:0004694
EFO	EFO:1001346
MeSH	D020065
DOID	DOID:900
ICD-10-CM	K76.81
ICD-11	866605228
SNOMED CT	371067004
UMLS	C0600452
MedGen	109071
Is cancer (heuristic)	no

Disease family

This is a subtype of liver disorder. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › digestive system disorder › hepatobiliary disorder › liver disorder › hepatopulmonary syndrome

Related subtypes (31): polycystic echinococcosis, autosomal dominant polycystic liver disease, hepatorenal syndrome, hepatitis, hepatic vascular disorder, hepatic porphyria, fatty liver disease, cirrhosis of liver, drug-induced liver injury, perinatal jaundice due to hepatocellular damage, Aagenaes syndrome, transient familial neonatal hyperbilirubinemia, hyperbiliverdinemia, transient infantile hypertriglyceridemia and hepatosteatosis, idiopathic copper-associated cirrhosis, familial intrahepatic cholestasis, bile duct cyst, nodular regenerative hyperplasia of the liver, hepatoportal sclerosis, primitive portal vein thrombosis, glycogen storage disease due to liver phosphorylase kinase deficiency, liver and intrahepatic bile duct neoplasm, alcoholic liver disease, early-onset familial noncirrhotic portal hypertension, liver failure, fibrotic liver disease, intestinal failure–associated liver disease, liver abscess (disease), membranous obstruction of inferior vena cava, liver disease, severe congenital, cystic fibrosis-related liver disease

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 13.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	7
PHASE2	4
PHASE1	2

Top trials by phase / activity

NCT	Phase	Status	Title
NCT00362752	PHASE2	COMPLETED	A Pilot Study of Norfloxacin for Hepatopulmonary Syndrome
NCT01518595	PHASE2	TERMINATED	Bosentan for Treatment of Hepatopulmonary Syndrome in Patients With Liver Cirrhosis
NCT02021929	PHASE2	TERMINATED	Sorafenib for Hepatopulmonary Syndrome
NCT04577001	PHASE2	TERMINATED	Letrozole in Patients With Hepatopulmonary Syndrome
NCT00593658	PHASE1	TERMINATED	Pilot Study of Pentoxifylline for Hepatopulmonary Syndrome
NCT03600870	PHASE1	COMPLETED	Midodrine in Hepatopulmonary Syndrome
NCT01676597	Not specified	UNKNOWN	Efficacy of Pentoxifylline and Rifaximin Combination in Pentoxifylline Refractory Clinical and Subclinical Hepatopulmonary Syndrome
NCT02148536	Not specified	COMPLETED	Effect of Transjugular Intrahepatic Portosystemic Shunt on Oxygenation in Cirrhotic Patients With Hepatopulmonary Syndrome
NCT03092401	Not specified	UNKNOWN	Hepatopulmonary Syndrome and Postoperative Complications After Liver Transplantation : A Case-control Study
NCT03435406	Not specified	UNKNOWN	Investigation of the Prevalence of Hepatopulmonary Syndrome in Cirrhosis Patients Caused by Hepatitis B in Western China
NCT04004104	Not specified	UNKNOWN	Supine Exercise in Hepatopulmonary Syndrome Patients With Orthodeoxia
NCT05373134	Not specified	UNKNOWN	Efficacy and Safety of Pentoxifylline in Improving Oxygenation in Hepatopulmonary Syndrome
NCT05932927	Not specified	UNKNOWN	Analysis of Clinical Features of Chronic Liver Disease Complicated With Hepatopulmonary Syndrome

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
BOSENTAN	4	1
MIDODRINE	4	1
NORFLOXACIN	4	1
SORAFENIB	4	1