Definition and research guide
This page combines a MeSH definition, publication statistics and selected research abstracts. It is a starting point for reading the literature, not a medical textbook or a systematic evidence review.
Content available: 4 complete abstracts (812 words), 10 references. Full article text is not stored or reproduced here; follow the PMID, DOI or PMC links to read the original sources and their access terms.
Definition
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).
Definition: NLM Medical Subject Headings (MeSH) scope note (Public domain (U.S. National Library of Medicine); attribution requested). View the MeSH record.
Also known as: Hepatopulmonary Syndromes; Syndrome, Hepatopulmonary; Syndromes, Hepatopulmonary; Hepato-Pulmonary Syndrome; Hepato Pulmonary Syndrome; Hepato-Pulmonary Syndromes; Syndrome, Hepato-Pulmonary; Syndromes, Hepato-Pulmonary.
Where it fits in the MeSH classification
- Broader topics
- Liver Diseases; Lung Diseases
Related concepts
- Hypoxemia
- Liver Cirrhosis
- Hypertension
- Portal
Research landscape
PubMed indexes 952 records tagged with the MeSH heading “Hepatopulmonary Syndrome” (counts retrieved 2026-11-04). 163 of them (17.1%) were published from 2020 onward, and the busiest period so far is 2010-2019 with 402 records.
Reviews account for 198 records (20.8%) and meta-analyses for 0. Randomized controlled trials number 4 (0.4%) as a share of all records on the topic. Randomized trials make up only a small share of this literature, so much of what is known comes from laboratory, observational or descriptive work.
Show the data
| Period | Records |
|---|---|
| through 1999 | 39 |
| 2000-2009 | 348 |
| 2010-2019 | 402 |
| 2020 onward | 163 |
Publication-type chart data
| Type | Records | Share |
|---|---|---|
| Reviews | 198 | 20.8% |
| Meta-analyses | 0 | 0.0% |
| Randomized controlled trials | 4 | 0.4% |
Method: PubMed query "Hepatopulmonary Syndrome"[MeSH Terms] with publication-type and date filters, retrieved 2026-11-04.
Reading the evidence in respiratory medicine
Research on airway and lung disease mixes lung-function tests, symptom diaries, imaging and hospital records, so what was measured and in whom matters as much as the result. Many conditions here, such as asthma and COPD, vary between people and over time, and exposure studies (smoking, pollution, workplace dust) show association rather than proof of cause. Nothing on this page is advice about treatment or inhalers; anyone with breathing difficulty should seek care from a qualified clinician. Check how the condition was defined, the severity of the people studied and whether outcomes were symptoms, lung function or hospital admissions.
Evidence from open-access studies
These open-access studies come from PubMed’s best-match results for this heading (papers whose main topic it is), ranked here by citation count, with one recent addition. Each abstract is reproduced in full under its recorded open licence so that you can read the authors’ own words. They illustrate the literature; they are not a systematic summary of it. Citation counts measure attention, not reliability or clinical benefit.
| Study / authors | Year | Source label / design | Europe PMC citations | Licence / source |
|---|---|---|---|---|
| Hepatocyte-derived exosomal MiR-194 activates PMVECs and promotes angiogenesis in hepatopulmonary syndrome | 2019 | Research article Design not recorded in structured metadata | 30 | cc by PMID 31700002 · DOI · PMC6838168 |
| Hepatopulmonary syndrome in patients with chronic liver disease: role of pulse oximetry | 2006 | Clinical trial Design not recorded in structured metadata | 28 | cc by PMID 16638132 · DOI · PMC1508152 |
| Pharmacological treatment for hepatopulmonary syndrome | 2013 | Review article Design not recorded in structured metadata | 14 | cc by PMID 24102057 · DOI · PMC3786536 |
| Wearable echocardiography enables real-time perioperative monitoring in a pediatric liver transplant recipient with hepatopulmonary syndrome and pulmonary arteriovenous malformations: a case report | 2026 | Research article Design not recorded in structured metadata | 0 | cc by PMID 42972513 · DOI · PMC13729345 |
Hepatocyte-derived exosomal MiR-194 activates PMVECs and promotes angiogenesis in hepatopulmonary syndrome [1]
Abstract reading summary — source excerpts
- Opening excerpt
- Hepatopulmonary syndrome (HPS) is a serious vascular complication in the setting of liver disease.
- Closing excerpt
- Thus, our results highlight that the exosome/miR-194 axis plays a critical pathologic role in pulmonary angiogenesis, representing a new therapeutic target for HPS.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (182 words)
Hepatopulmonary syndrome (HPS) is a serious vascular complication in the setting of liver disease. Factors produced by the liver are essential to regulate pulmonary angiogenesis in the pathogenesis of HPS; however, the pathogenic mechanisms of pulmonary angiogenesis are not fully understood. We investigated the role of HPS rat serum exosomes (HEs) and sham-operated rat serum exosomes (SEs) in the regulation of angiogenesis. We found that HEs significantly enhance PMVEC proliferation, migration, and tube formation. We further identified miR-194 was the most notably increased miRNA in HEs compared to SEs. Once released, hepatocyte-derived exosomal miR-194 was internalized by PMVECs, leading to the promotion of PMVEC proliferation, migration, and tube formation through direct targeting of THBS1, STAT1, and LIF. Importantly, the pathogenic role of exosomal miR-194 in initiating angiogenesis was reversed by P53 inhibition, exosome secretion inhibition or miR-194 inhibition. Additionally, high levels of miR-194 were found in serum exosomes and were positively correlated with P(A-a)O2 in HPS patients and rats. Thus, our results highlight that the exosome/miR-194 axis plays a critical pathologic role in pulmonary angiogenesis, representing a new therapeutic target for HPS.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 31700002 · DOI · PMC6838168
Hepatopulmonary syndrome in patients with chronic liver disease: role of pulse oximetry [2]
Abstract reading summary — source excerpts
- Opening excerpt
- BackgroundHepatopulmonary syndrome (HPS) is a rare complication of liver diseases of different etiologies and may indicate a poor prognosis.
- Closing excerpt
- ConclusionArterial SaO2 determination in supine and upright position is a useful non-invasive screening test for HPS and correlates well with the intrapulmonary shunt volume.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (236 words)
BackgroundHepatopulmonary syndrome (HPS) is a rare complication of liver diseases of different etiologies and may indicate a poor prognosis. Therefore, a simple non-invasive screening method to detect HPS would be highly desirable. In this study pulse oximetry was evaluated to identify patients with HPS.MethodsIn 316 consecutive patients with liver cirrhosis (n = 245), chronic hepatitis (n = 69) or non-cirrhotic portal hypertension (n = 2) arterial oxygen saturation (SaO2) was determined using a pulse oximeter. In patients with SaO2 or = 4% after change from supine to upright position further diagnostic procedures were performed, including contrast-enhanced echocardiography and perfusion lung scan.ResultsSeventeen patients (5.4%) had a pathological SaO2. Four patients (1.3%) had HPS. HPS patients had a significant lower mean SaO2 in supine (89.7%, SD 5.4 vs. 96.0%, SD 2.3; p = 0.003) and upright position (84.3%, SD 5.0 vs. 96.0%, SD 2.4; p = 0.001) and had a lower mean PaO2 (56.2 mm Hg, SD 15.2 vs. 71.2 mm Hg, SD 20.2; p = 0.02) as compared to patients without HPS. The mean deltaSaO2 (difference between supine and upright position) was 5.50 (SD 7) in HPS patients compared to non-HPS patients who showed no change (p = 0.001). There was a strong correlation between shunt volume and the SaO2 values (R = -0.94).ConclusionArterial SaO2 determination in supine and upright position is a useful non-invasive screening test for HPS and correlates well with the intrapulmonary shunt volume.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 16638132 · DOI · PMC1508152
Pharmacological treatment for hepatopulmonary syndrome [3]
Abstract reading summary — source excerpts
- Opening excerpt
- AimHepatopulmonary syndrome is a pulmonary dysfunction in the context of liver cirrhosis characterized by arterial deoxygenation.
- Closing excerpt
- Multicentered clinical trials are necessary to investigate these drugs.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (188 words)
AimHepatopulmonary syndrome is a pulmonary dysfunction in the context of liver cirrhosis characterized by arterial deoxygenation. Affected patients have increased morbidity and mortality, and many of them expire before undergoing liver transplantation. Therefore, finding medical therapy as a bridge to transplantation or as a final treatment is necessary. In this study, we aimed to review the current literature about pharmacological options available for treatment of hepatopulmonary syndrome.MethodsA 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.ResultsOut of 451 studies, 29 relevant articles were included. The number of patients, type, dose, duration, and mechanism of drugs in these studies was extracted and summarized separately. Most of pharmacologic agents act through inhibition of nitric oxide synthase and reduction in nitric oxide production, inactivation of endothelin-1, and treatment of bacterial translocation and pulmonary angiogenesis.ConclusionSeveral drugs have been applied for the treatment of HPS with conflicting results. However, no large randomized trial has been conducted probably due to low number of patients. Multicentered clinical trials are necessary to investigate these drugs.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 24102057 · DOI · PMC3786536
Wearable echocardiography enables real-time perioperative monitoring in a pediatric liver transplant recipient with hepatopulmonary syndrome and pulmonary arteriovenous malformations: a case report [4]
Abstract reading summary — source excerpts
- Opening excerpt
- BackgroundHepatopulmonary syndrome (HPS) with pulmonary arteriovenous malformations (PAVMs) creates high risks of hemodynamic instability and gas embolism during pediatric liver transplantation.
- Closing excerpt
- This technology may facilitate individualized fluid management and enable early warning of catastrophic complications in pediatric liver transplantation when standard echocardiographic modalities are contraindicated or insufficient.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (206 words)
BackgroundHepatopulmonary syndrome (HPS) with pulmonary arteriovenous malformations (PAVMs) creates high risks of hemodynamic instability and gas embolism during pediatric liver transplantation. Transesophageal echocardiography is relatively contraindicated due to esophageal varices, while intermittent transthoracic echocardiography lacks the temporal resolution to capture transient events. This is the first report of continuous wearable echocardiography for real-time perioperative monitoring in a pediatric liver transplant recipient with HPS and PAVMs.Case presentationA 3-year-old girl with biliary atresia and decompensated cirrhosis underwent deceased donor liver transplantation. Preoperative evaluation confirmed severe HPS with PAVMs. During surgery, fluid management was guided by continuous monitoring using wearable echocardiography to maintain hemodynamic stability. In particular, within 10 s after graft reperfusion, wearable echocardiography revealed numerous bright echogenic foci in all visualized cardiac chambers. This real-time finding provided immediate visual confirmation of gas embolism, which was associated with targeted multidisciplinary intervention. The surgery was successfully completed, and the patient was discharged on postoperative day 24.ConclusionsThis is the first detailed report demonstrating the utility of wearable echocardiography for continuous, real-time cardiac monitoring in a pediatric liver transplant recipient with severe HPS and PAVMs. This technology may facilitate individualized fluid management and enable early warning of catastrophic complications in pediatric liver transplantation when standard echocardiographic modalities are contraindicated or insufficient.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 42972513 · DOI · PMC13729345
Key reviews and meta-analyses
The most cited review-type papers among PubMed’s best-match results for this heading, listed with bibliographic details only. Follow the links for the full text and access terms.
- Research article Pulmonary-Hepatic vascular Disorders (PHD) [5]
- Research article Hepatopulmonary syndrome--a liver-induced lung vascular disorder [6]
- Research article International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension [7]
- Research article Portopulmonary hypertension and hepatopulmonary syndrome [8]
- Research article Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care [9]
- Research article The multiple dimensions of Platypnea-Orthodeoxia syndrome: A review [10]
References
- Chen L, Han Y, Li Y, Chen B, Bai X, Belguise K, et al.. Hepatocyte-derived exosomal MiR-194 activates PMVECs and promotes angiogenesis in hepatopulmonary syndrome. . 2019. PMID 31700002 · DOI · PMC6838168
- Deibert P, Allgaier HP, Loesch S, Müller C, Olschewski M, Hamm H, et al.. Hepatopulmonary syndrome in patients with chronic liver disease: role of pulse oximetry. . 2006. PMID 16638132 · DOI · PMC1508152
- Eshraghian A, Kamyab AA, Yoon SK. Pharmacological treatment for hepatopulmonary syndrome. . 2013. PMID 24102057 · DOI · PMC3786536
- Ye M, Zhang P, Jiang C, Zhou B, Yang X, Song H, et al.. Wearable echocardiography enables real-time perioperative monitoring in a pediatric liver transplant recipient with hepatopulmonary syndrome and pulmonary arteriovenous malformations: a case report. . 2026. PMID 42972513 · DOI · PMC13729345
- Rodríguez-Roisin R, Krowka MJ, Hervé P, Fallon MB, ERS Task Force Pulmonary-Hepatic Vascular Disorders (PHD) Scientific Committee. Pulmonary-Hepatic vascular Disorders (PHD). Eur Respir J. 2004. PMID 15516683 · DOI
- Rodríguez-Roisin R, Krowka MJ. Hepatopulmonary syndrome--a liver-induced lung vascular disorder. N Engl J Med. 2008. PMID 18509123 · DOI
- Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, Heimbach JK, Ramsay MA, et al.. International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension. Transplantation. 2016. PMID 27326810 · DOI
- Hoeper MM, Krowka MJ, Strassburg CP. Portopulmonary hypertension and hepatopulmonary syndrome. Lancet. 2004. PMID 15121411 · DOI
- Sundaram SS, Mack CL, Feldman AG, Sokol RJ. Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care. Liver Transpl. 2017. PMID 27650268 · DOI · PMC5177506
- Agrawal A, Palkar A, Talwar A. The multiple dimensions of Platypnea-Orthodeoxia syndrome: A review. Respir Med. 2017. PMID 28732833 · DOI
About this article
- Sources
- NLM Medical Subject Headings (definition, synonyms); NCBI PubMed (counts, identifiers); Europe PMC (open-access abstracts and citation counts).
- Verification
- Every reference was resolved against PubMed on 2026-11-04; title, year and DOI matched.
- Review state
- automated source verified; no human expert review has been recorded yet.
- Version
- 1, last updated 2026-11-04.
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Evidence strength
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