Impact of Hepatopulmonary Syndrome in Liver Transplantation Candidates and the Role of Angiogenesis

2021 ◽  
pp. 2102304
Author(s):  
Steven M. Kawut ◽  
Michael J. Krowka ◽  
Kimberly A. Forde ◽  
Nadine Al-Naamani ◽  
Karen L. Krok ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Liver Disease ◽  
Vascular Complications ◽  
Hepatopulmonary Syndrome ◽  
Functional Class ◽  
Hazard Ratio ◽  
Arterial Oxygen ◽  
Risk Of Death ◽  
Increased Risk

Hepatopulmonary syndrome affects 10–30% of patients with cirrhosis and portal hypertension. We evaluated the serum angiogenic profile of hepatopulmonary syndrome and assessed the clinical impact of hepatopulmonary syndrome in patients evaluated for liver transplantation.The Pulmonary Vascular Complications of Liver Disease 2 study was a multicentre, prospective cohort study of adults undergoing their first liver transplantation evaluation. Hepatopulmonary syndrome was defined as an alveolar-arterial oxygen gradient ≥15 mmHg (≥20 mmHg if age >64 years), positive contrast-enhanced transthoracic echocardiography, and absence of lung disease.We included 85 patients with hepatopulmonary syndrome and 146 patients without hepatopulmonary syndrome. Patients with hepatopulmonary syndrome had more complications of portal hypertension and slightly higher Model for End-stage Liver Disease-Na score compared to those without hepatopulmonary syndrome (median [interquartile range] 15 [12, 19] versus 14 [10, 17], p=0.006). Hepatopulmonary syndrome patients had significantly lower six minute walk distance and worse functional class. Hepatopulmonary syndrome patients had higher circulating angiopoietin-2, Tie2, tenascin-C, c-kit, VCAM-1, and von Willebrand factor levels, and lower E-selectin levels. Patients with hepatopulmonary syndrome had an increased risk of death (hazard ratio 1.80 [1.03–3.16], p=0.04) which persisted despite adjustment for covariates (hazard ratio 1.79 [1.02–3.15], p=0.04). This association did not vary based on levels of oxygenation reflecting the severity of hepatopulmonary syndrome.Hepatopulmonary syndrome was associated with a profile of abnormal systemic angiogenesis, worse exercise and functional capacity, and an overall increased risk of death.

scholarly journals Hepatopulmonary Syndrome. Review

2021 ◽  
Vol 6 (3) ◽  
pp. 45-52
Author(s):  
V. V. Potii ◽  
◽  
V. T. Kiriienko ◽  
E. I. Glukhova ◽  
O. S. Kunickaya ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Hepatopulmonary Syndrome ◽  
Gas Analysis ◽  
Arterial Oxygen ◽  
Common Symptom ◽  
Portopulmonary Hypertension ◽  
Oxygen Gradient ◽  
Arterial Blood ◽  
Intrapulmonary Shunting

Liver cirrhosis is often accompanied by complications from the pulmonary system. These include hydrothorax, portopulmonary hypertension and hepatopulmonary syndrome. Hepatic hydrothorax affects about 6-10% of patients with end-stage disease, which results in the passage of ascetic fluid into the pleural space through diaphragm defects. The common cause of the hepatopulmonary syndrome and portopulmonary hypertension is portal hypertension and portosystemic shunting, indicating that vasoactive and angiogenetic factors originating from the liver normally control the pulmonary circulation. Portopulmonary hypertension is like pulmonary arterial hypertension, which develops against the background of portal hypertension as a result of chronic liver disease or without other causes of increased pressure in the pulmonary vessels. The prevalence of portopulmonary hypertension ranges from 2% to 8.5% among patients with portal hypertension and is associated with a poor prognosis. Hepatopulmonary syndrome is characterized by intrapulmonary dilatation of microvessels, which causes intrapulmonary shunting and leads to impaired gas exchange in liver diseases, and is associated with a decrease in the quality and duration of life in patients with cirrhosis. Nitric oxide overproduction and angiogenesis seem to be the hallmarks of a complicated pathogenetic mechanism, leading to intrapulmonary shunting and ventilation-perfusion mismatch. A classification of hepatopulmonary syndrome according to the severity of hypoxemia has been suggested. Hepatopulmonary syndrome includes a triad: hepatic dysfunction and / or portal hypertension, dilatation of intrapulmonary vessels, and increased alveolar-arterial oxygen gradient. The prevalence of hepatopulmonary syndrome varies depending on the study groups from 5% to 30%. The most common symptom of the complication is shortness of breath, but in most cases, hepatopulmonary syndrome is asymptomatic. A decrease in oxygen saturation less than 96% corresponds to a decrease in PaO2<70 mm Hg and testifies to the possible development of hepatopulmonary syndrome. In the case of a positive screening, the patient should undergo arterial blood gas analysis, which helps to determine PaO2 and alveolar to arterial oxygen gradient. Conclusion. Contrast-enhanced echocardiography with agitated saline is the gold standard in the diagnosis of intrapulmonary dilatation. The only effective treatment for hepatopulmonary syndrome is liver transplantation. Complete recovery of hepatopulmonary syndrome after liver transplantation is observed within a year in most patients with cirrhosis and hepatopulmonary syndrome

scholarly journals The hepatopulmonary syndrome

2014 ◽  
Vol 27 (2) ◽  
pp. 145-147 ◽  
Author(s):  
Lucas Souto NACIF ◽  
Wellington ANDRAUS ◽  
Rafael Soares PINHEIRO ◽  
Liliana DUCATTI ◽  
Luciana BP HADDAD ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Clinical Manifestation ◽  
Therapeutic Option ◽  
Hepatopulmonary Syndrome ◽  
Supplemental Oxygen ◽  
Vascular Complication ◽  
Low Flow ◽  
Arterial Oxygen ◽  
Effective Therapeutic Option

INTRODUCTION: The hepatopulmonary syndrome has been acknowledged as an important vascular complication in lungs developing systemic hypoxemia in patients with cirrhosis and portal hypertension. Is formed by arterial oxygenation abnormalities induced from intrapulmonary vascular dilatations with liver disease. It is present in 4-32% of patients with cirrhosis. It increases mortality in the setting of cirrhosis and may influence the frequency and severity. Initially the hypoxemia responds to low-flow supplemental oxygen, but over time, the need for oxygen supplementation is necessary. The liver transplantation is the only effective therapeutic option for its resolution. AIM: To update clinical manifestation, diagnosis and treatment of this entity. METHOD: A literature review was performed on management of hepatopulmonary syndrome. The electronic search was held of the Medline-PubMed, in English crossing the headings "hepatopulmonary syndrome", "liver transplantation" and "surgery". The search was completed in September 2013. RESULTS: Hepatopulmonary syndrome is classically defined by a widened alveolar-arterial oxygen gradient (AaPO2) on room air (>15 mmHg, or >20 mmHg in patients >64 years of age) with or without hypoxemia resulting from intrapulmonary vasodilatation in the presence of hepatic dysfunction or portal hypertension. Clinical manifestation, diagnosis, classification, treatments and outcomes are varied. CONCLUSION: The severity of hepatopulmonary syndrome is an important survival predictor and determine the improvement, the time and risks for liver transplantation. The liver transplantation still remains the only effective therapeutic.

scholarly journals Pulmonary Complications in Candidates for Liver Transplantation

2020 ◽  
Vol 12 (3) ◽  
pp. 145-153
Author(s):  
Niloofar Razavi-Khorasani ◽  
Bobak Moazzami ◽  
Arash Dooghaie Moghadam ◽  
Pegah Eslami ◽  
Ermia Farokhi ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Liver Disease ◽  
Hepatopulmonary Syndrome ◽  
Pulmonary Complications ◽  
Clinical Complications ◽  
Organ Systems ◽  
Increased Risk ◽  
Risk Of Progression ◽  
Life Threatening ◽  
End Stage

The liver plays a pivotal role in maintaining the homeostasis of various organ systems. Also, end-stage liver disease and its complications are major causes of morbidity and mortality among adults. Individuals who develop a chronic liver disease are at increased risk of progression to multi-organ dysfunction, including the pulmonary system. The clinical complications of pulmonary problems related to the presence of liver disease range from mild (such as hypoxemia) to life-threatening diseases (such as portopulmonary hypertension and hepatopulmonary syndrome). Herein, the major pulmonary complications related to liver cirrhosis and considerations for performing liver transplantation are reviewed.

Hepatopulmonary Syndrome in an Adolescent With Insidious Hypoxia and Small Intrahepatic Portal Venous Shunts: Posttransplant Benefit From Sildenafil

2020 ◽  
Vol 23 (6) ◽  
pp. 467-471
Author(s):  
Voytek Slowik ◽  
Amber Hildreth ◽  
M Cristina Pacheco ◽  
Laura S Finn ◽  
Jeremy King ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Liver Disease ◽  
Hepatopulmonary Syndrome ◽  
Normal Activity ◽  
Portosystemic Shunting ◽  
Allograft Function ◽  
Persistent Fatigue

We report a patient without known preexisting liver disease who presented with hepatopulmonary syndrome (HPS) due to aberrant intrahepatic portal venous development leading to portosystemic shunting. Liver transplantation resulted in resolution of portal hypertension and HPS and sildenafil was safely tolerated in the treatment of persistent fatigue and hypoxemia. Twelve months later, patient has normal allograft function and has returned to normal activity.

Cognitive Impairment after Lacunar Stroke and the Risk of Recurrent Stroke and Death

2021 ◽  
pp. 1-7
Author(s):  
Abraham Kwan ◽  
Jingkai Wei ◽  
N. Maritza Dowling ◽  
Melinda C. Power ◽  
Zurab Nadareshvili ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Memory Impairment ◽  
Recurrent Stroke ◽  
Cox Proportional Hazards ◽  
Hazard Ratio ◽  
Cognitive Screening ◽  
Lacunar Stroke ◽  
Domain Specific ◽  
Risk Of Death ◽  
Increased Risk

Introduction: Patients with poststroke cognitive impairment appear to be at higher risk of recurrent stroke and death. However, whether cognitive impairment after lacunar stroke is associated with recurrent stroke and death remains unclear. We assessed whether global or domain-specific cognitive impairment after lacunar stroke is associated with recurrent stroke and death. Methods: We considered patients from the Secondary Prevention of Small Subcortical Strokes (SPS3) trial with a baseline cognitive exam administered in English by certified SPS3 personnel, 14–180 days after qualifying lacunar stroke. We considered a baseline score of ≤86 on the Cognitive Assessment Screening Instrument to indicate global cognitive impairment, <10 on the Clock Drawing on Command test to indicate executive function impairment, and domain-specific summary scores in the lowest quartile to indicate memory and nonmemory impairment. We used Cox proportional hazards models to estimate the association between poststroke cognitive impairment and subsequent risk of recurrent stroke and death. Results: The study included 1,528 participants with a median enrollment time of 62 days after qualifying stroke. During a mean follow-up of 3.9 years, 11.4% of participants had recurrent stroke and 8.2% died. In the fully adjusted models, memory impairment was independently associated with an increased risk of recurrent stroke (hazard ratio, 1.48; 95% confidence interval [95% CI]: 1.04–2.09) and death (hazard ratio, 1.87; 95% CI: 1.25–2.79). Global impairment (hazard ratio, 1.66; 95% CI: 1.06–2.59) and nonmemory impairment (hazard ratio, 1.74; 95% CI: 1.14–2.67) were associated with an increased risk of death. Discussion/Conclusion: After lacunar stroke, memory impairment was an independent predictor of recurrent stroke and death, while global and nonmemory impairment were associated with death. Cognitive screening in lacunar stroke may help identify populations at higher risk of recurrent stroke and death.

Portal Angiogenesis in Chronic Liver Disease Patients Correlates with Portal Pressure and Collateral Formation

2019 ◽  
Vol 37 (6) ◽  
pp. 498-508 ◽  
Author(s):  
Carolina A. Serrano ◽  
Simon C. Ling ◽  
Sofia Verdaguer ◽  
Miguel León ◽  
Nicolás Jarufe ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Liver Disease ◽  
Chronic Liver Disease ◽  
Venous Pressure ◽  
Portal Pressure ◽  
Peripheral Circulation ◽  
Chronic Liver ◽  
Noninvasive Markers ◽  
Collateral Formation

Background/Aims: One hallmark of chronic liver disease in patients with portal hypertension is the formation of portal-systemic collaterals in which angiogenesis has a fundamental role. We studied patients with chronic liver disease undergoing liver transplantation to correlate levels of circulating angiogenic factors in portal and peripheral circulation with portal pressure and portal-systemic collaterals. Methods: Sixteen patients who underwent liver transplantation were enrolled. During transplant surgery, we determined portal venous pressure and portal-systemic collateral formation. We determined angiogenics mediator levels in systemic and portal plasma. Peripheral plasma from healthy donors was measured as controls. Results: Vascular endothelial growth factor (VEGF)-R1 and 2, Ang-1 and 2, Tie2, FGF- 1 and 2, CD163, PDGFR-β, PDGFsRα, PDGF-AB and BB, CD163, TGF-β VASH-1 levels were significantly different in the controls in comparison to cases. Significantly decreased portal venous levels of Ang-1, FGF-1, PDGF-AB/BB, and CC were observed in patients with higher portal pressure. Peripheral VEGF, Ang-1, pPDGF-AB, BB, and CC were significantly decreased in patients with more severe collateral formation. While peripheral VEGF-R1 was higher in patients with severe collateral formation. For portal circulation, VEGF, Ang-1, ­pPDGF-AB, BB, and CC were significantly decreased in patients with more severe collateral formation Conclusions: Angiogenesis factors correlated with portal pressure and collateral formation and different patterns of circulating angiogenesis mediators were found in peripheral and portal blood of patients with chronic liver disease. These results support the importance of angiogenic pathways in cirrhosis and portal hypertension and highlight areas for further study to identify clinically useful noninvasive markers of portal pressure and collateral formation.

scholarly journals Current management of NAFLD

2016 ◽  
Vol 89 (1) ◽  
pp. 19-23 ◽  
Author(s):  
Mihai Alexandru Munteanu ◽  
Georgiana Anca Nagy ◽  
Petru Adrian Mircea
Keyword(s):  
Liver Disease ◽  
General Population ◽  
Pharmacological Therapy ◽  
Lifestyle Changes ◽  
Current Management ◽  
Alcoholic Fatty Liver ◽  
Risk Of Death ◽  
Increased Risk ◽  
Diet And Lifestyle ◽  
Alcoholic Fatty Liver Disease

Non-alcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease in Western countries. It affects about 1 billion individuals worldwide. While people with simple steatosis have no higher risk of death than the general population, people with non-alcoholic steatohepatitis are at increased risk of death compared to general population. Current management for NAFLD includes diet and lifestyle changes, management of underlying metabolic risk factors and pharmacological therapies. The objective of therapy is to prevent the complications. The problem with dietary and lifestyle interventions is that they are hard to implement. Compliance is the key. Until now, there is still no approved drug for the treatment of NAFLD. Insulin resistance is the main target of pharmacological therapy, but the question that we ask ourselves as physicians is who should receive medical treatment among NAFLD patients and for how long.

scholarly journals Portal Vein Thrombosis in a 21-Year-Old Man with Membranoproliferative Glomerulonephritis and Nephrotic Syndrome

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Ilya Seleznev ◽  
Dinara Jumadilova ◽  
Assiya Naushabayeva ◽  
Kairat Kabulbayev ◽  
Gulaiym Karashasheva ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Portal Hypertension ◽  
Liver Disease ◽  
Kidney Disease ◽  
Portal Vein ◽  
Membranoproliferative Glomerulonephritis ◽  
Microscopic Haematuria ◽  
Vein Thrombosis ◽  
Increased Risk ◽  
Portosystemic Collaterals

Membranoproliferative glomerulonephritis, one of the main causes of nephrotic syndrome, is associated with a state of hypercoagulability that leads to increased risk of thrombotic events. Portosystemic collaterals may reopen due to reversal of the flow within the existing veins and be a presenting feature of thrombosis. We describe a patient who presented with large portosystemic collaterals and signs of portal hypertension and was subsequently found to be affected by membranous proliferative glomerulonephritis. Proteinuria and microscopic haematuria in a patient with signs of portal hypertension and no pre-existing liver disease should raise the suspicion of an underlying kidney disease.

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