Materiały źródłowe

• #1 Current concepts in the management of hepatopulmonary syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2605324/

  The hepatopulmonary syndrome is characterized as the triad of liver disease, pulmonary gas exchange abnormalities leading to arterial deoxygenation and evidence of intrapulmonary vascular dilatations. […] Liver transplantation is considered to be the definitive treatment of hepatopulmonary syndrome with often successful reversal of hypoxemia, however other treatments have been trialed. […] The evidence base to support pharmacological and nonpharmacological therapies is discussed. […] The clinical features of HPS are typically pulmonary manifestations. Dyspnea is the most common complaint and can be insidious in presentation. […] The prognosis associated with HPS is poor. […] Many pharmacological treatments have been trialed in the management of HPS. […] An important therapy for HPS is oxygen supplementation which does lead to significant improvements in hypoxemia.

• #2 Review article: Update on current and emergent data on hepatopulmonary syndrome

  https://www.wjgnet.com/1007-9327/full/v24/i12/1285.htm

  Hepatopulmonary syndrome (HPS) is a frequent pulmonary complication of end-stage liver disease, characterized by impaired arterial oxygenation induced by intrapulmonary vascular dilatation. Its prevalence ranges from 4% to 47% in patients with cirrhosis due to the different diagnostic criteria applied among different studies. The only effective treatment which can modify the syndromes natural history is liver transplantation. Although it is usually asymptomatic, HPS imparts a high risk of pretransplantation mortality, independently of the severity of liver disease, while there is variable data concerning survival rates after liver transplantation. The aim of this review is to critically approach the existing literature of HPS and emphasize unclear points that remain to be unraveled by future research.

• #3 Review article: Update on current and emergent data on hepatopulmonary syndrome

  https://www.wjgnet.com/1007-9327/full/v24/i12/1285.htm

  Hepatopulmonary syndrome (HPS) constitutes a relatively frequent complication of end-stage liver disease, characterized by impairment of arterial oxygenation. The only effective treatment is liver transplantation, improving hypoxemia. While there are controversial data regarding HPS prognosis before and after liver transplantation, the question remains whether HPS constitutes an independent factor of morbidity, providing HPS patients priority for liver transplantation. […] The aim of this review is to provide a critical overview on prevalence, pathogenesis, diagnosis, clinical manifestations, treatment options and current data regarding prognosis before and after liver transplantation in patients with HPS. […] Diagnosis should be based on arterial blood gas analysis and alveolar arterial gradient calculation rather than a simple assessment of arterial hypoxemia. Several techniques have been developed for the evaluation of intrapulmonary vasodilatation, but contrast-enhanced echocardiography with agitated saline is considered the gold standard.

• #4 Hepatopulmonary syndrome

  https://johnsonmemorial.org/jmh-health/disease-conditions/con-20373340

  Hepatopulmonary syndrome is caused by blood vessels in the lungs expanding, also called dilating, and increasing in number. This condition affects the lungs of people who have advanced liver disease. […] A liver transplant is the only cure for hepatopulmonary syndrome. […] Being given oxygen, called supplemental oxygen therapy, is the main treatment for shortness of breath caused by low oxygen levels in the blood. A liver transplant is the only cure for hepatopulmonary syndrome.

• #5 Pulmonary complications of liver disease | Deranged Physiology

  https://derangedphysiology.com/main/required-reading/gastrointestinal-intensive-care/Chapter-523/pulmonary-complications-liver-disease

  Hepatopulmonary syndrome and porto-pulmonary hypertension had appeared in the exam at least once, as Question 28 from the second paper of 2022. In this case the candidates were instructed to compare these conditions with hepatorenal syndrome in a table presenting their definitions pathophysiology and specific treatment. […] The definition for this entity, given by the ERS task force in 2005, is „An arterial oxygenation defect induced by intrapulmonary vascular dilatations associated with hepatic disease”. […] The usual association is with chronic liver disease where such dilatations are more likely to form, but in all fairness even acute liver failure can produce this sort of thing, because there is nothing in the pathophysiology that might insist on a slow onset. […] In liver disease (acute or chronic), there is an increase in the amount of circulating vasodilatory mediators, specifically nitric oxide, endothelin-1 and carbon monoxide.

• #6 Pulmonary complications of liver disease | Deranged Physiology

  https://derangedphysiology.com/main/required-reading/gastrointestinal-intensive-care/Chapter-523/pulmonary-complications-liver-disease

  The result is the delivery of vasodilatory mediators to the pulmonary circulation. […] The excess vasodilation affects oxygenation by: Impairing hypoxic pulmonary vasoconstriction, Increasing intrapulmonary shunting, With chronic exposure, enhanced angiogenesis occurs, producing chronic arteriovenous shunts. […] This is apparently seen in a large number of cirrhosis patients (up to 30%). Platypnoea (where the patients experience an improvement in respiratory distress when they are laying flat) is often found, together with orthodeoxia (where oxygenation improves with lying down and deteriorates with upright position). […] Specific treatment includes Long term home oxygen, Cavoplasty, Angioembolisation of shunting AVMs, TIPS, and Transplant.

• #7 Decompensated Dyspneic Dilemma | AASLD

  https://www.aasld.org/liver-fellow-network/core-series/clinical-pearls/decompensated-dyspneic-dilemma

  Hepatopulmonary syndrome (HPS), a term coined in 1977, has been increasingly recognized as an important clinical cause of dyspnea in decompensated cirrhosis, which can significantly impact mortality and influence liver transplant candidacy. […] No disease specific treatment modalities exist for HPS, aside from liver transplantation. […] HPS may present with dyspnea, cyanosis, spider angiomata, clubbing, platypnea, orthodeoxia. […] Treatment: Supportive care. No disease specific treatment modalities exist for HPS, aside from liver transplantation.

• #8 Hepatopulmonary Syndrome: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/24190-hepatopulmonary-syndrome

  Hepatopulmonary syndrome causes shortness of breath, which worsens over time. It may improve when lying flat and worsen in an upright position. It can become so severe that its present even at rest. This gradual worsening process is also known as chronic respiratory failure. Everything doesnt fail at once, but eventually, your organs and tissues become seriously oxygen deprived (hypoxia). This is a dangerous condition that can do permanent damage to your brain and heart. If it goes untreated, it can be fatal. […] Supplemental oxygen is the only available treatment for hepatopulmonary syndrome. Oxygen therapy means breathing in 100% oxygen to supplement the oxygen your body makes. You may have hyperbaric oxygen therapy in sessions inside a special pressurized chamber at the hospital. Or you may have equipment you can take home, such as an oxygen mask. Oxygen helps alleviate symptoms and keep your tissues functioning.

• #9 Hepatopulmonary Syndrome – Pulmonary Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pulmonary-disorders/pulmonary-hypertension/hepatopulmonary-syndrome

  Hepatopulmonary syndrome should be suspected in patients with known liver disease who report dyspnea (particularly platypnea). Patients with such symptoms should have pulse oximetry. […] The main treatment is supplemental oxygen for symptoms. […] If the diagnosis is suspected, do pulse oximetry and consider arterial blood gas measurement and imaging (eg, contrast echocardiography). […] Treat with supplemental oxygen.

• #10 Liver Cirrhosis: Nursing Diagnoses, Care Plans, Assessment & Interventions | NurseTogether

  https://www.nursetogether.com/liver-cirrhosis-nursing-diagnosis-care-plan/

  Ineffective Breathing Pattern […] Advanced liver cirrhosis results in hepatopulmonary syndrome that causes shortness of breath and significantly low blood oxygen levels. […] Nursing Diagnosis: Ineffective Breathing Pattern […] Related to: […] Hepatopulmonary syndrome […] Expected outcomes: […] Patient will demonstrate a normal breathing pattern without respiratory distress. […] Patient will report the ability to breathe comfortably while sitting or lying flat. […] Interventions: […] 5. Prepare the patient for surgical interventions. Surgical interventions like abdominal paracentesis can help relieve abdominal pressure associated with liver cirrhosis and fluid accumulation, enabling the patient to breathe comfortably.

• #11 Chronic liver failure

  https://aci.health.nsw.gov.au/networks/eci/clinical/tools/chronic-liver-failure

  Hepato-pulmonary syndrome (HPS) is a disorder of pulmonary vascular dilatation and shunting. […] Supplemental oxygen is the mainstay of treatment. […] For refractory hypoxaemia, liver transplant is considered and higher priority is given to those with liver failure and HPS.

• #12 CE Activity | Hepatopulmonary Syndrome | Nurses

  https://www.statpearls.com/nurse/ce/activity/73756

  Hepatopulmonary syndrome is hypoxemia due to dilated intrapulmonary vasculature in the presence of liver disease or portal hypertension. It is a serious condition and can develop in any patient with chronic or acute liver disease. To avoid the high morbidity and mortality associated with this condition, it must be promptly diagnosed and treated. This activity reviews the evaluation and treatment of hepatopulmonary syndrome and highlights the role of the interprofessional team in the care of patients with this condition. […] Collaborate with an interprofessional team to enhance the care of patients with hepatopulmonary syndrome.

• #13 Hepatopulmonary syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hepatopulmonary-syndrome/diagnosis-treatment/drc-20477679

  Our caring team of Mayo Clinic experts can help you with your hepatopulmonary syndrome-related health concerns […] Being given oxygen, called supplemental oxygen therapy, is the main treatment for shortness of breath caused by low oxygen levels in the blood. A liver transplant is the only cure for hepatopulmonary syndrome. […] Hepatopulmonary syndrome care at Mayo Clinic.

• #14 Hepatopulmonary Syndrome – MD Searchlight

  https://mdsearchlight.com/gut-health/hepatopulmonary-syndrome/

  Oxygen therapy is commonly used for patients who have severe trouble getting enough oxygen (a condition known as hypoxemia). This treatment can provide relief until a more lasting solution, such as a liver transplant, is available. By enhancing oxygen levels and reducing hypoxemia, patients typically experience improved physical capability and overall quality of life. […] For individuals with Hepatopulmonary Syndrome (HPS), liver transplantation is the only recognized treatment that offers long-term survival advantages. Generally, this procedure helps improve hypoxemia within 6 to 12 months. Research indicates that the concentration of oxygen in your arteries (PaO2) and the difference in oxygen partial pressures in the alveoli and arterial blood (A-aO2) typically improve quickly after transplant, mostly within 6 months.

• #15 Medical treatment for hepatopulmonary syndrome: a systematic review

  https://rcm.mums.ac.ir/article_3255.html

  Hepatopulmonary syndrome (HPS) is known as a chronic liver disease associated with severe pulmonary deoxygenation due to intrapulmonary vascular vasodilation. […] Although liver transplantation is accepted as a main treatment of HPS, identifying effective drugs for recovery of HPS can be effective in postponing the transplantation and decreasing the mortality rate of patients before the transplantation. […] Various drugs have been proposed in treatment of HPS but more large controlled trial studies, is necessary to determine the exact efficacy of each drugs for HPS recovery. […] Although liver transplantation can be proposed as the main therapeutic strategy of HPS which have shown the most efficacy in treating HPS patients, pharmacological treatments can also result in beneficial effects not only as a bridge to transplant but also as an ultimate treatment of HPS.

• #16 Medical treatment for hepatopulmonary syndrome: a systematic review

  https://rcm.mums.ac.ir/article_3255.html

  According to these studies increased levels of TNF-alpha is a result of macrophages activation or bacterial translocation. […] Although PTX application has become beneficial during experimental studies, its efficacy on human is not clear. […] In one pilot trial PTX was not successful in improving the arterial oxygenation and declining the TNF-alpha levels which might be due to dose limiting gastrointestinal toxicity, severe HPS in patients, and intense oxygenation abnormalities compared with experimental models. […] Improvement of arterial oxygenation and hypoxemia is also shown by administrating one bolus of 3 mg/kg methylene blue in one controlled trial on 7 patients with cirrhosis and HPS. […] Garlic (Allium sativum L.) is not only a food but also a medicinal herb which can be applied as medical supplementation.

• #17 Medical treatment for hepatopulmonary syndrome: a systematic review

  https://rcm.mums.ac.ir/article_3255.html

  Unlike the previous mentioned medication (PTX and methylene blue) one recent randomized controlled trial has been performed on the efficacy of garlic on HPS patients. […] According to literature suppressing the bacterial translocation can be helpful in preventing HPS. […] According to one case report, although administering L-NAME reduced the exhaled NO and cardiac output of the patient with HPS, no significant improvement was observed in patients arterial oxygenation. […] Although various drugs have been proposed in treatment of HPS, Each medication is only studied through case reports or small trials, so revealing an effective therapeutic modality is essential to be based on powerful evidence.

• #18 Hepatopulmonary Syndrome – MD Searchlight

  https://mdsearchlight.com/gut-health/hepatopulmonary-syndrome/

  Patients with chronic liver disease who have a Model for End-Stage Liver Disease (MELD) score of 15 or more are referred for a liver transplant assessment. The MELD score is a reliable system used to gauge the severity of chronic liver disease. Patients with HPS are given extra points on the MELD score, pushing them higher up on the waiting list for liver transplantation. Its usually recommended that patients undergo liver transplantation before their disease becomes severe. […] Presently, theres no approved medical therapy specifically for HPS. Various treatments, such as garlic, pentoxifylline, mycophenolate mofetil, aspirin, methylene blue, inhaled nitric oxide, nitric oxide inhibitors, and somatostatin, have been experimented with but none have provided sufficient benefits and have not been approved by the FDA.

• #19 Successful treatment of severe hepatopulmonary syndrome with a sequential use of TIPS placement and liver transplantation | Annals of Hepatology

  https://www.elsevier.es/en-revista-annals-hepatology-16-articulo-successful-treatment-severe-hepatopulmonary-syndrome-S1665268119318162

  Hepatopulmonary syndrome (HPS) is a complication of portal hypertension (PH) defined by the presence of liver disease, abnormal pulmonary gas exchange and evidence of intrapulmonary vascular dilatations (IPVD) producing a right to left intrapulmonary shunt. […] Currently, liver transplantation is considered the treatment of choice for HPS. However, because of severe hypoxemia, the procedure is contraindicated in many cases. […] Because of lack of data, it is not possible to recommend the routine use of TIPS as a part of the conventional management of HPS. However, in patients with severe hypoxemia TIPS placement can reasonably be used as a bridge towards transplantation. […] At present, there is no effective medical therapy considered useful in the management of HPS. In fact, the current treatment of choice for HPS is liver transplantation with a mean in the largest single-institution series.

• #20 Hepatopulmonary Syndrome – MD Searchlight

  https://mdsearchlight.com/gut-health/hepatopulmonary-syndrome/

  A transjugular intrahepatic portosystemic shunt (TIPS), which is a procedure that creates a new connection inside the liver for blood flow, has limited data available regarding its use, and its results can vary. There are risks associated with TIPS, such as worsening of hypoxemia, as it could increase the widening and abnormal connection of blood vessels within the lungs. […] The use of pulmonary arterial coil embolization, which is a procedure to block blood flow in abnormal vessels, is limited. It can only be applied to select cases where large abnormal connections between arteries and veins are present.

• #21 Successful treatment of severe hepatopulmonary syndrome with a sequential use of TIPS placement and liver transplantation | Annals of Hepatology

  https://www.elsevier.es/en-revista-annals-hepatology-16-articulo-successful-treatment-severe-hepatopulmonary-syndrome-S1665268119318162

  Our patient was not considered a good transplant candidate due to her functional capacity and therefore TIPS placement was proposed as a bridge to liver transplantation. […] This allowed us to establish a clear relation between the presence of a functional TIPS and the resolution of the symptoms. […] In conclusion, based on the available evidence, it is not possible at present time to recommend the use of TIPS as a part of the conventional management of HPS. However, in patients with severe hypoxemia and marked deterioration of functional capacity who cannot undergo a liver transplantation, as the patient presented here, the placement of TIPS can reasonably be used as a bridge towards it.

• #22 Review article: Update on current and emergent data on hepatopulmonary syndrome

  https://www.wjgnet.com/1007-9327/full/v24/i12/1285.htm

  Liver transplantation constitutes the only established successful treatment that modifies the natural history of HPS, improving arterial hypoxemia within 6-12 mo. The identification of HPS through established diagnostic protocols among liver transplantation candidates in combination with the model for end-stage liver disease (MELD) exception policy to facilitate liver transplantation may achieve an 88% 5-year posttransplantation survival for HPS patients. […] Many pharmaceutical interventions have been studied both in humans and animal models, targeting the syndromes pathogenetic pathways, without reaching encouraging outcomes. […] Clearly, their poor outcomes as well as the small number of enrolled patients make the aforementioned studies insufficient to suggest effective therapeutic options for the management of HPS. In addition, these data underline the complexity of pathogenetic interactions in HPS and outline potential areas of interest and future research.

• #23 Hepatopulmonary syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hepatopulmonary-syndrome/symptoms-causes/syc-20373350

  Hepatopulmonary syndrome care at Mayo Clinic […] A liver transplant is the only cure for hepatopulmonary syndrome.

• #24 SciELO Brazil – The hepatopulmonary syndrome The hepatopulmonary syndrome

  https://www.scielo.br/j/abcd/a/GPzm8Rj8YvWQWpHJG7xzSyQ/

  The hepatopulmonary syndrome has been acknowledged as an important vascular complication in lungs developing systemic hypoxemia in patients with cirrhosis and portal hypertension. […] 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. […] The severity of hepatopulmonary syndrome is an important survival predictor and determine the improvement, the time and risks for liver transplantation. […] The treatment consists in oxygen supplementation and the transplantation continues being the only effective therapeutic. […] Liver transplantation is the only effective established therapy for HPS. […] The total resolution or significant improvement in gas exchange post-liver transplant is observed in more than 85% of reported patients and arterial hypoxemia normalization after transplantation is variable and may be superior to one year. […] The severity of HPS is an important predictor to determine survival, the better moment and the risks for liver transplantation.

• #25 Hepatopulmonary Syndrome: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/24190-hepatopulmonary-syndrome

  Like liver disease, hepatopulmonary syndrome is progressive over time. If youre already on the liver transplant waiting list, a diagnosis of hepatopulmonary syndrome will move you higher on the list. Healthcare providers understand that hepatopulmonary syndrome diminishes your overall prognosis and quality of life but also that a liver transplant can cure it. In the meantime, regular oxygen therapy can help sustain you.

• #26 Hepatic Cirrhosis Nursing Care Management and Study Guide

  https://nurseslabs.com/hepatic-cirrhosis/

  Nursing management for the patient with cirrhosis of the liver should focus on promoting rest, improving nutritional status, providing skin care, reducing risk of injury, and monitoring and managing complications. […] Assessment of the patient with cirrhosis should include assessing for: […] The major goals for a patient with cirrhosis are: […] The patient with cirrhosis needs close observation, first-class supportive care, and sound nutrition counseling. […] Monitor for bleeding and hemorrhage. […] The focus of discharge education is dietary instructions. […] The focus of documentation may include:

• #27 Hepatopulmonary Syndrome Masked by Chronic Obstructive Pulmonary Disease-A Case Report

  https://www.gavinpublishers.com/article/view/hepatopulmonary-syndrome-masked-by-chronic-obstructive-pulmonary-disease-a-case-report-

  Hepatopulmonary syndrome (HPS) is an uncommon complication of hepatic cirrhosis, which presents with dyspnea, platypnea and orthodeoxia. […] Hepatopulmonary syndrome is characterized by poor arterial oxygenation and intrapulmonary shunting. […] Till date, liver transplantation is the only proven treatment that has shown benefit in hepatopulmonary syndrome. […] Clinicians should always consider HPS as a differential diagnosis in any cirrhotic patient regardless of underlying pulmonary pathology. […] Prompt and early diagnosis could affect clinical decision-making and inform therapeutic management. […] More research is also needed on therapeutic interventions for patients with HPS as there is a dearth in the literature on comprehensive strategies for management. […] There needs to be more guideline directives from thought leaders and experts in the field of gastroenterology for a holistic approach to HPS research and clinical translation of research findings.

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