Materiały źródłowe

• #1 Therapy Insight: hepatopulmonary syndrome and orthotopic liver transplantation | Nature Reviews Gastroenterology & Hepatology

  https://www.nature.com/articles/ncpgasthep0965

  Hepatopulmonary syndrome (HPS) a pulmonary complication observed in patients who have chronic liver disease and/or portal hypertension is attributed to intrapulmonary vascular dilatation and induces severe hypoxemia. […] When a diagnosis of HPS is confirmed, knowing the degree of hypoxemia is crucial for optimum patient management. Patients who have a PaO2 50 mmHg but 60 mmHg should be prioritized for OLT. […] The decision to perform liver transplantation in patients with hepatopulmonary syndrome and a PaO2 below 50 mmHg is made on a case-by-case basis. […] When the diagnosis of hepatopulmonary syndrome is certain, the severity of the syndrome should be regularly assessed. […] The priority for liver allocation in patients with hepatopulmonary syndrome and a partial pressure of arterial oxygen (PaO2) in the range 5060 mmHg should be confirmed.

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

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

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

• #3 Hepatopulmonary Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562169/

  Oxygen therapy is recommended for patients with severe hypoxemia. It is usually given until a more definitive treatment like liver transplantation can be performed. An increase in oxygenation and reduction in hypoxemia leads to better exercise tolerance and improved quality of life. […] Liver transplantation is the only established treatment shown to provide long-term survival benefits for patients with HPS; it improves hypoxemia in 6 to 12 months. Studies have shown that PaO2 and A-aO2 reverse rapidly after transplant, mostly within 6 months. Intrapulmonary shunts also reverse but may take longer than 6 months. […] There is currently no medical therapy approved for HPS. Many medical treatments, including garlic, pentoxifylline, mycophenolate mofetil, aspirin, methylene blue, inhaled nitric oxide, nitric oxide inhibitors, and somatostatin, have been tried; however, none have been of conclusive benefit not FDA-approved.

• #4 Hepatopulmonary Syndrome: Symptoms, Causes & Treatment

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

  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. […] Liver transplantation is the only known cure for hepatopulmonary syndrome. After a successful liver transplant, vasodilation in your lungs and hypoxemia begin to reverse. It takes between six and 12 months for your pulmonary function to return to normal. However, not everyone qualifies for a liver transplant, and those who do often have to wait for one. This cure can be a race against time.

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

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

  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.

• #6 Hepatopulmonary syndrome: An update

  https://www.wjgnet.com/1948-5182/full/v13/i11/1699.htm

  Thus, patients with HPS are given higher priority for liver transplants compared to other factors. LT has been shown to improve oxygenation and shunt within the first year of transplant. […] All the patients with mild to moderate HPS should be evaluated every 3 to 6 mo with ABG. All patients with oxygen saturation less than 89% or partial pressure of oxygen less than 55 mmHg at rest, exercise and while sleep should be provided supplemental oxygen. […] Investigational therapies include pentoxifylline, garlic, inhaled NO, methylene blue, and others, but their effectiveness varies and further studies are needed to establish their benefits. […] There is no definitive proven treatment plan for HPS except LT. Thus, all patients with HPS should undergo expedited evaluation of LT.

• #7 Current concepts in the management of hepatopulmonary syndrome

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

  Liver transplantation is considered to be the definitive treatment of hepatopulmonary syndrome with often successful reversal of hypoxemia, however other treatments have been trialed. […] Many pharmacological treatments have been trialed in the management of HPS. Ostensibly such treatments target the putative mediators of HPS such as nitric oxide and TNF- although quite often pharmacological agents are tried with no substantive physiological basis. […] Antibiotic treatment has resulted in improvements in arterial oxygenation in HPS patients. […] An important therapy for HPS is oxygen supplementation which does lead to significant improvements in hypoxemia. Long-term supplementary oxygenation thus is advised in all hypoxemic HPS patients despite the lack of data to show improvements in survival.

• #8 Current concepts in the management of hepatopulmonary syndrome

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

  The only definitive treatment for HPS is orthotopic liver transplantation. The data to support this is incontrovertible although the mechanism of how the pulmonary vasculature is remodeled after transplantation is not clearly understood. What is known is that at least 85% of all cases of patients with HPS undergoing liver transplantation experience either significant improvement or complete resolution in hypoxemia. […] The understanding that orthotopic liver transplantation provides the best and often complete management of HPS has revolutionized the therapeutic approach to this unique disease entity. No longer is hypoxemia in such patients considered a relative contraindication for transplantation. In fact the degree of hypoxemia is considered critical in the consideration for liver transplantation. […] As no other accepted medical therapy exists at the present time for HPS other than supplementary oxygen, proceeding with orthotopic liver transplantation in suitable HPS patients is the best step forward.

• #9

  https://www.xiahepublishing.com/2310-8819/JCTH-2015-00044

  A severe and common pulmonary vascular complication of liver disease is hepatopulmonary syndrome (HPS. […] The only effective therapy is considered to be liver transplantation. Complete resolution of HPS after liver transplantation is seen within a year in most HPS patients. […] The only known effective therapy for HPS is LT, with an improvement in oxygenation at 1 year. After LT in HPS patients, the 5-year survival rate was shown to be approximately 76%, which is similar to patients with non-HPS. […] Patients with advanced HPS (PaO2 60 mmHg) are eligible for MELD exception points to increase priority for transplantation. […] To improve survival after LT, patients who are on the waiting list should be screened properly, and adequate oxygen should be given. […] HPS patients who are not candidates for LT may be candidates for localized resection or coil embolization of the dilated pulmonary vessels as a palliative treatment.

• #10

  https://link.springer.com/article/10.1007/s11938-005-0031-y

  The hepatopulmonary syndrome (HPS) is an important and often under-recognized vascular complication of cirrhosis and portal hypertension characterized by pulmonary vascular dilatation, which results in hypoxemia. […] Currently, OLT is the only therapy established to reverse intrapulmonary vasodilatation, although postoperative mortality is increased in patients with severe hypoxemia. […] In cirrhotic patients with HPS, particularly those with moderate hypoxemia (PaO2 60 mmHg), OLT should be considered prior to the development of severe deoxygenation. […] Supplemental oxygen should be given to patients with a PaO2 60 mmHg or those with exercise oxygen desaturation. […] For those patients with mild hypoxemia or those who are not OLT candidates, a trial of pharmacologic treatment may be considered.

• #11 Hepatopulmonary Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562169/

  Limited data exist on transjugular intrahepatic portosystemic shunt (TIPS) use, and clinical outcomes can vary. TIPS can aggravate the hyperkinetic circulatory state, increasing intrapulmonary vasodilatation and shunting and worsening hypoxemia. […] The use of this procedure is limited, as it can only be used in selected cases where there are large AV communications.

• #12 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

  Successful treatment of severe hepatopulmonary syndrome with a sequential use of TIPS placement and liver transplantation. […] Liver transplantation (LT) is the treatment of choice; however, severe hypoxemia may contraindicate LT. […] The use of transjugular intrahepatic porto-systemic shunts (TIPS) could be effective in HPS, although available data is limited. […] 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 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.

• #13 Successful treatment of severe hepatopulmonary syndrome with a sequential use of TIPS placement and liver transplantation

  https://www.medigraphic.com/cgi-bin/new/resumenI.cgi?IDARTICULO=19171

  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. […] Liver transplantation (LT) is the treatment of choice; however, severe hypoxemia may contraindicate LT. The use of transjugular intrahepatic portosystemic shunts (TIPS) could be effective in HPS, although available data is limited. […] The patient underwent TIPS placement. After four weeks, a significant improvement of dyspnea and a complete remission of orthodeoxia were seen. One year later, the patient was successfully transplanted. […] 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.

• #14 Hepatopulmonary Syndrome | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/120609

  Limited data exist on transjugular intrahepatic portosystemic shunt (TIPS) use, and clinical outcomes can vary. TIPS can aggravate the hyperkinetic circulatory state, increasing intrapulmonary vasodilatation and shunting and worsening hypoxemia. There is also the risk of hepatic decompensation and encephalopathy after TIPS. […] The use of this procedure is limited, as it can only be used in selected cases where there are large AV communications.

• #15

  https://www.xiahepublishing.com/2310-8819/JCTH-2015-00044

  Since the main disease mechanism involves vasodilation and angiogenesis mediated by NO and CO, treatment studies have targeted this pathway but with disappointing results. […] Pentoxifylline (PTX) is a nonspecific phosphodiesterase inhibitor that also inhibits TNF-, monocyte chemoattractant protein-1 (MCP-1), macrophage inhibitory protein-1 (MIP-1), interleukin-6, and interleukin-8 and decreases the expression of adhesion molecules and activation and proliferation of neutrophils. […] Methylene blue (MB) is an oxidizing agent and has a vasoconstrictor effect by blocking the stimulation of soluble guanylate cyclase by NO. […] Norfloxacin decreases pulmonary intravascular macrophage accumulation and reduces HPS by decreasing bacterial translocation. […] Transjugular intrahepatic portosystemic shunt (TIPS) placement is a medical intervention that has been associated with improvement of HPS in several case reports, but there is also a risk that TIPS may worsen HPS by increasing the hyperkinetic state, leading to more pulmonary vasodilatation, shunting, and hypoxemia. The American Association for the Study of Liver Disease guidelines do not recommend TIPS placement for the treatment of HPS.

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

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

  Hepatopulmonary syndrome is hypoxemia caused by pulmonary microvascular vasodilation in patients with portal hypertension; dyspnea and hypoxemia are worse when the patient is upright. […] The main treatment is supplemental oxygen for symptoms. […] Other therapies, such as somatostatin to inhibit vasodilation, are of modest benefit in only some patients. […] Coil embolization is virtually impossible because of the number and size of the lesions. […] Inhaled nitric oxide synthesis inhibitors may be a future treatment option but remains experimental. […] Hepatopulmonary syndrome may regress after liver transplantation or if the underlying liver disease subsides. […] Prognosis is poor in patients with hepatopulmonary syndrome, ranging from 40 to 60% at 2.5 years. […] Treat with supplemental oxygen.

• #17 Frontiers | Advances in Diagnostic Imaging of Hepatopulmonary Syndrome

  https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2021.817758/full

  Hepatopulmonary syndrome (HPS) is a serious pulmonary complication of progressive liver disease that leads to a poor clinical prognosis. Patients with HPS may develop acute respiratory failure, which requires intensive care and therapy. At present, the only effective treatment is liver transplantation; therefore, early diagnosis and timely treatment are of considerable significance. […] HPS should be considered if there is abnormal pulmonary arterial oxygenation in the presence of liver disease; however, abnormal blood oxygen saturation alone is not enough to diagnose HPS. […] Early detection and timely treatment may help improve the prognosis. […] Pulmonary angiography can directly reveal the dilated blood vessels and can also be used for embolization and other treatments. […] Pulmonary angiography has not been widely used in clinical settings as it is invasive and expensive. It is mainly used in patients with persistent hypoxemia who have a poor response to inhaled pure oxygen and need intrapulmonary vascular embolization.

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

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

  Pentoxifylline has favorable effects by selectively restricting the circulating tumor necrosis factor-alpha (TNF-) expression in inflammatory cells. […] 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. […] Methylene blue is an oxidizing factor and acts as another inhibitor of NOS and nitric oxide on guanylate cyclase which might be useful in preventing the pulmonary vasodilation in patients with HPS. […] Garlic (Allium sativum L.) is not only a food but also a medicinal herb which can be applied as medical supplementation. […] Norfloxacin application can suppress the severity of HPS. […] In one patient with HPS and severe hypoxemia, applying MMF increased oxygenation and decreased intrapulmonary shunt and eventually improved HPS and associated clinical sings. […] 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.

• #19

  https://journals.lww.com/smj/fulltext/9900/pentoxifylline_therapy_for_persistent.11.aspx

  Hepatopulmonary syndrome (HPS) is characterised by hypoxaemia caused by intrapulmonary vascular dilatations in the setting of advanced liver disease. […] Hepatopulmonary syndrome can persist for months after liver transplantation, despite transplantation being the only established treatment for HPS. […] Pentoxifylline is a phosphodiesterase inhibitor that has been linked to improved oxygenation in HPS. […] However, evidence for its use in transplanted patients has thus far been limited to a case report by Aljudaibi et al., where oxygenation improved in two transplanted patients with persistent HPS within 15 days of initiating pentoxifylline. […] Our case suggests pentoxifylline is beneficial in the management of persistent HPS after liver transplantation. Further trials are needed to establish its efficacy and safety in this population.

• #20 Methylene Blue Therapy for Hepatopulmonary Syndromelogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/jg200101160000011/2001/01/16/methylene-blue-therapy-hepatopulmonary-syndrome

  Hepatopulmonary syndrome (HPS) is characterized by cirrhosis with portal hypertension, hypoxemia, and pulmonary vascular dilation. Patients with HPS experience significant right-to-left shunting in blood flow, ventilation-perfusion mismatching, and limitations in O2 diffusion capacity. HPS is associated with a 3-year mortality rate of about 41 percent. Researchers from Austria cite a case report of a patient with HPS who had significant improvement in blood oxygen levels after administration of methylene blue. They now report on 7 patients with severe HPS and PaO2 of 60 mm Hg or less who received similar therapy. […] All patients received 3 mg/kg of methylene blue intravenously over a 15-minute period. Gas exchange, hemodynamic circulation, and systemic circulation parameters were measured at baseline and then hourly for 10 hours. Significant improvements in PaO2 (58 mm Hg to 74 mm Hg; P=0.006), shunt fraction (41 percent to 25 percent; P < 0.001), and cardiac output (10.6 to 8.6 L/min; P=0.008) were noted after the administration of the agent; the peak response occurred after 3 to 5 hours.

• #21 Hepatopulmonary Syndrome: Recent Advances in Treatment – Page 4

  https://www.medscape.com/viewarticle/778720_4

  Most studies have found that patients with HPS have an increased mortality compared with cirrhotic patients without HPS who have a similar severity of liver dysfunction. […] Liver transplant remains the only effective treatment of HPS, although post-transplant survival is often reduced compared with patients without HPS. […] An effective medical therapy for HPS has yet to be established. […] Results of small human trials of medical therapies for HPS have, in general, been disappointing. […] Other therapies that have been tried without success includes somatostatin analogues and indomethacin. […] Two children with HPS improved with long-term aspirin therapy; however, there have been no other studies to confirm this finding. […] Finally, a beneficial effect of garlic on oxygenation and dyspnea in HPS has been documented in two pilot trials, although the mechanism of action is unknown.

• #22 Article: A severe (type II) hepatopulmonary syndrome in a patient with idiopathic portal hypertension and treatment with paroxetine (abstract) – December 2005 – NJM

  https://www.njmonline.nl/article.php?i=75&d=238&a=374

  Currently, liver transplantation is the only effective therapy for such patients. […] there is also a proposal about considering paroxetine, a potent nitric oxide synthase inhibitor, for use in the hepatopulmonary syndrome. […] we present a patient with severe (type II) hepatopulmonary syndrome caused by idiopathic portal hypertension and discuss the consequences of paroxetine therapy.

• #23 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.

• #24

  https://emergency.zaslavsky.com.ua/index.php/journal/article/view/1640

  Hepatopulmonary syndrome (HPS) is one of the lung diseases associated with liver cirrhosis and portal hypertension. […] Liver transplantation allows for regression of intrapulmonary vascular dilatation in almost 100 % of cases, normalization of gas exchange and improves a 5-year survival after transplantation from 76 to 87 %. […] This is the only treatment method indicated for patients with severe HPS, defined by an arterial partial pressure of oxygen (PaO2) below 60 mm Hg. […] However, in the face of a global shortage of transplants, it is necessary to develop medical therapies to delay or even defer liver transplantation. […] This goal seems possible due to the growing understanding of the HPS pathophysiology and the development of therapies targeting key mechanisms, mainly inflammatory and angiogenic. […] Raevens S, Geerts A, Paridaens A, et al. Placental growth factor inhibition targets pulmonary angiogenesis and represents a therapy for hepatopulmonary syndrome in mice. […] Eshraghian A, Kamyab AA, Yoon SK. Pharmacological treatment for hepatopulmonary syndrome.

• #25 Advance in diagnosis and treatment of hepatopulmonary syndrome | Articles | New Medicine

  https://yxxz.whuznhmedj.com/en/journal/6294.html

  Hepatopulmonary syndrome (HPS) is a common pulmonary complication of advanced liver disease. The early diagnosis rate of HPS is low, and the treatment is limited, which seriously affects the survival rate and quality of life of patients. In this article, we summary pathogenesis, clinical manifestations, diagnosis and treatment progress of HPS, in order to improve the clinical understanding and diagnosis level of HPS. […] Placental growth factor inhibition targets pulmonary angiogenesis and represents a therapy for hepatopulmonary syndrome in mice. […] Inhaled nitric oxide improves the hepatopulmonary syndrome: a physiologic analysis.

• #26 A randomized, double-blind, placebo-controlled, phase II study of sorafenib in subjects with hepatopulmonary syndrome | Columbia University Department of Surgery

  https://columbiasurgery.org/clinical-trials/sorafenib-hps

  The main purpose of this clinical trial is to determine the safety and effects of the study drug, sorafenib, in adults diagnosed with hepatopulmonary syndrome (HPS). The study will evaluate how well the drug is tolerated and its effect on the level of oxygen in the blood and the function of the lung vessels. […] Trial Website: https://clinicaltrials.gov/show/NCT02021929 […] Specialty Area(s) Liver […] Study Status Accepting Patients.

• #27 Severe Hypoxemia After Liver Transplantation in the Hepatopulmonary Syndrome – ATC Abstracts

  https://atcmeetingabstracts.com/abstract/severe-hypoxemia-after-liver-transplantation-in-the-hepatopulmonary-syndrome/

  Hepato-pulmonary syndrome (HPS) is a complication of liver disease with liver transplantation as the only curative treatment. […] Postoperative severe hypoxemia occurred in 12 patients (24%). Among 12 patients, 2 patients was treated successfully by using extracorporeal membrane oxygenation (ECMO). […] ECMO could be one of effective treatment for post-transplant severe hypoxia caused by HPS.

• #28 Supine vs upright exercise in patients with hepatopulmonary syndrome and orthodeoxia: study protocol for a randomized controlled crossover trial | Trials | Full Text

  https://trialsjournal.biomedcentral.com/articles/10.1186/s13063-021-05633-7

  If our hypothesis proves correct, a supine exercise protocol would empower these patients to exercise for longer periods of time. Furthermore, supine exercise could enable a more effective long-term exercise training program. […] This could be particularly impactful for patients with HPS awaiting liver transplant, given that this is the only treatment for HPS, and that pre-transplant exercise capacity predicts post-transplant survival. […] If positive, this study will establish the efficacy of supine exercise in this population, enabling this to become a routine part of HPS management, including in non-transplant and pre-transplant settings. […] Our results would warrant future studies investigating the long-term physiologic and clinical benefits of a supine exercise training program in HPS, including effects on patient-relevant outcomes such as quality of life, dyspnea, and liver transplant outcomes.

• #29

  https://link.springer.com/article/10.1007/s11936-007-0006-5

  Liver transplantation is the only effective medical therapy for HPS. […] Compared with HPS, there are more therapeutic options that can bridge patients with PPHTN to transplantation. Drugs used to manage idiopathic pulmonary hypertension have shown promise in the treatment of PPHTN. Prostanoids, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors have improved transplant survival. […] Even with successful transplantation, HPS and PPHTN can persist or develop de novo. Long-term follow-up and surveillance of liver transplant recipients is thus indicated to identify HPS and PPHTN following surgery. […] Sussman N, Kaza V, Barshes N, et al.: Successful transplantation following medical management of portopulmonary hypertension: a single-center series. […] Plotkin JS, Kuo PC, Rubin LJ, et al.: Successful use of chronic epoprostenol as a bridge to liver transplantation in severe portopulmonary hypertension.

• #30

  https://link.springer.com/article/10.1007/s11936-007-0006-5

  Halank M, Marx C, Miehlke S, Hoeffken G: Use of aerosolized inhaled iloprost in the treatment of portopulmonary hypertension. […] Minder S, Fischler M, Muellhaupt B, et al.: Intravenous iloprost bridging to orthotopic liver transplantation in portopulmonary hypertension. […] Hoeper MM, Halank M, Marx C, et al.: Bosentan therapy for portopulmonary hypertension. […] Kuntzen C, Gulberg V, Gerbes AL: Use of a mixed endothelin receptor antagonist in portopulmonary hypertension: a safe and effective therapy? […] Makisalo, H, Koivusalo A, Vakkuri A, Hockerstedt K: Sildenafil for portopulmonary hypertension in a patient undergoing liver transplantation. […] Chua R, Keogh A, Miyashita M: Novel use of sildenafil in the treatment of portopulmonary hypertension. […] Reichenberger F, Voswinckel R, Steveling E, et al.: Sildenafil treatment for portopulmonary hypertension.

• #31 Hepatopulmonary Syndrome: Symptoms, Causes, Treatment, and Outlook

  https://www.healthline.com/health/hepatopulmonary-syndrome

  Oxygen therapy may be recommended if you have severe hypoxemia. Hypoxemia is an abnormally low concentration of oxygen in your blood. Oxygen therapy may be recommended until you can undergo a liver transplant. […] Liver transplantation is the only known effective therapy for hepatopulmonary syndrome and usually leads to significant improvements in oxygenation within 1 year of transplant. About 80% to 85% of people see improvements. […] The only known effective treatment for hepatopulmonary syndrome is a liver transplant. The majority of people see an improvement in their symptoms within a year after the surgery.

• #32 Hepatopulmonary syndrome in adults: Natural history, treatment, and outcomes – UpToDate

  https://www.uptodate.com/contents/hepatopulmonary-syndrome-in-adults-natural-history-treatment-and-outcomes

  Hepatopulmonary syndrome (HPS) is characterized by the triad of abnormal arterial oxygenation caused by intrapulmonary vascular dilatations in the setting of liver disease, portal hypertension, or congenital portosystemic shunts. […] The natural history, treatment, and outcomes of HPS are reviewed here. […] TREATMENT AND PROGNOSIS […] Mild to moderate hepatopulmonary syndrome […] – Observation […] – Oxygen supplementation […] Severe or very severe hepatopulmonary syndrome […] – Oxygen supplementation […] – Liver transplantation […] Other therapies […] Investigational […] Vasoactive medications […] false

• #33 Letter for the article Management options in decompensated cirrhosis | HMER

  https://www.dovepress.com/highlighting-hepatopulmonary-syndrome-an-important-consideration-in-de-peer-reviewed-fulltext-article-HMER

  Hepatopulmonary syndrome (HPS) is a progressive condition associated with worse prognosis in individuals with chronic liver disease. […] Treatment options for hepatopulmonary syndrome (HPS) involve starting long-term oxygen therapy for patients with severe HPS (PaO2 60 mmHg). Liver transplantation (LT) is the only treatment that can resolve HPS. […] Timely diagnosis of hepatopulmonary syndrome is crucial as it significantly affects management strategies and treatment outcomes. Early identification enables prompt initiation of appropriate interventions such as oxygen therapy and prioritization for liver transplantation, thereby improving patient prognosis and survival rates.

Zobacz więcej