Materiały źródłowe

• #1 Hepatopulmonary syndrome: What we know and what we would like to know

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4932208/

  Hepatopulmonary syndrome (HPS) is characterized by abnormalities in blood oxygenation caused by the presence of intrapulmonary vascular dilations (IPVD) in the context of liver disease, generally at a cirrhotic stage. […] Although it is generally asymptomatic it does have an impact on their quality of life and survival. […] The symptoms which have classically been associated with HPS are dyspnea and platypnea. […] In the largest study covering patients listed for LT, dyspnea was present in 48% of HPS patients, and was more frequent than in patients without HPS, with significant differences. […] Platypnea, which means a worsening in dyspnea when a patient is standing rather than lying down, is considered a pathognomonic characteristic of HPS. […] HPS patients have a lower quality of life, rank higher in the New York Heart Association functional classification and suffer significant oxygen desaturation whilst sleeping.

• #2 Hepatopulmonary Syndrome – StatPearls – NCBI Bookshelf

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

  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. […] HPS is defined as reduced arterial oxygen saturation due to dilated pulmonary vasculature in the presence of advanced liver disease or portal hypertension. […] The patient usually presents with dyspnea in the setting of liver disease. The onset is insidious, and dyspnea worsens with exertion. In the early stages, most patients are asymptomatic. […] Patients with HPS have 2 times higher mortality compared with patients with cirrhosis without HPS. The condition is associated with a poor quality of life and inferior functional status. The average life span in a patient with HPS (10.5 months) is significantly reduced compared with patients with chronic liver disease patients without HPS (40.8 months). The mortality risk increases with the severity of the disease, with a worse prognosis in patients with very severe disease. […] HPS is a fatal disease that drastically reduces the life span of a patient with liver disease. Most patients will have progressive vasodilation and worsening hypoxemia. Without liver transplantation, death is inevitable, as no other medical therapies are currently available.

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

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

  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. […] These changes in the lungs make it hard for red blood cells to take in oxygen. Then the lungs can’t send enough oxygen to the body. This leads to low oxygen levels in the blood, also called hypoxemia. […] Often, there are no symptoms of hepatopulmonary syndrome. If there are symptoms, they can include: Shortness of breath that’s worse when sitting or standing and gets better when lying down. Clubbing of the fingers, in which the fingertips spread out and become rounder than usual. Broken blood vessels under the skin, called spider angioma. Bluish tinge of the lips and skin in white people. In Black and brown people, the lips or tongue might look light gray. This change in color is called cyanosis.

• #4 Hepatopulmonary syndrome – Hancock Health

  https://www.hancockhealth.org/de/mayo-health-library/hepatopulmonary-syndrome/

  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. […] These changes in the lungs make it hard for red blood cells to take in oxygen. Then the lungs cant send enough oxygen to the body. This leads to low oxygen levels in the blood, also called hypoxemia. […] Often, there are no symptoms of hepatopulmonary syndrome. If there are symptoms, they can include: Shortness of breath thats worse when sitting or standing and gets better when lying down. Clubbing of the fingers, in which the fingertips spread out and become rounder than usual. Broken blood vessels under the skin, called spider angioma. Bluish tinge of the lips and skin in white people. In Black and brown people, the lips or tongue might look light gray. This change in color is called cyanosis.

• #5 Hepatopulmonary Syndrome – StatPearls – NCBI Bookshelf

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

  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. […] HPS is defined as reduced arterial oxygen saturation due to dilated pulmonary vasculature in the presence of advanced liver disease or portal hypertension. […] The patient usually presents with dyspnea in the setting of liver disease. The onset is insidious, and dyspnea worsens with exertion. In the early stages, most patients are asymptomatic. […] Patients with HPS have 2 times higher mortality compared with patients with cirrhosis without HPS. The condition is associated with a poor quality of life and inferior functional status. The average life span in a patient with HPS (10.5 months) is significantly reduced compared with patients with chronic liver disease patients without HPS (40.8 months). The mortality risk increases with the severity of the disease, with a worse prognosis in patients with very severe disease. […] HPS is a fatal disease that drastically reduces the life span of a patient with liver disease. Most patients will have progressive vasodilation and worsening hypoxemia. Without liver transplantation, death is inevitable, as no other medical therapies are currently available.

• #6 Hepatopulmonary syndrome – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/hepatopulmonary-syndrome/

  Hepatopulmonary (hep-uh-toe-POOL-moe-nar-e) syndrome is an uncommon condition that affects the lungs of people with advanced liver disease. Hepatopulmonary syndrome is caused by blood vessels in the lungs expanding (dilating) and increasing in number, making it hard for red blood cells to properly absorb oxygen. This leaves the lungs unable to deliver adequate amounts of oxygen to the body, which leads to low oxygen levels (hypoxemia). […] Most people with hepatopulmonary syndrome have no symptoms. If symptoms occur, they may include: Shortness of breath, especially when sitting or standing, Clubbing of the fingers, in which the fingertips spread out and become rounder than normal, Broken blood vessels under the skin (spider angioma), Bluish tinge of the lips and skin (cyanosis). […] 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.

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

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

  Hepatopulmonary syndrome is a complication of liver disease that occurs when blood vessels in your lungs expand. This expansion of the blood vessels can interfere with your lungs ability to transfer oxygen to your red blood cells. The most prominent symptom is shortness of breath. […] The hallmark symptom of hepatopulmonary syndrome is severe shortness of breath, which is reported in about 95% of people. About 82% of people have symptoms of liver disease before developing lung symptoms. […] Some people experience platypnea, which is shortness of breath thats worse when standing or sitting upright and improves when lying down. It affects as many as 88% of people with hepatopulmonary syndrome. […] Hepatopulmonary syndrome usually develops after years of liver disease. It can develop in people with mildly impaired or severely impaired liver function.

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

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

  Hepatopulmonary syndrome is a complication of liver disease that occurs when blood vessels in your lungs expand. This expansion of the blood vessels can interfere with your lungs ability to transfer oxygen to your red blood cells. The most prominent symptom is shortness of breath. […] The hallmark symptom of hepatopulmonary syndrome is severe shortness of breath, which is reported in about 95% of people. About 82% of people have symptoms of liver disease before developing lung symptoms. […] Some people experience platypnea, which is shortness of breath thats worse when standing or sitting upright and improves when lying down. It affects as many as 88% of people with hepatopulmonary syndrome. […] Hepatopulmonary syndrome usually develops after years of liver disease. It can develop in people with mildly impaired or severely impaired liver function.

• #9 Hepatopulmonary syndrome history and symptoms – wikidoc

  https://www.wikidoc.org/index.php/Hepatopulmonary_syndrome_history_and_symptoms

  The hallmark of hepatopulmonary syndrome is platypnea and orthodeoxia. A positive history of liver disease and dyspnea is suggestive of hepatopulmonary syndrome. Other sign and symptoms of hepatopulmonary syndrome may include spider angiomata, clubbing of fingers or toes, and cyanosis. […] The majority of patients with hepatopulmonary syndrome (80 percent) initially present with their liver disease symptoms. The remaining 20 percent present with pulmonary symptoms. The most complication and presentation is insidiously progressive shortness of breath (dyspnea) at rest or upon exertion. […] The hallmark of hepatopulmonary syndrome is platypnea (dyspnea in the upright position which is improved in supine position), and orthodeoxia (a drop of 4mmHg in PaO2 or 5% in saturation when moving from the supine to the standing position). Almost 90 percent of patients with HPS present with this specific finding.

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

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

  Hepatopulmonary syndrome is a complication of liver disease that occurs when blood vessels in your lungs expand. This expansion of the blood vessels can interfere with your lungs ability to transfer oxygen to your red blood cells. The most prominent symptom is shortness of breath. […] The hallmark symptom of hepatopulmonary syndrome is severe shortness of breath, which is reported in about 95% of people. About 82% of people have symptoms of liver disease before developing lung symptoms. […] Some people experience platypnea, which is shortness of breath thats worse when standing or sitting upright and improves when lying down. It affects as many as 88% of people with hepatopulmonary syndrome. […] Hepatopulmonary syndrome usually develops after years of liver disease. It can develop in people with mildly impaired or severely impaired liver function.

• #11 SSA – POMS: DI 23022.955 – Hepatopulmonary Syndrome – 08/25/2020

  https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022955

  Hepatopulmonary syndrome (HPS) is a severe condition involving shortness of breath and hypoxemia in people with chronic liver disease that has advanced to the point that it affects their lungs. […] The signs of pulmonary involvement may include: Digital clubbing (the appearance of changes in the areas under and around the toenails and fingernails); Cyanosis (a condition in which the lips, fingers, and toes appear blue); Dyspnea (shortness of breath); Platypnea (shortness of breath that is relieved when lying down and worsens when standing or sitting); and Orthodeoxia (fall in arterial blood oxygen while in the upright position). […] HPS worsens the prognosis of individuals with cirrhosis and other liver diseases. Individuals who are not candidates for liver transplantation have a median survival of 2 years. Mortality is usually associated with complications of hepatic disease.

• #12

  https://step2.medbullets.com/pulmonary/121735/hepatopulmonary-syndrome

  A 60-year-old man presents with increasing shortness of breath. He reports that this symptom worsens when he is in the upright position and improves when he is laying in bed. […] Symptoms include dyspnea on exertion or at rest, platypnea (increased dyspnea when upright and improves when supine), and weakness and fatigue. […] Physical exam may reveal orthodeoxia (decreased arterial oxygen tension when upright and improves when supine) and signs of liver disease such as spider angiomata, palmar erythema, gynecomastia, and jaundice. […] Liver transplantation may result in resolution of hepatopulmonary syndrome.

• #13 5.00 – Digestive – Adult | Disability | SSA

  https://www.ssa.gov/disability/professionals/bluebook/5.00-Digestive-Adult.htm

  e. Hepatopulmonary syndrome (5.05E) is arterial deoxygenation due to intrapulmonary vascular dilation and arteriovenous shunting associated with CLD. Clinical findings of hepatopulmonary syndrome include platypnea (shortness of breath relieved when lying down) and orthodeoxia (low arterial blood oxygen while in the upright position), when presenting in the context of CLD. […] E. Hepatopulmonary syndrome (see 5.00C2e) documented by 1 or 2: 1. Arterial PaO2 measured by an ABG test, while at rest, breathing room air, less than or equal to: a. 60 mm Hg, at test sites less than 3,000 feet above sea level; or b. 55 mm Hg, at test sites from 3,000 through 6,000 feet above sea level; or c. 50 mm Hg, at test sites over 6,000 feet above sea level; or 2. Intrapulmonary arteriovenous shunting as shown by contrast-enhanced echocardiography or macroaggregated albumin lung perfusion scan.

• #14 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. […] Mild cases may not have any noticeable symptoms. The first symptom people do notice is typically shortness of breath (dyspnea). This can have a lot of causes, and liver disease can cause it in several different ways. So people with liver disease and their doctors dont always recognize it as a sign of hepatopulmonary syndrome. As the condition worsens, you may notice other symptoms, such as:

• #15 Hepatopulmonary Syndrome: Symptoms, Causes & Treatment

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

  Platypnoea: Shortness of breath that gets worse when you sit up and better when you lie down. […] Orthopnea: Shortness of breath that gets worse when you lie down and better when you sit up. […] Cyanosis: A blueish tint to your lips, nail beds or skin from the lack of oxygen. […] Clubbed fingers: Your fingers or toes bulge at the tips, a sign of hypoxemia. […] If you have hepatopulmonary syndrome, you probably already have chronic liver disease. End-stage liver disease itself is eventually fatal without a liver transplant. Hepatopulmonary syndrome is just one of many side effects and complications that can come with it. Most people with hepatopulmonary syndrome dont die from hypoxemia alone, but it does contribute to their overall decline. […] For those with cirrhosis and hepatopulmonary syndrome, the average is two years without a liver transplant. […] Like liver disease, hepatopulmonary syndrome is progressive over time.

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

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

  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. […] These changes in the lungs make it hard for red blood cells to take in oxygen. Then the lungs can’t send enough oxygen to the body. This leads to low oxygen levels in the blood, also called hypoxemia. […] Often, there are no symptoms of hepatopulmonary syndrome. If there are symptoms, they can include: Shortness of breath that’s worse when sitting or standing and gets better when lying down. Clubbing of the fingers, in which the fingertips spread out and become rounder than usual. Broken blood vessels under the skin, called spider angioma. Bluish tinge of the lips and skin in white people. In Black and brown people, the lips or tongue might look light gray. This change in color is called cyanosis.

• #17 Hepatopulmonary syndrome – Hancock Health

  https://www.hancockhealth.org/de/mayo-health-library/hepatopulmonary-syndrome/

  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. […] These changes in the lungs make it hard for red blood cells to take in oxygen. Then the lungs cant send enough oxygen to the body. This leads to low oxygen levels in the blood, also called hypoxemia. […] Often, there are no symptoms of hepatopulmonary syndrome. If there are symptoms, they can include: Shortness of breath thats worse when sitting or standing and gets better when lying down. Clubbing of the fingers, in which the fingertips spread out and become rounder than usual. Broken blood vessels under the skin, called spider angioma. Bluish tinge of the lips and skin in white people. In Black and brown people, the lips or tongue might look light gray. This change in color is called cyanosis.

• #18 Hepatopulmonary Syndrome: Symptoms, Causes & Treatment

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

  Platypnoea: Shortness of breath that gets worse when you sit up and better when you lie down. […] Orthopnea: Shortness of breath that gets worse when you lie down and better when you sit up. […] Cyanosis: A blueish tint to your lips, nail beds or skin from the lack of oxygen. […] Clubbed fingers: Your fingers or toes bulge at the tips, a sign of hypoxemia. […] If you have hepatopulmonary syndrome, you probably already have chronic liver disease. End-stage liver disease itself is eventually fatal without a liver transplant. Hepatopulmonary syndrome is just one of many side effects and complications that can come with it. Most people with hepatopulmonary syndrome dont die from hypoxemia alone, but it does contribute to their overall decline. […] For those with cirrhosis and hepatopulmonary syndrome, the average is two years without a liver transplant. […] Like liver disease, hepatopulmonary syndrome is progressive over time.

• #19 What Is Hepatopulmonary Syndrome?

  https://www.icliniq.com/articles/respiratory-health/hepatopulmonary-syndrome

  Hepatopulmonary syndrome (HPS) is the common cause of respiratory insufficiency in patients with chronic liver disease. When the liver is not functioning properly, the blood vessels in the lungs may get dilated. The severity of this condition results in the failure of the lungs to effectively transfer oxygen to the body. This is referred to as hepatopulmonary syndrome. It is commonly seen in patients with scarring of the liver. […] Many people do not present with symptoms. If symptoms occur, these include: Shortness of breath, particularly when sitting or standing. Spider angioma (broken or dilated blood vessels under the skin.) Cyanosis (bluish discoloration of the lips and skin.) Clubbing of the fingers or toes. The most common symptom is platypnea (breathlessness in an upright position which is improved with lying down (supine) position.) Orthodeoxia (decrease in the partial pressure of oxygen when moving from supine to upright position. The severity increases with the severity of hepatopulmonary syndrome.)

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

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

  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. […] These changes in the lungs make it hard for red blood cells to take in oxygen. Then the lungs can’t send enough oxygen to the body. This leads to low oxygen levels in the blood, also called hypoxemia. […] Often, there are no symptoms of hepatopulmonary syndrome. If there are symptoms, they can include: Shortness of breath that’s worse when sitting or standing and gets better when lying down. Clubbing of the fingers, in which the fingertips spread out and become rounder than usual. Broken blood vessels under the skin, called spider angioma. Bluish tinge of the lips and skin in white people. In Black and brown people, the lips or tongue might look light gray. This change in color is called cyanosis.

• #21 What Is Hepatopulmonary Syndrome?

  https://www.icliniq.com/articles/respiratory-health/hepatopulmonary-syndrome

  Hepatopulmonary syndrome (HPS) is the common cause of respiratory insufficiency in patients with chronic liver disease. When the liver is not functioning properly, the blood vessels in the lungs may get dilated. The severity of this condition results in the failure of the lungs to effectively transfer oxygen to the body. This is referred to as hepatopulmonary syndrome. It is commonly seen in patients with scarring of the liver. […] Many people do not present with symptoms. If symptoms occur, these include: Shortness of breath, particularly when sitting or standing. Spider angioma (broken or dilated blood vessels under the skin.) Cyanosis (bluish discoloration of the lips and skin.) Clubbing of the fingers or toes. The most common symptom is platypnea (breathlessness in an upright position which is improved with lying down (supine) position.) Orthodeoxia (decrease in the partial pressure of oxygen when moving from supine to upright position. The severity increases with the severity of hepatopulmonary syndrome.)

• #22 Hepatopulmonary Syndrome – StatPearls – NCBI Bookshelf

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

  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. […] HPS is defined as reduced arterial oxygen saturation due to dilated pulmonary vasculature in the presence of advanced liver disease or portal hypertension. […] The patient usually presents with dyspnea in the setting of liver disease. The onset is insidious, and dyspnea worsens with exertion. In the early stages, most patients are asymptomatic. […] Patients with HPS have 2 times higher mortality compared with patients with cirrhosis without HPS. The condition is associated with a poor quality of life and inferior functional status. The average life span in a patient with HPS (10.5 months) is significantly reduced compared with patients with chronic liver disease patients without HPS (40.8 months). The mortality risk increases with the severity of the disease, with a worse prognosis in patients with very severe disease. […] HPS is a fatal disease that drastically reduces the life span of a patient with liver disease. Most patients will have progressive vasodilation and worsening hypoxemia. Without liver transplantation, death is inevitable, as no other medical therapies are currently available.

• #23 Gastroenterology Education and CPD for trainees and specialists » Hepatopulmonary syndrome

  https://www.gastrotraining.com/category/hepatology/chronic-liver-disease-complications/hepatopulmonary-syndrome

  Hepatopulmonary syndrome is typically defined as a widened alveolar-arterial oxygen gradient (A-a pO2) 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. The hallmark of HPS is intrapulmonary vasodilatation of the precapillary and capillary vessels, impairing arterial oxygenation. A few pleural and pulmonary arteriovenous communications (shunts) and portopulmonary venous anastomosis are common findings as well. […] Clinical manifestations include insidious onset of exertional dyspnoea, platypnea (shortness of breath exacerbated by sitting up and improved by lying supine), and orthodeoxia (hypoxemia exacerbated by going from supine to an upright position). Pulmonary AVMs are predominantly situated at the lung bases. Hence, dependent blood pooling occurs on sitting/standing with subsequent increased AV shunting. Clubbing, cyanosis and spider naevi in the presence of liver disease/portal hypertension should raise the suspicion of HPS.

• #24 Hepatopulmonary Syndrome – MD Searchlight

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

  Hepatopulmonary syndrome (HPS) can happen alongside other heart and lung diseases, which can worsen abnormal blood flow in the lungs. When physically examining a patient, doctors may observe: Bluish discoloration of the skin and mucous membranes (Cyanosis) […] Patients with liver disease often experience breathing difficulties known as dyspnea. The onset is gradual and the breathing difficulty worsens with physical effort. In the early stages, most patients might not show symptoms. Its also possible for patients to show signs of chronic liver disease. […] The seriousness of a persons HPS depends on their oxygen levels. For example, a mild case could have oxygen levels 80 mm Hg with a A-aO2 of at least 15 mm Hg while breathing room air. This would continue to escalate until its classed as very severe, with oxygen levels below 50 mm Hg with an A-aO2 of at least 15 mm Hg while breathing room air, or with oxygen levels less than 300 mm Hg when breathing 100% oxygen.

• #25 Hepatopulmonary Syndrome: Symptoms, Causes & Treatment

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

  Platypnoea: Shortness of breath that gets worse when you sit up and better when you lie down. […] Orthopnea: Shortness of breath that gets worse when you lie down and better when you sit up. […] Cyanosis: A blueish tint to your lips, nail beds or skin from the lack of oxygen. […] Clubbed fingers: Your fingers or toes bulge at the tips, a sign of hypoxemia. […] If you have hepatopulmonary syndrome, you probably already have chronic liver disease. End-stage liver disease itself is eventually fatal without a liver transplant. Hepatopulmonary syndrome is just one of many side effects and complications that can come with it. Most people with hepatopulmonary syndrome dont die from hypoxemia alone, but it does contribute to their overall decline. […] For those with cirrhosis and hepatopulmonary syndrome, the average is two years without a liver transplant. […] Like liver disease, hepatopulmonary syndrome is progressive over time.

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

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

  Hepatopulmonary syndrome is a complication of liver disease that occurs when blood vessels in your lungs expand. This expansion of the blood vessels can interfere with your lungs ability to transfer oxygen to your red blood cells. The most prominent symptom is shortness of breath. […] The hallmark symptom of hepatopulmonary syndrome is severe shortness of breath, which is reported in about 95% of people. About 82% of people have symptoms of liver disease before developing lung symptoms. […] Some people experience platypnea, which is shortness of breath thats worse when standing or sitting upright and improves when lying down. It affects as many as 88% of people with hepatopulmonary syndrome. […] Hepatopulmonary syndrome usually develops after years of liver disease. It can develop in people with mildly impaired or severely impaired liver function.

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

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

  Hepatopulmonary syndrome is a complication of liver disease that occurs when blood vessels in your lungs expand. This expansion of the blood vessels can interfere with your lungs ability to transfer oxygen to your red blood cells. The most prominent symptom is shortness of breath. […] The hallmark symptom of hepatopulmonary syndrome is severe shortness of breath, which is reported in about 95% of people. About 82% of people have symptoms of liver disease before developing lung symptoms. […] Some people experience platypnea, which is shortness of breath thats worse when standing or sitting upright and improves when lying down. It affects as many as 88% of people with hepatopulmonary syndrome. […] Hepatopulmonary syndrome usually develops after years of liver disease. It can develop in people with mildly impaired or severely impaired liver function.

• #28

  https://www.tropicalgastro.com/articles/32/1/hepatopulmonary-syndrome-prevalence.html

  In summary, 34% of our patients hospitalized due to complications of CLD had HPS. Although these results were based on subset analysis and with the limitation of smaller sample size, these results suggest that HPS in Egypt is not less common, but is frequently under diagnosed, due to the fact that most of the affected patients are either asymptomatic or present vague complaints of dyspnoea and fatigue.

• #29 Hepatopulmonary Syndrome – StatPearls – NCBI Bookshelf

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

  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. […] HPS is defined as reduced arterial oxygen saturation due to dilated pulmonary vasculature in the presence of advanced liver disease or portal hypertension. […] The patient usually presents with dyspnea in the setting of liver disease. The onset is insidious, and dyspnea worsens with exertion. In the early stages, most patients are asymptomatic. […] Patients with HPS have 2 times higher mortality compared with patients with cirrhosis without HPS. The condition is associated with a poor quality of life and inferior functional status. The average life span in a patient with HPS (10.5 months) is significantly reduced compared with patients with chronic liver disease patients without HPS (40.8 months). The mortality risk increases with the severity of the disease, with a worse prognosis in patients with very severe disease. […] HPS is a fatal disease that drastically reduces the life span of a patient with liver disease. Most patients will have progressive vasodilation and worsening hypoxemia. Without liver transplantation, death is inevitable, as no other medical therapies are currently available.

• #30 Hepatopulmonary syndrome: What we know and what we would like to know

  https://www.wjgnet.com/1007-9327/full/v22/i25/5728.htm

  The prevalence of the syndrome has not been fully established since figures depend on the method employed for the diagnosis and the profile of the patients studied. […] Systematic HPS screening in cirrhosis patients listed for LT shows that the majority of HPS patients are mild or moderate (77%-88%). Severe cases (12%-17%) and very severe cases (4%-6.3%) are less common. […] If the disease progresses, hypoxemia becomes more severe and other abnormalities are detected in respiratory function tests, such as a decreased DLCOco or a positive 99mTc-MAA. […] This is the symptomatic period of HPS, with severe to very severe cases in which survival would probably be compromised, resulting in death if the patient does not receive an LT.

• #31

  https://journals.lww.com/hep/fulltext/2024/03000/physiologic_changes_in_the_hepatopulmonary.13.aspx

  Hepatopulmonary syndrome (HPS) is a common complication of liver disease defined by abnormal oxygenation and intrapulmonary vascular dilatation, treated with liver transplantation. […] Although experts have noted that HPS is a progressive condition, little is known about how oxygenation changes in patients with HPS over time. […] Among subjects with moderate to very severe HPS, PaO2 declined, with a slope of 3.7 (6.4, 0.96) mm Hg/year; DLCO declined, with a slope of 3.3 (6.6, 0.011) percent predicted/year; and 6MWD trended towards a decline, with a slope of 26 (96, 44) meters/year. […] In the first year after liver transplant, PaO2 improved, with a slope of 6.5 (5.3, 7.7) mm Hg/month; DLCO improved, with a slope of 11 (3.9, 19) percent predicted/year; and 6MWD improved, with a slope of by 190 (130, 250) m/year.

• #32 Hepatopulmonary syndrome: What we know and what we would like to know

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4932208/

  Hepatopulmonary syndrome (HPS) is characterized by abnormalities in blood oxygenation caused by the presence of intrapulmonary vascular dilations (IPVD) in the context of liver disease, generally at a cirrhotic stage. […] Although it is generally asymptomatic it does have an impact on their quality of life and survival. […] The symptoms which have classically been associated with HPS are dyspnea and platypnea. […] In the largest study covering patients listed for LT, dyspnea was present in 48% of HPS patients, and was more frequent than in patients without HPS, with significant differences. […] Platypnea, which means a worsening in dyspnea when a patient is standing rather than lying down, is considered a pathognomonic characteristic of HPS. […] HPS patients have a lower quality of life, rank higher in the New York Heart Association functional classification and suffer significant oxygen desaturation whilst sleeping.

• #33 Hepatopulmonary syndrome: What we know and what we would like to know

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4932208/

  Hepatopulmonary syndrome (HPS) is characterized by abnormalities in blood oxygenation caused by the presence of intrapulmonary vascular dilations (IPVD) in the context of liver disease, generally at a cirrhotic stage. […] Although it is generally asymptomatic it does have an impact on their quality of life and survival. […] The symptoms which have classically been associated with HPS are dyspnea and platypnea. […] In the largest study covering patients listed for LT, dyspnea was present in 48% of HPS patients, and was more frequent than in patients without HPS, with significant differences. […] Platypnea, which means a worsening in dyspnea when a patient is standing rather than lying down, is considered a pathognomonic characteristic of HPS. […] HPS patients have a lower quality of life, rank higher in the New York Heart Association functional classification and suffer significant oxygen desaturation whilst sleeping.

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

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

  Hepatopulmonary syndrome worsens the outlook for people with the underlying liver disease. People with hepatopulmonary syndrome and cirrhosis have about twice the mortality rate as people with only cirrhosis. Hepatopulmonary syndrome is associated with poorer quality of life and poorer daily function. […] The average survival time after hepatopulmonary syndrome diagnosis is 10.5 months compared with 40.8 months for people with chronic liver disease without hepatopulmonary syndrome. Half of people who arent candidates for liver transplantation die within 2 years, most often due to complications of liver disease.

• #35 Hepatopulmonary Syndrome – StatPearls – NCBI Bookshelf

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

  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. […] HPS is defined as reduced arterial oxygen saturation due to dilated pulmonary vasculature in the presence of advanced liver disease or portal hypertension. […] The patient usually presents with dyspnea in the setting of liver disease. The onset is insidious, and dyspnea worsens with exertion. In the early stages, most patients are asymptomatic. […] Patients with HPS have 2 times higher mortality compared with patients with cirrhosis without HPS. The condition is associated with a poor quality of life and inferior functional status. The average life span in a patient with HPS (10.5 months) is significantly reduced compared with patients with chronic liver disease patients without HPS (40.8 months). The mortality risk increases with the severity of the disease, with a worse prognosis in patients with very severe disease. […] HPS is a fatal disease that drastically reduces the life span of a patient with liver disease. Most patients will have progressive vasodilation and worsening hypoxemia. Without liver transplantation, death is inevitable, as no other medical therapies are currently available.

• #36 Hepatopulmonary Syndrome and Diffuse Interstitial Lung Disease: An Unusual Combination | Archivos de Bronconeumología

  https://www.archbronconeumol.org/en-hepatopulmonary-syndrome-diffuse-interstitial-lung-articulo-S1579212918303069

  The presence of HPS can limit the diagnostic process of ILD, due to the risk involved in performing a lung biopsy in these patients. Moreover, if the patient has a previous diagnosis of idiopathic pulmonary fibrosis (as in our first case), the available antifibrotic treatments would be contraindicated due to severe liver disease. […] Risk of death in HPS patients is double that of patients with cirrhosis without HPS. For this reason, prompt inclusion in an LT program after diagnosis is recommended. […] The main causes of death in HPS are derived from the complications of the liver disease itself.

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

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

  Hepatopulmonary syndrome worsens the outlook for people with the underlying liver disease. People with hepatopulmonary syndrome and cirrhosis have about twice the mortality rate as people with only cirrhosis. Hepatopulmonary syndrome is associated with poorer quality of life and poorer daily function. […] The average survival time after hepatopulmonary syndrome diagnosis is 10.5 months compared with 40.8 months for people with chronic liver disease without hepatopulmonary syndrome. Half of people who arent candidates for liver transplantation die within 2 years, most often due to complications of liver disease.

• #38 Hepatopulmonary Syndrome – MD Searchlight

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

  On average, a patient with HPS has a much shorter life expectancy (about 10.5 months) compared to those with other chronic liver diseases but without HPS (about 40.8 months). The risk of mortality or death increases as the disease gets worse, and those with a very severe form of the disease face a worse prognosis or outlook. […] Even after a liver transplant, survival is slightly less in those patients who had very severe HPS. This means that even after receiving a new liver, the patients life expectancy is less compared to people who didnt have HPS.

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

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

  Hepatopulmonary syndrome worsens the outlook for people with the underlying liver disease. People with hepatopulmonary syndrome and cirrhosis have about twice the mortality rate as people with only cirrhosis. Hepatopulmonary syndrome is associated with poorer quality of life and poorer daily function. […] The average survival time after hepatopulmonary syndrome diagnosis is 10.5 months compared with 40.8 months for people with chronic liver disease without hepatopulmonary syndrome. Half of people who arent candidates for liver transplantation die within 2 years, most often due to complications of liver disease.

• #40 SSA – POMS: DI 23022.955 – Hepatopulmonary Syndrome – 08/25/2020

  https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022955

  Hepatopulmonary syndrome (HPS) is a severe condition involving shortness of breath and hypoxemia in people with chronic liver disease that has advanced to the point that it affects their lungs. […] The signs of pulmonary involvement may include: Digital clubbing (the appearance of changes in the areas under and around the toenails and fingernails); Cyanosis (a condition in which the lips, fingers, and toes appear blue); Dyspnea (shortness of breath); Platypnea (shortness of breath that is relieved when lying down and worsens when standing or sitting); and Orthodeoxia (fall in arterial blood oxygen while in the upright position). […] HPS worsens the prognosis of individuals with cirrhosis and other liver diseases. Individuals who are not candidates for liver transplantation have a median survival of 2 years. Mortality is usually associated with complications of hepatic disease.

• #41 Hepatopulmonary Syndrome: Symptoms, Causes & Treatment

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

  Platypnoea: Shortness of breath that gets worse when you sit up and better when you lie down. […] Orthopnea: Shortness of breath that gets worse when you lie down and better when you sit up. […] Cyanosis: A blueish tint to your lips, nail beds or skin from the lack of oxygen. […] Clubbed fingers: Your fingers or toes bulge at the tips, a sign of hypoxemia. […] If you have hepatopulmonary syndrome, you probably already have chronic liver disease. End-stage liver disease itself is eventually fatal without a liver transplant. Hepatopulmonary syndrome is just one of many side effects and complications that can come with it. Most people with hepatopulmonary syndrome dont die from hypoxemia alone, but it does contribute to their overall decline. […] For those with cirrhosis and hepatopulmonary syndrome, the average is two years without a liver transplant. […] Like liver disease, hepatopulmonary syndrome is progressive over time.

• #42 Hepatopulmonary Syndrome and Diffuse Interstitial Lung Disease: An Unusual Combination | Archivos de Bronconeumología

  https://www.archbronconeumol.org/en-hepatopulmonary-syndrome-diffuse-interstitial-lung-articulo-S1579212918303069

  Hepatopulmonary syndrome (HPS) is characterized by a triad of impaired in arterial oxygenation caused by intrapulmonary vascular dilatations (IPVD) in the setting of advanced liver disease. The most common respiratory symptom is progressive dyspnea. Platypnea-orthodeoxia (increased dyspnea and reduced oxygen saturation in an orthostatic position) can occur in up to 25% of patients. Other signs suggestive of HPS are telangiectasia, nail clubbing, and peripheral cyanosis. […] HPS can be a cause of disproportionate hypoxemia in ILD patients. In this setting, a greater reduction in carbon monoxide diffusion than expected might indicate an associated vascular problem or the presence of emphysema. As a result, this parameter may be less sensitive for evaluating ILD progress or response to specific treatment.

• #43 Hepatopulmonary Syndrome and Diffuse Interstitial Lung Disease: An Unusual Combination | Archivos de Bronconeumología

  https://www.archbronconeumol.org/en-hepatopulmonary-syndrome-diffuse-interstitial-lung-articulo-S1579212918303069

  Hepatopulmonary syndrome (HPS) is characterized by a triad of impaired in arterial oxygenation caused by intrapulmonary vascular dilatations (IPVD) in the setting of advanced liver disease. The most common respiratory symptom is progressive dyspnea. Platypnea-orthodeoxia (increased dyspnea and reduced oxygen saturation in an orthostatic position) can occur in up to 25% of patients. Other signs suggestive of HPS are telangiectasia, nail clubbing, and peripheral cyanosis. […] HPS can be a cause of disproportionate hypoxemia in ILD patients. In this setting, a greater reduction in carbon monoxide diffusion than expected might indicate an associated vascular problem or the presence of emphysema. As a result, this parameter may be less sensitive for evaluating ILD progress or response to specific treatment.

• #44 Hepatopulmonary Syndrome and Diffuse Interstitial Lung Disease: An Unusual Combination | Archivos de Bronconeumología

  https://archbronconeumol.org/en-hepatopulmonary-syndrome-diffuse-interstitial-lung-articulo-S1579212918303069

  Hepatopulmonary syndrome (HPS) is characterized by a triad of impaired in arterial oxygenation caused by intrapulmonary vascular dilatations (IPVD) in the setting of advanced liver disease. The most common respiratory symptom is progressive dyspnea. Platypnea-orthodeoxia (increased dyspnea and reduced oxygen saturation in an orthostatic position) can occur in up to 25% of patients. Other signs suggestive of HPS are telangiectasia, nail clubbing, and peripheral cyanosis. […] HPS can be a cause of disproportionate hypoxemia in ILD patients. In this setting, a greater reduction in carbon monoxide diffusion than expected might indicate an associated vascular problem or the presence of emphysema. As a result, this parameter may be less sensitive for evaluating ILD progress or response to specific treatment.

• #45 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. […] Clinical features of cyanosis, platypnea and orthodeoxia are commonly associated findings. […] In the case of our patient, overt symptoms of HPS were masked by underlying COPD and possible pneumonia. […] The diagnosis of hepatopulmonary syndrome involves a triad criteria of a) portal hypertension, b) pulmonary vascular dilation typically by contrast-enhanced echocardiography and c) a partial pressure of oxygen (PaO2). […] 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.

• #46 :: KCJ :: Korean Circulation Journal

  https://e-kcj.org/DOIx.php?id=10.4070/kcj.2015.45.1.77

  Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN) are complications of portal hypertension and cirrhosis. HPS is characterized by a defect in arterial oxygenation induced by pulmonary vascular dilatation. […] HPS was aggravated after sildenafil administration for the treatment of PPHTN. […] The patient was diagnosed as PPHTN and was started on diuretics and sildenafil 25 mg thrice daily. However dyspnea worsened despite treatment. […] The lack of decompensating factors led to the consideration of IPS as the predominant cause of worsening dyspnea, after sildenafil administration. […] Therefore, coexisting HPS was diagnosed. […] Significant clinical improvement occurred on administration of diuretics and discontinuation of sildenafil. […] In conclusion, HPS and PPHTN can occur in the same patient and sildenafil may be associated with aggravation of HPS in patients with coexisting HPS and PPHTN.

• #47 Hepatopulmonary syndrome: What we know and what we would like to know

  https://www.wjgnet.com/1007-9327/full/v22/i25/5728.htm

  The prevalence of the syndrome has not been fully established since figures depend on the method employed for the diagnosis and the profile of the patients studied. […] Systematic HPS screening in cirrhosis patients listed for LT shows that the majority of HPS patients are mild or moderate (77%-88%). Severe cases (12%-17%) and very severe cases (4%-6.3%) are less common. […] If the disease progresses, hypoxemia becomes more severe and other abnormalities are detected in respiratory function tests, such as a decreased DLCOco or a positive 99mTc-MAA. […] This is the symptomatic period of HPS, with severe to very severe cases in which survival would probably be compromised, resulting in death if the patient does not receive an LT.

• #48 Hepatopulmonary syndrome as a cause of hypoxemia in hepatic diseases in children | Boletín Médico del Hospital Infantil de México (English Edition)

  https://www.elsevier.es/en-revista-boletin-medico-del-hospital-infantil-201-articulo-hepatopulmonary-syndrome-as-cause-hypoxemia-X2444340915346095

  Dyspnea has been described as a cardinal symptom for the suspicion of hepatopulmonary disease. Its onset is generally insidious and with exacerbation on exercise. […] Digital clubbing, marked as a physical sign of hypoxemia, was notable in this patient because, according to the history, she had a 7-month evolution. However, its presence has been reported as an indicator of advanced disease. […] The severity was determined due to the PaO2 value of 59 mmHg and it was classified as type II according to the positive hyperoxia test. The patient remained on home oxygen as supportive therapy while the liver transplant was planned, which was recommended to be done promptly to avoid disease progression and a fatal outcome. […] Poor prognostic factors are the PaO2 20% on the scan with albumin macro-aggregates, severe pulmonary hypertension and positive hyperoxia test. However, one should take into account that the prognosis without a transplant is ominous, with a survival rate of 16-30% at 1 year from the time of diagnosis.

• #49 Hepatopulmonary syndrome: What we know and what we would like to know

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4932208/

  The available data on the symptoms and exploration findings in HPS patients is mainly based on those with cirrhosis and therefore should not be extrapolated to all HPS patients. […] The natural history of HPS is unknown. Existing studies have been conducted in cirrhotic patients for whom the presence of HPS worsens their survival rate, independently of their age, sex, race, Child-Pugh score, blood urea levels and MELD score. […] If the disease progresses, hypoxemia becomes more severe and other abnormalities are detected in respiratory function tests, such as a decreased DLCOco or a positive 99mTc-MAA. […] The most widely studied treatment is LT. At the moment it is the only effective treatment for HPS and is proven to improve survival rates.

• #50 Hepatopulmonary Syndrome – MD Searchlight

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

  Hepatopulmonary syndrome (HPS) can happen alongside other heart and lung diseases, which can worsen abnormal blood flow in the lungs. When physically examining a patient, doctors may observe: Bluish discoloration of the skin and mucous membranes (Cyanosis) […] Patients with liver disease often experience breathing difficulties known as dyspnea. The onset is gradual and the breathing difficulty worsens with physical effort. In the early stages, most patients might not show symptoms. Its also possible for patients to show signs of chronic liver disease. […] The seriousness of a persons HPS depends on their oxygen levels. For example, a mild case could have oxygen levels 80 mm Hg with a A-aO2 of at least 15 mm Hg while breathing room air. This would continue to escalate until its classed as very severe, with oxygen levels below 50 mm Hg with an A-aO2 of at least 15 mm Hg while breathing room air, or with oxygen levels less than 300 mm Hg when breathing 100% oxygen.

• #51 Hepatopulmonary syndrome: What we know and what we would like to know

  https://www.wjgnet.com/1007-9327/full/v22/i25/5728.htm

  The prevalence of the syndrome has not been fully established since figures depend on the method employed for the diagnosis and the profile of the patients studied. […] Systematic HPS screening in cirrhosis patients listed for LT shows that the majority of HPS patients are mild or moderate (77%-88%). Severe cases (12%-17%) and very severe cases (4%-6.3%) are less common. […] If the disease progresses, hypoxemia becomes more severe and other abnormalities are detected in respiratory function tests, such as a decreased DLCOco or a positive 99mTc-MAA. […] This is the symptomatic period of HPS, with severe to very severe cases in which survival would probably be compromised, resulting in death if the patient does not receive an LT.

• #52 Internet Scientific Publications

  https://ispub.com/IJRA/6/2/4562

  Two patterns of vascular abnormality are seen on pulmonary angiography in this syndrome. The lesions of HPS have been classified into two types. The type 1 (minimal) pattern is most common (85%), it is associated with spidery appearance of peripheral vessels and usually a good response to treatment with 100% oxygen. Type 2 lesions (15%) represent small, discrete pulmonary arteriovenous fistulae, type 2 lesions are associated with a poor response to 100% oxygen.

• #53

  https://journals.lww.com/hep/fulltext/2024/03000/physiologic_changes_in_the_hepatopulmonary.13.aspx

  The median time to normalization was 149 (116, 184) days. […] The only significant predictor of the posttransplant rate of improvement in PaO2 was the magnitude of pretransplant orthodeoxia, with a faster rate of improvement in posttransplant PaO2 by 2.5 (0.1, 4.9) mm Hg/month for every 10 mm Hg increase in pretransplant orthodeoxia.

• #54 Hepatopulmonary syndrome: What we know and what we would like to know

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4932208/

  The available data on the symptoms and exploration findings in HPS patients is mainly based on those with cirrhosis and therefore should not be extrapolated to all HPS patients. […] The natural history of HPS is unknown. Existing studies have been conducted in cirrhotic patients for whom the presence of HPS worsens their survival rate, independently of their age, sex, race, Child-Pugh score, blood urea levels and MELD score. […] If the disease progresses, hypoxemia becomes more severe and other abnormalities are detected in respiratory function tests, such as a decreased DLCOco or a positive 99mTc-MAA. […] The most widely studied treatment is LT. At the moment it is the only effective treatment for HPS and is proven to improve survival rates.

• #55 Hepatopulmonary Syndrome – StatPearls – NCBI Bookshelf

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

  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. […] HPS is defined as reduced arterial oxygen saturation due to dilated pulmonary vasculature in the presence of advanced liver disease or portal hypertension. […] The patient usually presents with dyspnea in the setting of liver disease. The onset is insidious, and dyspnea worsens with exertion. In the early stages, most patients are asymptomatic. […] Patients with HPS have 2 times higher mortality compared with patients with cirrhosis without HPS. The condition is associated with a poor quality of life and inferior functional status. The average life span in a patient with HPS (10.5 months) is significantly reduced compared with patients with chronic liver disease patients without HPS (40.8 months). The mortality risk increases with the severity of the disease, with a worse prognosis in patients with very severe disease. […] HPS is a fatal disease that drastically reduces the life span of a patient with liver disease. Most patients will have progressive vasodilation and worsening hypoxemia. Without liver transplantation, death is inevitable, as no other medical therapies are currently available.

• #56 Hepatopulmonary syndrome – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/hepatopulmonary-syndrome/

  Hepatopulmonary (hep-uh-toe-POOL-moe-nar-e) syndrome is an uncommon condition that affects the lungs of people with advanced liver disease. Hepatopulmonary syndrome is caused by blood vessels in the lungs expanding (dilating) and increasing in number, making it hard for red blood cells to properly absorb oxygen. This leaves the lungs unable to deliver adequate amounts of oxygen to the body, which leads to low oxygen levels (hypoxemia). […] Most people with hepatopulmonary syndrome have no symptoms. If symptoms occur, they may include: Shortness of breath, especially when sitting or standing, Clubbing of the fingers, in which the fingertips spread out and become rounder than normal, Broken blood vessels under the skin (spider angioma), Bluish tinge of the lips and skin (cyanosis). […] 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.

• #57 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. […] Hepatopulmonary syndrome can cause platypnea (dyspnea when sitting or standing, which improves on lying down) and orthodeoxia (hypoxemia when upright, which improves on lying down), which occur when the patient is seated or standing and are relieved by recumbency. […] 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. […] Patients with hepatopulmonary syndrome tend to have findings of chronic liver disease and may have platypnea. […] If the diagnosis is suspected, do pulse oximetry and consider arterial blood gas measurement and imaging (eg, contrast echocardiography). […] Treat with supplemental oxygen.

• #58

  https://link.springer.com/article/10.1007/s00535-006-1965-0

  Hepatopulmonary syndrome (HPS) is a complication of liver disease that is characterized by hypoxemia and intrapulmonary vascular dilatations. […] We gave the patients low-dose oxygen supplementation to improve their respiratory symptoms. Surprisingly, their liver function improved from Child Pugh class C to class A, and ascites disappeared after a year of oxygen supplementation. […] Long-term oxygen therapy might offer a new therapeutic approach to improve liver function in patients with cirrhosis with hypoxemia.

• #59 What is Hepatopulmonary Syndrome – HPS

  https://hpscare.com/for-patients/

  Hepatopulmonary Syndrome can cause low oxygen levels in your blood. Having low oxygen levels in your blood means people with HPS experience shortness of breath, which can become more and more severe over time. When oxygen is not travelling through your body properly, you may notice that your fingertips turn blue or that your fingernails change shape over time (called clubbing). You may also experience more breathlessness in the sitting or standing position, and feel better when lying flat (this is called platypnea). […] The prognosis in hepatopulmonary syndrome is mainly determined by whether or not a liver transplant can be performed. The majority of patients with hepatopulmonary syndrome have gradually worsening shortness of breath and oxygen levels over time, until they get a liver transplant. As time progresses, many patients may require oxygen therapy. When compared to patients who have a similar extent of liver disease but do not have hepatopulmonary syndrome, patients with hepatopulmonary syndrome also tend to have more complications of their liver disease and have a higher chance of dying from these complications. This is why liver transplant is so important. However, there are also some patients who stabilize and can survive for many years with chronically low oxygen levels due to hepatopulmonary syndrome. So far, there is not much research in this population of patients, and more information is required. […] Currently, the only known effective therapy for HPS is a liver transplant. When the damaged liver is replaced, the majority of HPS patients will gradually experience either significant improvement or complete resolution of blood oxygen levels and no longer feel short of breath.

• #60 Hepatopulmonary syndrome | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/hepatopulmonary-syndrome

  Often, there are no symptoms of hepatopulmonary syndrome. If there are symptoms, they can include: […] Shortness of breath that’s worse when sitting or standing and gets better when lying down. […] A liver transplant is the only cure for hepatopulmonary syndrome.

• #61

  https://journals.lww.com/hep/fulltext/2024/03000/physiologic_changes_in_the_hepatopulmonary.13.aspx

  Hepatopulmonary syndrome (HPS) is a common complication of liver disease defined by abnormal oxygenation and intrapulmonary vascular dilatation, treated with liver transplantation. […] Although experts have noted that HPS is a progressive condition, little is known about how oxygenation changes in patients with HPS over time. […] Among subjects with moderate to very severe HPS, PaO2 declined, with a slope of 3.7 (6.4, 0.96) mm Hg/year; DLCO declined, with a slope of 3.3 (6.6, 0.011) percent predicted/year; and 6MWD trended towards a decline, with a slope of 26 (96, 44) meters/year. […] In the first year after liver transplant, PaO2 improved, with a slope of 6.5 (5.3, 7.7) mm Hg/month; DLCO improved, with a slope of 11 (3.9, 19) percent predicted/year; and 6MWD improved, with a slope of by 190 (130, 250) m/year.

• #62

  https://journals.lww.com/hep/fulltext/2024/03000/physiologic_changes_in_the_hepatopulmonary.13.aspx

  The median time to normalization was 149 (116, 184) days. […] The only significant predictor of the posttransplant rate of improvement in PaO2 was the magnitude of pretransplant orthodeoxia, with a faster rate of improvement in posttransplant PaO2 by 2.5 (0.1, 4.9) mm Hg/month for every 10 mm Hg increase in pretransplant orthodeoxia.

• #63 Hepatopulmonary syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Hepatopulmonary_syndrome

  In medicine, hepatopulmonary syndrome is a syndrome of shortness of breath and hypoxemia (low oxygen levels in the blood of the arteries) caused by vasodilation (broadening of the blood vessels) in the lungs of patients with liver disease. Dyspnea and hypoxemia are worse in the upright position (which is called platypnea and orthodeoxia, respectively). […] The hepatopulmonary syndrome is suspected in any patient with known liver disease who reports dyspnea (particularly platypnea). Patients with clinically significant symptoms should undergo pulse oximetry. If the syndrome is advanced, arterial blood gasses should be measured on air. […] Hepatopulmonary syndrome (HPS) consists of the triad of liver dysfunction, otherwise unexplained hypoxemia, and intrapulmonary vascular dilation (IPVD). […] With liver transplantation, the 5 year survival rate is 74%, which is comparable to patients who undergo liver transplants who do not suffer from hepatopulmonary syndrome.

• #64 Hepatopulmonary Syndrome – MD Searchlight

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

  On average, a patient with HPS has a much shorter life expectancy (about 10.5 months) compared to those with other chronic liver diseases but without HPS (about 40.8 months). The risk of mortality or death increases as the disease gets worse, and those with a very severe form of the disease face a worse prognosis or outlook. […] Even after a liver transplant, survival is slightly less in those patients who had very severe HPS. This means that even after receiving a new liver, the patients life expectancy is less compared to people who didnt have HPS.

• #65 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

  At present, there is no effective medical therapy considered useful in the 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.

• #66 Ancient Remedies Revisited: Does Allium Sativum (Garlic) Palliate the Hepatopulmonary Syndrome? – University of Miami

  https://scholarship.miami.edu/esploro/outputs/journalArticle/Ancient-Remedies-Revisited-Does-Allium-Sativum/991031560069802976

  Hypoxia in the setting of liver disease is often multifactorial. […] Symptoms may be ameliorated by somatostatin and reversed by successful live transplantation. […] She has experienced partial palliation of her symptoms and some objective signs of improvement over 18 months of continuous self-medication.

• #67 Hepatopulmonary Syndrome: Oxidative Stress and Physical Exercise – European Medical Journal

  https://www.emjreviews.com/hepatology/article/hepatopulmonary-syndrome-oxidative-stress-and-physical-exercise/

  Physiological alterations present in HPS patients are similar to the alterations present in experimental models of BDL. The clinical features of intrapulmonary vasodilation and gas exchange abnormalities are better understood when investigated in combination with the antioxidant system, inflammatory processes associated with bacterial translocation, and the presence of portosystemic and intrapulmonary shunts. HPS, and other diseases that affect the hepatic system, may contribute to the development of sarcopenia and cachexia because of hyperammonaemia, malnutrition, hypermetabolism, and proteolysis. Physical exercise is a potential therapeutic approach to revert existing impairments in the musculoskeletal and pulmonary systems and is an option for the treatment of HPS patients and other individuals with hepatic injury.

• #68 Hepatopulmonary Syndrome Diagnosis Guide – Acibadem Health Point – ACIBADEM Hospitals – Acibadem Health Group

  https://www.acibademhealthpoint.com/hepatopulmonary-syndrome-diagnosis-guide/

  People might also notice their lips and fingertips turning blue. This comes from not enough oxygen in their blood. If someone’s fingers start to look swollen and their nails curve down, that’s a sign too. […] The importance of finding liver disease early is big. Catching hepatopulmonary syndrome (HPS) at the start is key. It makes outcomes better and improves how we deal with liver disease. Early action can slow down the disease and help with health plans. […] Spotting HPS early helps start treatments quickly. This can make the patient’s condition better. It also may slow down the damage to the liver and lungs. […] The main sign of HPS is not enough oxygen in the blood. This happens because the liver is not working like it should. So, patients have trouble getting oxygen into their blood.

• #69 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. […] Clinical features of cyanosis, platypnea and orthodeoxia are commonly associated findings. […] In the case of our patient, overt symptoms of HPS were masked by underlying COPD and possible pneumonia. […] The diagnosis of hepatopulmonary syndrome involves a triad criteria of a) portal hypertension, b) pulmonary vascular dilation typically by contrast-enhanced echocardiography and c) a partial pressure of oxygen (PaO2). […] 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.

• #70 Hepatopulmonary syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Hepatopulmonary_syndrome

  In medicine, hepatopulmonary syndrome is a syndrome of shortness of breath and hypoxemia (low oxygen levels in the blood of the arteries) caused by vasodilation (broadening of the blood vessels) in the lungs of patients with liver disease. Dyspnea and hypoxemia are worse in the upright position (which is called platypnea and orthodeoxia, respectively). […] The hepatopulmonary syndrome is suspected in any patient with known liver disease who reports dyspnea (particularly platypnea). Patients with clinically significant symptoms should undergo pulse oximetry. If the syndrome is advanced, arterial blood gasses should be measured on air. […] Hepatopulmonary syndrome (HPS) consists of the triad of liver dysfunction, otherwise unexplained hypoxemia, and intrapulmonary vascular dilation (IPVD). […] With liver transplantation, the 5 year survival rate is 74%, which is comparable to patients who undergo liver transplants who do not suffer from hepatopulmonary syndrome.

• #71 Hepatopulmonary Syndrome Diagnosis Guide – Acibadem Health Point – ACIBADEM Hospitals – Acibadem Health Group

  https://www.acibademhealthpoint.com/hepatopulmonary-syndrome-diagnosis-guide/

  Arterial blood gas (ABG) testing is key in diagnosing hepatopulmonary syndrome. It gives key info on oxygen levels in the blood. Healthcare workers use this info to check for the syndrome and its seriousness. […] Knowing these signs well helps doctors and nurses do their job better. Fast reaction to these clues is key. It can change how well the patient does over time.

• #72

  https://journals.lww.com/lt/fulltext/2018/09000/hepatopulmonary_syndrome_in_children__a_20_year.14.aspx

  Hepatopulmonary syndrome (HPS) in stable patients with cirrhosis can easily be overlooked. We report on the presenting symptoms, disease progression, and outcomes after liver transplantation (LT) in children with HPS. HPS presentations were as follows; dyspnea (n = 17) and pneumonia (n = 3). A marked rise in hemoglobin (Hb; median, 125-143.5 g/L) and modest decrease in oxygen saturation (SpO2; median 91% to 88% room air) were evident over this time. In conclusion, HPS is a life-threatening complication of cirrhosis which usually develops insidiously. This combined with the often-stable nature of the liver disease leads to delays in diagnosis and listing for LT. Progressive polycythemia extends the need for supplementary O2 and PICU stay. We advocate screening for HPS with a combination of SpO2 and Hb monitoring to facilitate earlier recognition, timely LT, and shortened recovery periods.

• #73

  https://journals.lww.com/lt/fulltext/2018/09000/hepatopulmonary_syndrome_in_children__a_20_year.14.aspx

  Hepatopulmonary syndrome (HPS) in stable patients with cirrhosis can easily be overlooked. We report on the presenting symptoms, disease progression, and outcomes after liver transplantation (LT) in children with HPS. HPS presentations were as follows; dyspnea (n = 17) and pneumonia (n = 3). A marked rise in hemoglobin (Hb; median, 125-143.5 g/L) and modest decrease in oxygen saturation (SpO2; median 91% to 88% room air) were evident over this time. In conclusion, HPS is a life-threatening complication of cirrhosis which usually develops insidiously. This combined with the often-stable nature of the liver disease leads to delays in diagnosis and listing for LT. Progressive polycythemia extends the need for supplementary O2 and PICU stay. We advocate screening for HPS with a combination of SpO2 and Hb monitoring to facilitate earlier recognition, timely LT, and shortened recovery periods.

• #74

  https://journals.lww.com/lt/fulltext/2018/09000/hepatopulmonary_syndrome_in_children__a_20_year.14.aspx

  Hepatopulmonary syndrome (HPS) in stable patients with cirrhosis can easily be overlooked. We report on the presenting symptoms, disease progression, and outcomes after liver transplantation (LT) in children with HPS. HPS presentations were as follows; dyspnea (n = 17) and pneumonia (n = 3). A marked rise in hemoglobin (Hb; median, 125-143.5 g/L) and modest decrease in oxygen saturation (SpO2; median 91% to 88% room air) were evident over this time. In conclusion, HPS is a life-threatening complication of cirrhosis which usually develops insidiously. This combined with the often-stable nature of the liver disease leads to delays in diagnosis and listing for LT. Progressive polycythemia extends the need for supplementary O2 and PICU stay. We advocate screening for HPS with a combination of SpO2 and Hb monitoring to facilitate earlier recognition, timely LT, and shortened recovery periods.

• #75

  https://journals.lww.com/hep/fulltext/2024/03000/physiologic_changes_in_the_hepatopulmonary.13.aspx

  Hepatopulmonary syndrome (HPS) is a common complication of liver disease defined by abnormal oxygenation and intrapulmonary vascular dilatation, treated with liver transplantation. […] Although experts have noted that HPS is a progressive condition, little is known about how oxygenation changes in patients with HPS over time. […] Among subjects with moderate to very severe HPS, PaO2 declined, with a slope of 3.7 (6.4, 0.96) mm Hg/year; DLCO declined, with a slope of 3.3 (6.6, 0.011) percent predicted/year; and 6MWD trended towards a decline, with a slope of 26 (96, 44) meters/year. […] In the first year after liver transplant, PaO2 improved, with a slope of 6.5 (5.3, 7.7) mm Hg/month; DLCO improved, with a slope of 11 (3.9, 19) percent predicted/year; and 6MWD improved, with a slope of by 190 (130, 250) m/year.

• #76 Hepatopulmonary Syndrome – OpenAnesthesia

  https://www.openanesthesia.org/keywords/hepatopulmonary-syndrome/

  Hepatopulmonary syndrome (HPS) is characterized by intrapulmonary vascular dilatations in the setting of portal hypertension, leading to right-to-left shunts and hypoxia. […] Patients with HPS typically present with hypoxia. […] The most common presenting symptom in patients with HPS is worsening dyspnea and hypoxia. Orthodeoxia (oxygen desaturation with movement from supine to upright position) and platypnea (dyspnea with movement from supine to upright position) may also be present. […] Patients with HPS typically have a progressive decline in PaO2 over time. This has led to additional Model for End-stage Liver Disease points (MELD: a scoring system utilized for organ allocation) given for HPS to expedite access to transplantation prior to reaching this PaO2 cutoff.

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