Evaluation and Management of Pulmonary Hypertension in Noncardiac Surgery: A Scientific Statement From the American Heart Association | Circulation
Portal Hypertension Symptoms & Their Most Common Complications
Portal Hypertension Symptoms and Their ComplicationsPortal hypertension is a condition in which increased pressure within the portal vein—an important vessel that carries blood from the stomach and intestines to the liver—rises beyond normal levels. This rise in pressure often occurs due to advanced liver disease, particularly cirrhosis. Cirrhosis causes the liver to develop scars that replace healthy liver tissue, which makes it difficult for blood to flow freely through the organ. When that primary route of blood flow becomes blocked or narrowed, pressure in the portal vein begins to climb, forcing blood to divert into smaller blood vessels. Over time, these smaller vessels can swell under the added load, eventually leading to various complications [1].
Table of Contents
Causes of Portal HypertensionPortal hypertension can be caused by various factors, including liver disease, blood clots in the portal vein, blockages of the veins that carry blood from the liver to the heart, schistosomiasis, and focal nodular hyperplasia. The most common cause of portal hypertension is cirrhosis of the liver, which is scarring that accompanies the healing of liver injury caused by hepatitis, alcohol, or other less common causes of liver damage.
How the Portal Vein Works
Esophageal varices and and illustration of a portal vein.
(Alila Medical Media)
The portal vein plays a central role in transporting nutrient-rich blood from the digestive organs straight to the liver. There, the liver filters out toxins and processes nutrients. Increased portal blood flow, driven by vasodilators and shear stress, contributes to the buildup of pressure in the portal vein. When it is scarred by cirrhosis, it is no longer an open highway for blood flow. This buildup of pressure is often first noticed by signs such as fluid accumulation, swelling of veins in the digestive tract, or changes in organ function.
Risk FactorsRisk factors for developing portal hypertension include chronic liver disease, family history of liver disease, viral hepatitis, and alcohol abuse. Regular monitoring and follow-up with a healthcare provider can help detect portal hypertension early and improve outcomes.
Clinical Signs and SymptomsThe main symptoms and complications of portal hypertension include ascites (fluid buildup in the abdomen), dilated veins or varices, and bleeding from varices. Doctors make the diagnosis of portal hypertension based on the presence of ascites or dilated veins or varices as seen during a physical exam of the abdomen or the anus. Various lab tests, X-ray tests, and endoscopic exams may also be used to diagnose portal hypertension. Symptoms of portal hypertension may not always be present, but when they do occur, they can include abdominal swelling, vomiting of blood, and lab abnormalities such as elevated liver enzymes or low platelet counts.
Major Complications of Portal Hypertension
Vector medical illustration of normal liver and cirrhosis with portal hypertension.
(FuHonra)
Portal hypertension can affect many parts of the body. Bleeding varices are one of the significant complications of portal hypertension. Below are the most significant complications that arise when the portal vein remains under high pressure over extended periods.
Varices (Enlarged Veins in the Digestive Tract)What Happens? When blood cannot pass smoothly through the scarred liver, it flows into smaller veins near the esophagus and stomach, causing these veins to grow larger than normal [1]. This is somewhat similar to having a clogged pipe in a plumbing system: the backup of pressure forces fluid into adjacent, more fragile channels. These swollen veins are called varices, and complications such as bleeding varices can arise from them.
Symptoms
An episode of variceal bleeding is considered an emergency because it can lead to rapid blood loss.
Treatment and Prevention
Regular checkups, including periodic endoscopy, are often recommended to monitor for new or recurring varices.
Ascites (Fluid Buildup in the Abdomen)What Happens? Ascites refers to the accumulation of fluid in the abdominal cavity. As increased pressure in the portal venous system rises, it disturbs fluid balance in the body. The liver can no longer regulate the flow of salt and water properly, causing fluid to leak from blood vessels into the belly [1].
Symptoms
Treatment
Ascites is a strong indicator of advanced liver disease and should not be ignored. Frequent medical monitoring can help avoid complications such as infection of the ascitic fluid.
Hepatic Encephalopathy (Brain Dysfunction Due to Toxins)What Happens? A key function of the liver is to eliminate toxins such as ammonia from the blood. When it is damaged, these toxins linger, eventually traveling to the brain and disrupting its normal activities. This condition is called hepatic encephalopathy [3].
Portal hypertension can also lead to an enlarged spleen, which decreases the count of white blood cells, increasing the risk of infections.
Symptoms
Treatment
Early detection of hepatic encephalopathy is vital because therapy can often reverse the mental changes and improve overall well-being.
Hepatopulmonary Syndrome (Lung Problems Due to Liver Disease)What Happens? In advanced liver disease, blood vessels inside the lungs may expand, affecting how oxygen enters the bloodstream. This phenomenon is known as hepatopulmonary syndrome [4]. Increased pressure in the hepatic vein contributes to the symptoms by causing blood flow obstruction and the development of collateral circulation. Individuals may notice they become short of breath more easily, especially when upright.
Symptoms
Treatment
Diagnosis often requires specific imaging tests and measurement of oxygen levels. People with advanced liver disease should promptly report any persistent breathing issues to their healthcare provider.
Portopulmonary Hypertension (High Blood Pressure in Lung Arteries)What Happens? Portopulmonary hypertension emerges when high pressure in the portal system affects the arteries in the lungs. These arteries narrow or tighten, forcing the heart to work harder to move blood through them [4]. Over time, this strain can lead to heart and lung challenges.
Symptoms
Treatment
Because this complication involves both the liver and the lungs, specialists typically coordinate care—often including a gastroenterologist, a hepatologist, and a pulmonologist.
Hepatorenal Syndrome (Kidney Failure Due to Liver Disease)What Happens? With severe liver damage, overall circulation is disrupted. As a result, not enough blood reaches the kidneys, leading them to fail. This condition is called hepatorenal syndrome (HRS) [3]. It can develop quickly and is a serious concern for those with advanced cirrhosis.
The splenic vein, which drains blood from the spleen into the portal vein, plays a significant role in contributing to elevated portal venous pressure. Increased blood flow or obstruction in the splenic vein can lead to complications such as portal hypertension.
Symptoms
Treatment
Hepatorenal syndrome usually signals an urgent state of liver disease. Monitoring kidney function through regular tests can help catch early warning signs and begin treatments sooner.
Final ThoughtsPortal hypertension is far more than just high pressure in a single vein. Its far-reaching effects can strain multiple organs and systems throughout the body. Varices in the esophagus and stomach may burst, causing life-threatening bleeding. If portal hypertension compromises blood flow to the kidneys, hepatorenal syndrome can result in kidney failure.
Portal hypertension, often complicated by inferior vena cava obstruction, can be managed through medication, procedures like endoscopic band ligation or TIPS, and in severe cases, liver transplantation. Recognizing warning signs like sudden weight gain, confusion, difficulty breathing, or unusual bleeding allows for quicker intervention and better outcomes. Routine checkups and tests are vital for early detection and effective management, enabling many patients to live fuller, healthier lives.
References[1] Simonetto, D. A., Liu, M., & Kamath, P. S. (2019). Portal Hypertension and Related Complications: Diagnosis and Management. Mayo Clinic proceedings, 94(4), 714–726. Https://doi.Org/10.1016/j.Mayocp.2018.12.020
[2] Bosch, J., & García-Pagán, J. C. (2000). Complications of cirrhosis. I. Portal hypertension. Journal of hepatology, 32(1 Suppl), 141–156. Https://doi.Org/10.1016/s0168-8278(00)80422-5
[3] Garcia-Tsao G. (2005). Portal hypertension. Current opinion in gastroenterology, 21(3), 313–322. Https://doi.Org/10.1097/01.Mog.0000158110.13722.E0
[4] Bommena, S., & Fallon, M. B. (2024). Pulmonary Complications of Portal Hypertension. Clinics in liver disease, 28(3), 467–482. Https://doi.Org/10.1016/j.Cld.2024.03.005
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Differential Diagnosis Of Pulmonary Arterial Hypertension
Panelists discuss how pulmonary arterial hypertension diagnosis requires comprehensive evaluation including right heart catheterization to confirm hemodynamic criteria, with normal mean PA pressure being ≤20 mmHg and the need to rule out other causes of pulmonary hypertension.
Diagnostic Criteria and Disease Classification
Pulmonary arterial hypertension diagnosis requires comprehensive evaluation to distinguish it from other forms of pulmonary hypertension, as PAH represents a rare subset of the broader pulmonary hypertension spectrum. The definitive diagnostic tool remains right heart catheterization, which provides accurate hemodynamic assessment essential for proper classification. Current diagnostic criteria have evolved to define normal mean pulmonary artery pressure as ≤20 mmHg, with pulmonary hypertension diagnosed when pressures exceed this threshold.
Pre-capillary pulmonary hypertension, which includes PAH, is characterized by elevated pulmonary artery pressures with normal left-sided filling pressures (≤15 mmHg) and elevated pulmonary vascular resistance (≥2 Wood units). These three hemodynamic components must be present to establish a diagnosis of pre-capillary pulmonary hypertension. However, clinical presentation often involves mixed disease patterns, requiring careful evaluation to rule out lung disease, chronic thromboembolic disease, and other secondary causes.
The diagnostic process is complicated by significant overlap between different forms of pulmonary hypertension and the reality that patients often present with multiple comorbidities. Comprehensive evaluation must exclude heart disease, lung disease, and chronic blood clots while considering the possibility of mixed-type pulmonary hypertension with both pre-capillary and post-capillary components. This complexity underscores the importance of specialist evaluation and systematic diagnostic approaches.
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Her Baby Brother Died Of A Rare Disease. Years Later, She Was Diagnosed With The Same Thing
When Heather Kauffman was a child, her brother BJ died when he was 5 of what she thought at the time was a "rare lung disease." For much of her life, she had no idea what it was and just knew that doctors didn't have anything to treat it. In 2017, Kauffman began experiencing what she thought were panic attacks. After numerous tests, she learned what was causing this sensation: She had pulmonary arterial hypertension (PAH). When the doctors told the family, her parents immediately understood what it was.
"My dad looks at me and he says, 'We know what that is. That's what our son died from, and I was mortified because I had no idea that's what BJ had died from," the 48-year-old from Charlotte, Michigan, tells TODAY.Com. "Now I have something that I've watched my brother die of."
After being diagnosed with the same rare condition that her brother died from, Heather Kauffman joined a clinical trial and underwent genetic testing to see if her sister and her children would be impacted. Luckily, she did not have the gene.Courtesy Heather Kauffman Much had changed since her brother's diagnosis, and she started taking numerous medications and even joined a clinical trial of a drug to treat PAH in August of 2021. She hoped that her participation would help her family and the lives of others with the condition.
"From the very beginning, I told (the doctors) I would do anything I could do to help (cure) this disease," she says. "My parents lost one child and watching me go through it, I have to be able to make a difference for my sister and her three kids."
Breathing problems lead to diagnosisIn 1982, Kauffman's parents learned that their son had a lung condition. At the time, she was 7 and he was 5. Doctors didn't really have much to help treat his condition.
"I remember them doing low sodium and things like that," she says. "I really didn't know a lot about the disease."
Doctors told her parents that Kauffman shouldn't be impacted.
"They were told at the time there was no genetic connection, and they didn't need to worry about their living child," Kauffman says. "If you would have asked me six years ago what my brother passed away (from) I would have said a rare lung disease. I had no idea the name of the disease."
Because doctors told the family there was no genetic link, she never underwent testing or thought it could be something that affected her. Then in 2017, Kauffman was experiencing a lot of stress. Her husband was deployed to the Middle East, and she thought she was having "panic attacks."
"I wasn't breathing very well," she says. "I didn't think it was bronchitis, but I thought it was something."
Doctors at urgent care worried it could be a blood clot and sent her to the emergency room. After an EKG, she was admitted to a small local hospital. Doctors ordered a follow up EKG and then an echocardiogram and she was transferred to a larger hospital.
"Initially it was water around my heart," Kauffman says. "They gave me Lasix so the water reduced."
For most of her life, Heather Kauffman knew her brother died of a 'rare' lung condition. When she was diagnosed with pulmonary arterial hypertension, she learned her brother died from it.Courtesy Heather Kauffman That's what allowed doctors to see what was really impacting Kauffman — pulmonary arterial hypertension. She texted her diagnosis to her parents, not knowing that PAH killed her brother. She later learned it takes years for a proper diagnosis. She began going to the University of Michigan for treatment, which included taking medications delivered by a pump. As her disease progressed, doctors thought she might need a lung transplant. But before that happened, she joined a clinical trial run by one of her doctors, Vallerie McLaughlin.
"In 2020, Dr. McLaughlin's like, 'There's this new drug trial Stellar. We think you're a great candidate,'" Kauffman recalls. "I said, 'Let's do it."
Pulmonary arterial hypertension and clinical trialPulmonary arterial hypertension is a type of pulmonary hypertension, which is high blood pressure in the lungs.
"It comes from the very small blood vessels in the lungs and that can cause the resistance in the lungs to go up," Dr. Vallerie McLaughlin, endowed professor of cardiovascular medicine at the University of Michigan Medical School, tells TODAY.Com. "That causes the right side of the heart to have to work harder to pump against this high pressure and high resistance."
She adds that the right side of the heart isn't "big, muscular" like the left side and struggles to pump as hard. In some patients it can cause heart failure. The cause can be complex.
"Pulmonary arterial hypertension can be what we call idiopathic, there's no reason for it. We can't find anything," she says. "There are some genes that can cause it."
Drugs, such as methamphetamines, contribute to people developing it and it can also occur at a "higher frequency" with some diseases, such as scleroderma, portal hypertension, liver problems and people with HIV.
It can be difficult to diagnose because the shortness of breath is normally the first sign something is wrong.
"Having trouble walking with exertion, you are starting to develop shortness of breath," she says. "Many different heart and lung disease can cause shortness of breath. Musculoskeletal disease can cause it."
Heather Kauffman's lung health improved since she started a clinical trail for pulmonary arterial hypertension.Courtesy Heather Kauffman What's more pulmonary arterial hypertension is rare, "a 25 per million disease," and providers look for more common causes of shortness of breath, such as COPD, asthma, heart failure, McLaughlin says. Proper diagnosis remains important, and it is diagnosed after a right heart catheterization that measures the pressure in the lungs. There are several drugs available to treat it and most work by "relaxing the blood vessels" to reduce pressure, McLaughlin says. McLaughlin and her colleagues were involved in a clinical trial of a new potential medicine, Sotatercept, that reduces the number of cells that shouldn't be there. Experts think that excessive cells in the arterial lung walls contribute to PAH.
"It's what we call reverse remodel, getting rid of some of those overgrowing … blood vessels to help attack the actual problem," McLaughlin says. "This medication is potentially reverse remodeling what has happened to the blood vessels in pulmonary arterial hypertension."
For the trial, researchers looked at people on the standard of care treatment. Some received a placebo while others received the drug they were investigating. Participants did a six-minute hall walk before and after the trial to determine their ability.
"The six-minute hall walk is a very common endpoint in clinical trials in pulmonary hypertension," she says.
They also measured clinical outcomes such as patients' need for hospitalization, different therapies and quality of life.
"At the end of the 24 weeks in this trial, there was a statistically significant improvement in the six-minute hall walk, of about 40 meters, in patients who received the Sotatercept versus placebo," she says. "There were also improvements in the first eight secondary endpoint so a really strong positive trials in almost everything."
At the time her brother had pulmonary arterial hypertension it seemed like there were few treatments for it. Years later, Heather Kauffman joined a clinical trial to help find better therapies for the condition.Courtesy Heather Kauffman The researchers published the results in The New England Journal of Medicine. Currently, the medication is awaiting FDA approval before others can have access to it.
'Looking forward'While Kauffman doesn't know if she was taking the medication or the placebo during the trial, her health has improved (she's now officially on it because participants are all offered access to it following the end of the study). After first meeting with a transplant doctor in 2020, they planned on her coming back annually to see if she still needed a transplant — being in a clinical trial didn't exclude her from it. But when she returned after being in the study, her outlook changed.
"When I went back for that next annual appointment we agreed that I was doing so much better that I no longer needed to come back," she says. "It's (been) put on hold."
If she starts having more problems, they might reassess her need for new lungs. Kauffman also underwent genetic testing to see if she had genes for PAH because she worried that her younger sister and her children could be impacted by it.
"I don't have any of the genes that they test for," she says.
She feels grateful she had a chance to participate in a clinical trial for the condition.
"I just want to keep looking forward," Kauffman says.
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