There is no specific symptom associated with portal hypertension. The chances of developing this disease increase with diseases of the liver such as cirrhosis. The complications or symptoms associated with portal hypertension are:

• Gastrointestinal bleeding: If the stool has blood or appears tarry, it is a symptom of gastrointestinal bleeding. Sometimes blood in the vomit is caused due to spontaneous rupture of the varices and hence must not be neglected. 
• Ascites: In this condition, there is an accumulation of the fluid in abdomen. 
• Encephalopathy: Poor liver function can also cause confusion and forgetfulness.
• Altered blood counts: Decline in the number of platelets and white blood cells (WBCs) are common in patients with portal hypertension [6].

• Pain

  BACKGROUND Splenic arteriovenous fistula (AVF) is a rare cause of portal hypertension which may manifest with abdominal pain, diarrhea, ascites, and/or hematemesis. Fistula formation may be traumatic or spontaneous. [ncbi.nlm.nih.gov]

  […] levels and causes complications such as: Ascites (fluid in the abdomen) Hepatic encephalopathy (confusion) Variceal bleeding Vomiting of blood Bloody or black stools Splenomegaly, or an enlarged spleen May have decreased platelets and left abdominal pain [uclahealth.org]

• Pulmonary Valve Stenosis

  A 60-year-old women with a history of congenital pulmonary valve stenosis developed right heart failure, cardiac cirrhosis and end-stage renal disease requiring renal replacement therapy. [ncbi.nlm.nih.gov]

• Splenomegaly

  The SBLS is more feasible and effective than ALS in patients with massive splenomegaly ( 30 cm) secondary to portal hypertension and liver cirrhosis. [ncbi.nlm.nih.gov]

• Abdominal Pain

  BACKGROUND Splenic arteriovenous fistula (AVF) is a rare cause of portal hypertension which may manifest with abdominal pain, diarrhea, ascites, and/or hematemesis. Fistula formation may be traumatic or spontaneous. [ncbi.nlm.nih.gov]

  pain or fullness as a result Because portal hypertension is commonly caused by cirrhosis, liver failure symptoms can also indicate portal hypertension: Jaundice, or yellowing of the skin Easy bruising or bleeding Weakness Weight loss Abdominal pain Diagnosis [uclahealth.org]

• Melena

  Dizziness, nausea, vomiting with red blood, melena was presented on admission. Esophagogastroduodenoscopy revealed III degree varicose of veins from middle part of the esophagus to cardiofundal part of the stomach. [ncbi.nlm.nih.gov]

  Patients present with sudden hematemesis and melena, as well as hypovolemic shock in some cases. [amboss.com]

  Esophageal and gastric varices pose an ongoing risk of life-threatening hemorrhage, with hematemesis or melena. Back to top Vascular Surgery and Laser Vein Center Please contact us at 877.WMC.VEIN or 877.962.8346. [westchestermedicalcenter.org]

• Hematemesis

  A 62-year-old Brazilian man who lived in endemic areas of tropical diseases had an episode of hematemesis associated with portal hypertension. [ncbi.nlm.nih.gov]

  Patients present with sudden hematemesis and melena, as well as hypovolemic shock in some cases. [amboss.com]

• Nausea

  Dizziness, nausea, vomiting with red blood, melena was presented on admission. Esophagogastroduodenoscopy revealed III degree varicose of veins from middle part of the esophagus to cardiofundal part of the stomach. [ncbi.nlm.nih.gov]

  He also reported intermittent nausea, early satiety, and diarrhea but did not have fevers, chills, or vomiting. A new Clinical Problem-Solving article summarizes. [blogs.nejm.org]

  Nausea. Jaundice (yellowing of your skin and eyes). Dark brown Red palms. Vomiting blood. Menstrual problems (in women). Mental confusion, such as a hard time focusing or remembering. Itchy skin. [familydoctor.org]

  Since portal hypertension is a result of liver diseases like cirrhosis, the following symptoms may also be present in the condition : Jaundice Nausea and vomiting Dark stools Pale urine Generalized itching (pruritus) Bleeding disorders Weakness Malaise [healthhype.com]

• Loss of Appetite

  Patients with ascites may have abdominal swelling and pain, which can lead to loss of appetite from the swollen abdomen pressing on the stomach and shortness of breath from the swollen abdomen pressing on the lungs. [nyp.org]

  Loss of appetite. Unplanned weight loss. Weakness and fatigue. Nausea. Jaundice (yellowing of your skin and eyes). Dark brown Red palms. Vomiting blood. Menstrual problems (in women). Mental confusion, such as a hard time focusing or remembering. [familydoctor.org]

  […] of appetite Weight loss Portal Hypertension Causes The cause of portal hypertension is extensive. [healthhype.com]

• Jaundice

  Liver Diseases and Transplantation Autoimmune Hepatitis Bile Duct Cancer Bile Duct Disorders (Noncancerous) Cirrhosis Fatty Liver Disease Hepatitis B Hepatitis C Liver Transplant Liver Tumors Obstructive Jaundice Portal Hypertension Portal hypertension [nyp.org]

  […] part of the abdomen, caused by an enlarged spleen Because portal hypertension is itself often a complication of advanced liver disease, children with the condition may also experience symptoms of poor liver function: poor weight gain or weight loss jaundice [childrenshospital.org]

  […] black stools Splenomegaly, or an enlarged spleen May have decreased platelets and left abdominal pain or fullness as a result Because portal hypertension is commonly caused by cirrhosis, liver failure symptoms can also indicate portal hypertension: Jaundice [uclahealth.org]

  Presentation History For causes of liver disease: History of jaundice. Alcohol consumption. Blood transfusion, especially abroad; lifestyles that predispose to hepatitis B or hepatitis C. [patient.info]

• Hepatomegaly

  In POEMS syndrome, a rare multi-systemic disease, the most frequent liver manifestation is hepatomegaly(1). [ncbi.nlm.nih.gov]

  Subsequent Problems That Can Develop Due to Portal Hypertension: Hepatomegaly (enlarged liver), splenomegaly (enlarged spleen due to increased pressure in the liver), esophageal varices (distended, weakened, and blood-filled veins under pressure, or sacs [arpkdchf.org]

  Ascites Caput medusae Hepatomegaly and splenomegaly Haemorrhoids Oesophageal varices (and thus haematemesis) Major complications of portal hypertension include the following: Variceal haemorrhage Portal hypertensive gastropathy Spontaneus bacterial peritonitis [derangedphysiology.com]

  ) cystic disease of liver (congenital) ( Q44.6 ) hepatic vein thrombosis ( I82.0 ) hepatomegaly NOS ( R16.0 ) pigmentary cirrhosis (of liver) ( E83.110 ) portal vein thrombosis ( I81 ) toxic liver disease ( K71.- ) Other diseases of liver Clinical [icd10data.com]

  Hepatomegaly may also be seen, but the liver parenchyma is of normal echo texture. [doi.org]

• Asterixis

  They may also have a flapping tremor (asterixis), fetor hepaticus (a sweet musty aroma of the breath), hyperventilation and hypothermia. [patient.info]

  […] atrophy – Varices: may be asymptomatic or present with hemorrhage – Portal gastropathy: asymptomatic, chronic anemia, or acute upper GI bleeding – Ascites: abdominal distention, abdominal pain, early satiety, or dyspnea – Altered neurocognitive function: asterixis [clinicaladvisor.com]

  […] possibly, displays of inappropriate or bizarre behavior Impotence and sexual dysfunction Muscle cramps (common in patients with cirrhosis), muscle wasting Dupuytren contracture Palmar erythema and leukonychia: May be present in patients with cirrhosis Asterixis [emedicine.medscape.com]

• Hepatosplenomegaly

  CT scan revealed massive hepatosplenomegaly with focal splenic lesions, soft tissue around renal pelvis, mesenteric masses compressing bowel loops and perilymphatic nodules in lungs. [ncbi.nlm.nih.gov]

  Other features of autosomal recessive osteopetrosis can include slow growth and short stature, dental abnormalities, and an enlarged liver and spleen (hepatosplenomegaly). [ghr.nlm.nih.gov]

• Confusion

  They may also prescribe an antibiotic and/or lactulose, a type of laxative, which help reduce the confusion and other mental changes caused by hepatic encephalopathy. [nyp.org]

  Portal hypertension can lead to a swollen abdomen ( ascites ), abdominal discomfort, confusion, and bleeding in the digestive tract. [msdmanuals.com]

  Portal hypertension can lead to a swollen abdomen (ascites), abdominal discomfort, confusion, and bleeding in the digestive tract. [merckmanuals.com]

  Portal hypertension itself does not cause symptoms, but complications from the condition can lead to an enlarged abdomen, abdominal discomfort, confusion, drowsiness and internal bleeding. [rarediseases.info.nih.gov]

• Complete blood count: Though this is not a specific test to ascertain diagnosis of the disease, it is still advised. In patients with portal hypertension, levels of platelets and WBCs fall.
• Liver function tests: Determining the levels of aspartate aminotransferase [AST], alanine aminotransferase [ALT], and bilirubin determines the functioning of the liver. 
• Coagulation studies: Tests related to coagulation of blood can also prove beneficial in diagnosing the portal hypertension. Some of such tests are: prothrombin time [PT], international normalized ratio [INR] and partial thromboplastin time [PTT]. 
• Some of the other important tests are: Blood urea nitrogen level, levels of creatinine and electrolytes, pH, arterial blood gas, iron indices, hepatic and hepatitis serologies, albumin and antinuclear antibody levels.
• Imagining studies: Such studies include Duplex Doppler ultrasonography, magnetic resonance imaging, bleeding scan and CT scan. 

• Gastric Varices

  The management of oesophageal and gastric varices is particularly important, and both the emergency management together with prophylactic management of this condition are described. [ncbi.nlm.nih.gov]

  Current guidelines recommend endoscopic therapy as first-line treatment for bleeding gastric varices. Endoscopic therapeutic options for gastric variceal bleeding include band ligation, cyanoacrylate (CYA) injection, and thrombin. [elsevier.es]

• Complete Blood Count Normal

  The complete blood count normalized and the biochemistry tests were within normal range after surgery. Postoperative ultrasound and CT confirmed shunt patency and satisfactory flow in the splenoadrenal shunt in all patients. [ncbi.nlm.nih.gov]

• Enlargement of the Spleen

  These include: Enlarged liver and spleen Enlarged veins (varices) of the esophagus and stomach. These can cause abnormal bleeding, such as vomiting blood. [urmc.rochester.edu]

  Other symptoms associated with portal hypertension include enlarged veins, enlargement of the spleen, as well as anemia and vomiting blood. [belmarrahealth.com]

  Splenomegaly (enlargement of the spleen) with consequent sequestration therein of red blood cells, white blood cells, and platelets, together leading to mild pancytopenia. [westchestermedicalcenter.org]

• Paracoccidioides Brasiliensis

  Genotypic analysis revealed that Paracoccidioides brasiliensis S1 was the phylogenic species involved in both cases. Patients presented a good clinical response to amphotericin B and sulfamethoxazole-trimethoprim. [ncbi.nlm.nih.gov]

Treatment of portal hypertension is focused on the prevention and management of the complications associated with the disease, especially bleeding. The severity of the disease is dependent on the extent of liver function. Modification in diet, medications, surgery and endoscopic procedure can help combat the complications of portal hypertension.

1. Endoscopic therapy: This procedure is the first line of treatment for variceal bleeding. Gastroenterologist can also use rubber bands to stop the bleeding by a procedure called banding. In cases where banding cannot be done, sclerotherapy is the option. In this procedure, a blood-clotting solution is injected to stop bleeding.
2. Medications: Beta-blockers such as nadolol and propranolol may be prescribed which reduce the pressure in the varices, reducing the risk of bleeding. Mental changes that accompany encephalopathy can be managed with lactulose.
3. Transjugular intrahepatic portosystemic shunt (TIPS): In this method, a stent is placed in the middle of the liver rerouting the blood flow to the liver.
4. Devascularization: This is a surgical procedure to remove the bleeding varices.
5. Liver transplant: In case of end stage liver disease, transplantation of the liver is the only option [10].

Higher mortality and morbidity rates are reported in patients with severe and persistent gastrointestinal hemorrhage. 90% of complicated cases (due to cirrhosis) of portal hypertension cause variceal hemorrhage.

The mortality rate of the patients who show the first episode of variceal hemorrhage is around 50%, while that with known diagnosis of esophageal varices is 30%.

Patients who report at least one episode of bleeding from the esophageal varices have 80% chances of re-bleeding within 1 year. In patients with esophageal varices, complications arising from immune, cardiovascular, pulmonary, cardiovascular systems contribute to around 20-65% of the deaths [5].

Complications

Common complications associated with portal hypertension are hepatic encephalopathy, aspiration pneumonia, sepsis, renal failure, cardiomyopathy, arrhythmias and hypotension.

Cirrhosis of the liver is the most important cause of portal hypertension. Cirrhosis is the aftermath (healing) of the liver injury which is caused by hepatitis, or excessive alcohol consumption. The scar in the liver, which is caused due to cirrhosis, blocks the flow of blood through this organ affecting its function.

There are some other causes of portal hypertension namely:

1. Blood clots in the portal vein 
2. Blockage of the veins 
3. Schistosomiasis: Parasitic infection
4. Focal nodular hyperplasia [2]

Portal hypertension is the most common manifestation of the liver. In the United States, the deaths associated with the liver cirrhosis (of which portal hypertension is a manifestation) is around 30,000. The international data for portal hypertension is not known, though 30% and 70% of patients with compensated and decompensated cirrhosis respectively suffer from portal hypertension at the time of diagnosis.

In Sub-Saharan Africa, Sudan and Egypt, the most common cause of portal hypertension is Schistosomiasis, while in South America, the Far-East and Southeast Asia, hepatitis B contribute to this condition.

More than 60% of the patients with cirrhosis (which eventually causes portal hypertension) are men; hence gender plays a significant important role in the etiology of the disease. Among children, portal vein thrombosis and secondary biliary cirrhosis contribute to esophageal varices [3] [4].

Portal vein carries the blood from the large and small intestine, stomach and spleen to the liver. Any obstruction of this vein can cause complications leading to portal hypertension.

This change causes an increase in the venous pressure, leading to development of collateral circulation which diverts the blood to the systemic veins. High portal pressure is responsible for the development of the portosystemic collaterals, including esophageal varices [7] [8] [9].

In patients with increased risk of portal hypertension, beta-blockers must be used to prevent the first variceal hemorrhage. To prevent the variceal re-bleeding, the combination of non-selective beta-blockers and a nitrate has been shown to be effective [11].

When there is an increase in blood pressure in the system of veins referred to as a portal system, it is called portal hypertension.

Normal portal pressure ranges between 5 mmHg and 10 mmHg. Generally a portal blood pressure of greater than 12 mmHg causes complications such as ascites and varices. Two factors that contribute to the development of portal hypertension are vascular resistance and blood flow [1].

Definition

Portal hypertension is the elevation of the blood pressure in the portal vein. The portal vein is vital for the normal functioning of the body.

Causes

Obstruction in the portal vein (the blood vessel that carries blood from the intestines, stomach and spleen to the liver) leads to the development of collateral circulation, causing portal hypertension.

Symptoms

Though there are no specific symptoms of the disease, blood in the stool or in vomit can be considered as reason enough to visit the doctor. Some of the other symptoms of portal hypertension are

Diagnosis

Diagnosis of the disease can be done by blood-tests, X-rays and endoscopic examination for the presence of ascites and/or varices.

Treatment

Though the treatment of the disease remains a challenge, management strategy of disease is focused on the prevention of the complications. Beta-blockers and lactulose are some medications which are prescribed to patients with portal hypertension. Treatment with endoscopic therapy has been proved to be most effective. 

1. Harmanci O, Bayraktar Y. Portal hypertension due to portal venous thrombosis: etiology, clinical outcomes. World J Gastroenterol. 2007 May 14;13(18):2535-40.
2. Lubel JS, Angus PW. Modern management of portal hypertension. Intern Med J. Jan 2005;35(1):45-9.
3. Garcia-Tsao G, Sanyal AJ, Grace ND, Carey WD, and the Practice Guidelines Committee of the American Association for the Study of Liver Diseases, the Practice Parameters Committee of the American College of Gastroenterology. Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis. Am J Gastroenterol. Sep 2007;102(9):2086-102.
4. Kim WR, Brown RS Jr, Terrault NA, et al. Burden of liver disease in the United States: Summary of a workshop conducted by the AASLD. May 2001.
5. Benedeto-Stojanov D, Nagorni A, Bjelakovic G, et al. The model for the end-stage liver disease and Child-Pugh score in predicting prognosis in patients with liver cirrhosis and esophageal variceal bleeding. Vojnosanit Pregl. 2009 Sep;66(9):724-8.
6. Barua UK, Hossain AS, Roy GC, et al. Portopulmonary hypertension. Mymensingh Med J. 2013 Jul;22(3):618-24.
7. Obara K. Hemodynamic mechanism of esophageal varices. Dig Endosc. Jan 2006;18(1):6-9.
8. Ravindra KV, Eng M, Marvin M. Current management of sinusoidal portal hypertension. Am Surg. Jan 2008;74(1):4-10. 
9. Cichoz-Lach H, Celinski K, Slomka M, et al. Pathophysiology of portal hypertension. J Physiol Pharmacol. 2008 Aug;59 Suppl 2:231-8.
10. Bari K, Garcia-Tsao G. Treatment of portal hypertension. World J Gastroenterol. 2012 Mar 21;18(11):1166-75.
11. Garcia-Tsao G, Sanyal AJ, Grace ND, et al. Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis. Hepatology, vol. 46, no. 3, pp. 922–938, 2007.