Portal hypertension complications

Tekeste, H., Latour, F., Levitt, R.E. Portal hypertension complicating sarcoid liver disease case report and review of the literature. Amer. J. Gastroenterol. 1984 79 389 - 396... 

List the treatment goals for a patient with portal hypertension and its complications. 

O Portal hypertension is the precipitating factor for the complications of cirrhotic liver disease—ascites, spontaneous bacterial peritonitis (SBP), variceal bleeding, and hepatic encephalopathy. Lowering portal pressure can reduce the complications of cirrhosis and decrease morbidity and mortality. 

Cirrhosis is the progressive replacement of normal hepatic cells by fibrous scar tissue. This scarring is accompanied by the loss of viable hepatocytes, which are the functional cells of the liver. Progressive cirrhosis is irreversible and leads to portal hypertension that is in turn responsible for many of the complications of advanced liver disease. These consequences include (but are not limited to) spontaneous bacterial peritonitis (SBP), hepatic encephalopathy, and variceal bleeding.1... 

Clinical Presentation of Cirrhosis and Complications of Portal Hypertension... 

FIGURE 19-4. Treatment algorithm for active gastrointestinal bleeding resulting from portal hypertension. (From Schianotd, Bodenheimer HC. Complications of Chronic Liver Disease. In Friedman SL, McQuaid KR, Grendell JH (eds.)... 

Drug therapy for portal hypertension and cirrhosis can alleviate symptoms and prevent complications but it cannot reverse cirrhosis. Drug therapy is available to treat the complications of ascites, varices, spontaneous bacterial peritonitis, hepatic encephalopathy, and coagulation abnormalities. 

Only non-selective p-blockers reduce bleeding complications in patients with known varices. Blockade of P, receptors reduces cardiac output and splanchnic blood flow. 02-Adrenergic blockade prevents p2-receptor-mediated splanchnic vasodilation while allowing unopposed a-adrenergic effects this enhances vasoconstriction of both the systemic and splanchnic vascular beds. The combination of P, and P2 effects makes the non-selective p-blockers preferable to car-dioselective agents in treating portal hypertension.1,36,41... 

Garcia-Tsao G Current management of the complications of cirrhosis and portal hypertension Variceal hemorrhage, ascites, and spontaneous bacterial peritonitis. Gastroenterology 2001 120 726-748. 

Approximately 20% of patients with chronic HBV infection develop complications of decompensated cirrhosis, including hepatic insufficiency and portal hypertension. HBV is a risk factor for development of hepatocellular carcinoma. 

The patient was a 35-year-old white male with a x-antitrypsin deficiency. He received a combined liver-kidney transplant for cirrhosis complicated by portal hypertension, renal insufficiency secondary to membranoproliferative glomerulonephritis, and combined restrictive and obstructive pulmonary disease at age 18 years. 

This patient illustrates a complicated clinical course of oq-antitrypsin deficiency. Our patient had liver disease that presented during infancy and developed into hepatic cirrhosis. He exhibited most of the complications of cirrhosis, including portal hypertension with ascites, hyperammonemia, malnutrition, and variceal hemorrhage. These complications of cirrhosis are not unique to a,-antitrypsin deficiency, but it is important to note the potential severity of the liver disease associated with this condition. 

Liver disease is managed conventionally, maintaining appropriate nutritional support and managing the complications of portal hypertension and hepatic failure as they occur. We counsel heterozygote parents of a PIZZ... 

Transjugular intrahepatic portosystemic shunt (TIPS) is a side-to-side non-selective portosystemic shunt that is frequently performed in cirrhosis to manage the complications of portal hypertension, such as variceal bleeding. The observation that the bioavailability of oral midazolam was significantly higher in cirrhotic patients with TIPS than in cirrhotic controls and healthy volunteers [57] may be due to reduced intestinal CYP3A activity or reduced contact with CYP3A in the entero-cyte due to increased splanchnic blood flow [57, 92]. 

Transmission of HBV occurs sexually, parenteraUy, and perinataHy. In the United States, transmission occurs predominantly through sexual contact or injection-drug use. International travel is also an important risk factor. Approximately 20% of patients with chronic HBV infection develop complications of decompensated cirrhosis, including hepatic insufficiency and portal hypertension. HBV is a risk factor for development of hepatocellular carcinoma. 

Up to 50% of patients with portal hypertension bleed from oesophageal or gastric varices and half die from complications of their first bleed. Hypovolaemia must be corrected with plasma expanders and blood transfusion. Sepsis is common the incidence rises from 20% at 48 hours to over 60% at 7 days and antimicrobial prophylaxis should be given with ciprofloxacin (Ig/day). Some 70% will stop bleeding spontaneously but over half rebleed within 10 days. 

Hypertension of the portal vein, with its numerous intrinsic or acquired causes, may not display any symptoms for several years. Portal hypertension itself is very often a concomitant symptom in a number of liver diseases. (65) It can lead to severe or even fatal complications. For this reason, hepatological investigation frequently needs to explore (7.) the presence of portal hypertension, (2.) its aetiology, (i.) its severity, and (4.) potentially successful treatment of the underlying causes - in order to produce a favourable effect on portal hypertension. (22, 23, 33, 37, 38, 69, 158)... 

Hadengne, A., Benhayonn, M,K., Lebrec, D., Benhamou, J.-R Pulmonary l pertension complicating portal hypertension prevalence and relation to splanchnic hemodynamics. Gastroenterology 1991 100 520-528... 

Complications The following complications have been reported (i.) cholangitis, (2.) obstructive jaundice, (i.) intrahepatic cholelithiasis, (4.) sepsis, (J.) portal hypertension (oesophageal varices, portal vein thrombosis, chronic Budd-Chiari syndrome, etc.), (6.) thrombosis of the inferior vena cava, (7.) amyloidosis, (8.) immune complex-associated glomerulonephritis, (9.) metastases, (10.) acute on chronic liver insufficiency or acute liver failure, and (11.) bronchobiliary fistula. 

Additional complications were observed during the course of disease, including pronounced cholestasis (113), portal hypertension (95) and liver rupture (lOl). 

Complications In 10% of cases, there is a potential risk of vein obstruction (51) or bleeding. Occasionally, portal hypertension develops, in some cases with pulmonary hypertension. Spontaneous regression has been reported. (49) Malignant degeneration is extremely rare. 

Complications Blunt abdominal trauma increases the risk of rupture, which can also occur spontaneously. The mortality rate is 60-80%. (71) Large shunt volumes may give rise to the development of cardiac insufficiency, particularly during childhood. The development of portal hypertension has also been observed. (92) Anaemia, thrombopenia and hypofibrinogenaemia may occur due to the haemangioma-thrombocytopathy syndrome (= Kasabach-Merritt syndrome). (82)... 

Hepatic veno-occlusive disease occurred in a 38-year-old woman who had occasionally consumed Huamanrripa (Senecio tephrosioides) as a cough remedy for many years (35). She had abdominal pain, jaundice, and anasarca. A hepatic biopsy showed pronounced congestion with a centrilobular predominance, foci of necrosis, and in some areas a reversed lobulation pattern. During the next 13 months she was hospitalized four times with complications of portal hypertension. 

Intestinal transplantation is combined with liver transplantation in 46% of cases, because of terminal liver failure (93). Of 78 patients who had received parenteral nutrition for more than 2 years n — 66) and/ or had short bowel syndrome and could not be weaned from parenteral nutrition (n = 12), 58 developed chronic cholestasis and 37 developed one or more severe liver complication (serum bilirubin concentration 60 pmol/l, factor V (proaccelerin) 50%, portal hypertension, encephalopathy, ascites, bleeding from the gastrointestinal tract, or histological findings consisting of extensive fibrosis and cirrhosis) after 6 (3-132) months and 17 (2-155) months respectively. Liver disease was responsible for deaths in 6.5% of the patients (22% of deaths). 

Cirrhosis and the pathophysiologic abnormalities that cause it result in the commonly encountered problems of ascites, portal hypertension and esophageal varices, hepatic encephalopathy, and coagulation disorders. Other less commonly seen problems in patients with cirrhosis include hepatorenal syndrome, hepatopulmonary syndrome, and endocrine dysfunction, and these are discussed in the section dealing with management of complications. 

The development of the TIPS provided a major improvement in the management of refractory or severe cases of esophagogastric variceal bleeding and other complications of portal hypertension. The TIPS procedure involves the placement of one or more stents between the hepatic vein and the portal vein (Fig. 37-6). This procedure is widely used because it provides an effective decompressive shunt without laparotomy, and can be employed regardless of Child-Pugh score. Survival rates with TIPS in patients refractory to endoscopic treatment are comparable to rates achieved with portacaval... 

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