Portal hypertension natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Natural History, Complications, and Prognosis
- Portal hypertension is increased hepatic venous pressure gradient (HVPG) above 5 mmHg.
- 90% of the cases are due to hepatic cirrhosis.[1]
- The stage of cirrhosis severity is determined base on Child-Pugh-Turcotte (CPT) scoring system, including Child A if 5-6 points, Child B if 7-9 points, and Child C if 10-15 points.
| Parameter | Points | ||
|---|---|---|---|
| 1 | 2 | 3 | |
| Ascites | None | Mild/Moderate | Tense |
| Hepatic encephalopathy | None | Grade 1-2 | Grade 3-4 |
| Bilirubin μMol/L (mg/dL) | <34.2 (<2) | 34.2–51.3 (2-3) | >51.3 (>3) |
| Albumin g/L (g/dL) | >35 (>3.5) | 28–35 (2.8–3.5) | <28 (<2.8) |
| PT (Sec)
or INR |
<4 | 4–6 | >6 |
| <1.7 | 1.7–2.3 | >2.3 | |
Natural History
- The symptoms of portal hypertension usually develop in the third and fourth decades of life, and generally start with symptoms such as esophageal varices, caput medusae, spider angioma, and splenomegaly.
- The HVPG threshold for portal hypertension to become clinically symptomatic is 10-12mm Hg.[2]
- Esophageal varices occur when the HVPG reaches 10 mmHg. 40% of Child A and 85% of Child C cirrhosis may progress to esophageal varices.[3]
- Esophageal varices are typically developed 5-15% per year after cirrhosis. Most of the cirrhotic patients will develop the varices during the lifetime.[1]
- Esophageal varices enlarge and progress about 8% per year.[4] The varices are bleeding 5-15% per year.[5]
 |  | ||||||||||||||||||||||||||||||||||
| Gastroesophageal varices type 1 Extend along the lesser curvature | Gastroesophageal varices type 2 Extend along the fundus | ||||||||||||||||||||||||||||||||||
 |  | ||||||||||||||||||||||||||||||||||
| Isolated gastric varices type 1 Located in the fundus and tend to be tortuous and complex | Isolated gastric varices Located in the body, antrum, or around the pylorus | ||||||||||||||||||||||||||||||||||
- Approximately 60% of patients with cirrhosis develop ascites in 10 years.[6]
- At least 1500 cc ascites is necessary to become identifiable through physical exam. The severe increase in intra-abdominal pressure may lead to respiratory problems or abdominal hernias.[7]
- Based on grade of cirrhosis, international ascites club presented a grading system.[8]
| Grade | Definition |
|---|---|
| Grade 1 | Mild ascites only detectable by ultrasound |
| Grade 2 | Moderate ascites evident by moderate symmetrical distension of abdomen |
| Grade 3 | Large or gross ascites with marked abdominal distension |
10% of hospitalized patients with cirrhosis will involve in spantanous bacterial peritonitis (SBP).[9]
- If left untreated, 20-40% of patients with SBP may progress to death.[10]
- Hepatorenal syndrome incidence rate in cirrhotic patients is 20% in 1 year and 40% in 5 years period.[11]
- If left untreated, 100% of patients with type 1 hepatorenal syndrome may progress to death.[12]
- If left untreated, most of the patients with portal hypertension and cirrhosis may progress to hepatic encephalopathy.
Complications
- Common complications of portal hypertension include:
- Spontaneous bacterial peritonitis
- Hepatorenal syndrome
- Hepatic encephalopathy
- Congestive gastropathy
- Hypersplenism
- Ascites
- Variceal bleeding
- Renal failure
- Hepatic hydrothorax
- Hepatopulmonary syndrome
- Portopulmonary hypertension
- Cirrhotic cardiomyopathy
Prognosis
- The presence of variceal bleeding, spontaneous bacterial peritonitis, and hepatorenal syndrome are associated with a particularly poor prognosis among patients with portal hypertension. They are the leading causes of death among patients with portal hypertension.
- When esophageal varices bleed the average rate of 6-week mortality become ≥ 20%, despite improved therapies.[13]
- When ascites occurs the 2-year mortality rate become 50%, without liver transplantation.[14]
- When SBP occurs the mortality rate of 20-40% is expected.[10]
- Prognosis is generally very poor in type 1 hepatorenal syndrome, all of them would die despite appropriate treatments.[12]
References
- ↑ 1.0 1.1 Al-Busafi, Said A.; McNabb-Baltar, Julia; Farag, Amanda; Hilzenrat, Nir (2012). "Clinical Manifestations of Portal Hypertension". International Journal of Hepatology. 2012: 1–10. doi:10.1155/2012/203794. ISSN 2090-3448.
- ↑ Groszmann, Roberto J.; Garcia-Tsao, Guadalupe; Bosch, Jaime; Grace, Norman D.; Burroughs, Andrew K.; Planas, Ramon; Escorsell, Angels; Garcia-Pagan, Juan Carlos; Patch, David; Matloff, Daniel S.; Gao, Hong; Makuch, Robert (2005). "Beta-Blockers to Prevent Gastroesophageal Varices in Patients with Cirrhosis". New England Journal of Medicine. 353 (21): 2254–2261. doi:10.1056/NEJMoa044456. ISSN 0028-4793.
- ↑ Garcia-Tsao, Guadalupe; Groszmann, Roberto J.; Fisher, Rosemarie L.; Conn, Harold O.; Atterbury, Colin E.; Glickman, Morton (1985). "Portal pressure, presence of gastroesophageal varices and variceal bleeding". Hepatology. 5 (3): 419–424. doi:10.1002/hep.1840050313. ISSN 0270-9139.
- ↑ Merli, Manuela; Nicolini, Giorgia; Angeloni, Stefania; Rinaldi, Vittorio; De Santis, Adriano; Merkel, Carlo; Attili, Adolfo Francesco; Riggio, Oliviero (2003). "Incidence and natural history of small esophageal varices in cirrhotic patients". Journal of Hepatology. 38 (3): 266–272. doi:10.1016/S0168-8278(02)00420-8. ISSN 0168-8278.
- ↑ "Prediction of the First Variceal Hemorrhage in Patients with Cirrhosis of the Liver and Esophageal Varices". New England Journal of Medicine. 319 (15): 983–989. 1988. doi:10.1056/NEJM198810133191505. ISSN 0028-4793.
- ↑ Ginés, Pere; Quintero, Enrique; Arroyo, Vicente; Terés, Josep; Bruguera, Miguel; Rimola, Antoni; Caballería, Joan; Rodés, Joan; Rozman, Ciril (1987). "Compensated cirrhosis: Natural history and prognostic factors". Hepatology. 7 (1): 122–128. doi:10.1002/hep.1840070124. ISSN 0270-9139.
- ↑ Cárdenas, Andrés; Arroyo, Vicente (2003). "Mechanisms of water and sodium retention in cirrhosis and the pathogenesis of ascites". Best Practice & Research Clinical Endocrinology & Metabolism. 17 (4): 607–622. doi:10.1016/S1521-690X(03)00052-6. ISSN 1521-690X.
- ↑ Moore KP, Wong F, Gines P, Bernardi M, Ochs A, Salerno F, Angeli P, Porayko M, Moreau R, Garcia-Tsao G, Jimenez W, Planas R, Arroyo V (2003). "The management of ascites in cirrhosis: report on the consensus conference of the International Ascites Club". Hepatology. 38 (1): 258–66. doi:10.1053/jhep.2003.50315. PMID 12830009.
- ↑ Nousbaum, Jean-Baptiste; Cadranel, Jean-François; Nahon, Pierre; Khac, Eric Nguyen; Moreau, Richard; Thévenot, Thierry; Silvain, Christine; Bureau, Christophe; Nouel, Olivier; Pilette, Christophe; Paupard, Thierry; Vanbiervliet, Geoffroy; Oberti, Frédéric; Davion, Thierry; Jouannaud, Vincent; Roche, Bruno; Bernard, Pierre-Henri; Beaulieu, Sandrine; Danne, Odile; Thabut, Dominique; Chagneau-Derrode, Carinne; de Lédinghen, Victor; Mathurin, Philippe; Pauwels, Arnaud; Bronowicki, Jean-Pierre; Habersetzer, François; Abergel, Armand; Audigier, Jean-Christian; Sapey, Thierry; Grangé, Jean-Didier; Tran, Albert (2007). "Diagnostic accuracy of the Multistix 8 SG reagent strip in diagnosis of spontaneous bacterial peritonitis". Hepatology. 45 (5): 1275–1281. doi:10.1002/hep.21588. ISSN 0270-9139.
- ↑ 10.0 10.1 Tandon P, Garcia-Tsao G (2008). "Bacterial infections, sepsis, and multiorgan failure in cirrhosis". Semin. Liver Dis. 28 (1): 26–42. doi:10.1055/s-2008-1040319. PMID 18293275.
- ↑ Ginès A, Escorsell A, Ginès P, Saló J, Jiménez W, Inglada L, Navasa M, Clària J, Rimola A, Arroyo V (1993). "Incidence, predictive factors, and prognosis of the hepatorenal syndrome in cirrhosis with ascites". Gastroenterology. 105 (1): 229–36. PMID 8514039.
- ↑ 12.0 12.1 Salerno, F.; Gerbes, A.; Gines, P.; Wong, F.; Arroyo, V. (2008). "Diagnosis, prevention and treatment of hepatorenal syndrome in cirrhosis". Postgraduate Medical Journal. 84 (998): 662–670. doi:10.1136/gut.2006.107789. ISSN 0032-5473.
- ↑ D'Amico, G (2003). "Upper digestive bleeding in cirrhosis. Post-therapeutic outcome and prognostic indicators". Hepatology. 38 (3): 599–612. doi:10.1053/jhep.2003.50385. ISSN 0270-9139.
- ↑ D'amico, Gennaro; Morabito, Alberto; Pagliaro, Luigi; Marubini, Ettore (1986). "Survival and prognostic indicators in compensated and decompensated cirrhosis". Digestive Diseases and Sciences. 31 (5): 468–475. doi:10.1007/BF01320309. ISSN 0163-2116.