Portal Hipertansiyon

Yazarlar

Mesut Kaya

Özet

Portal hipertansiyon, portal venöz sistemdeki basıncın normal değerlerin üzerine çıkmasıyla karakterize, çoğunlukla intrahepatik direnç artışından kaynaklanan klinik bir tablodur. Endüstrileşmiş toplumlarda en sık siroza bağlı gelişen bu durum, özofagogastrik varis kanamaları, asit, hipersplenizm ve karaciğer ensefalopatisi gibi hayati komplikasyonlara yol açar. Teşhiste laboratuvar testleri, Doppler ultrasonografi ve endoskopi kritik öneme sahipken; fonksiyonel karaciğer rezervi Child-Pugh sınıflamasıyla değerlendirilir. Akut varis kanamalarında resüsitasyon sonrası endoskopik skleroterapi veya bant ligasyonu birincil tedavi seçeneğidir; medikal farmakoterapi ve Sengstaken-Blakemore tüpü ise süreci destekler. Tedaviye dirençli vakalarda veya Budd-Chiari sendromu gibi nadir portal hipertansiyon formlarında TIPS (transjuguler intrahepatik portosistemik şant) ve cerrahi portosistemik şantlar (DSRS, portakaval şantlar) devreye girer. Cerrahi şant yapılamayan anatomilerde transeksiyon ve devaskülarizasyon (Sugiura) ameliyatları tercih edilirken, son evre karaciğer yetmezliği veya fulminan tablolarda kesin çözüm karaciğer transplantasyonudur. Ayrıca, patofizyolojide amonyak birikimine bağlı gelişen karaciğer ensefalopatisinde laktüloz ve antibiyotikler kullanılırken; trombotik olmayan sinüzoidal tıkanıklıkla seyreden venookluzif hastalık da benzer bir klinik yönetim gerektirir.

Portal hypertension is a clinical manifestation characterized by elevated pressure within the portal venous system, primarily initiated by increased resistance to blood flow. Most commonly resulting from liver cirrhosis in industrialized nations, this condition precipitates life-threatening complications such as esophagogastric variceal bleeding, ascites, hypersplenism, and hepatic encephalopathy. Diagnostic approaches rely heavily on laboratory testing, Doppler ultrasonography, and endoscopy, while hepatic functional reserve is calculated using the Child-Pugh classification. For acute variceal hemorrhage, endoscopic sclerotherapy or band ligation serves as the primary intervention following resuscitation, supported by pharmacological therapies and Sengstaken-Blakemore balloon tamponade. In treatment-resistant cohorts or rare variants like Budd-Chiari syndrome, TIPS (transjugular intrahepatic portosystemic shunt) and surgical portosystemic shunts (e.g., DSRS or portacaval shunts) are leveraged to reduce portal pressure. When shunting is anatomically unfeasible, transection and devascularization procedures (Sugiura) are performed, whereas liver transplantation remains the definitive solution for end-stage failure. Furthermore, management of hepatic encephalopathy—driven by neurotoxic ammonia accumulation—involves lactulose and antibiotics, while non-thrombotic terminal venule obstruction in veno-occlusive disease demands comparable specialized clinical tracking.

Referanslar

Ohtake M. The role of the abdominal sympathetic nervous system in regulating portal venous flow and its functional distribution. Surg Today. 1992;22(2):128-36. doi: 10.1007/BF00311337. PMID: 1498491.

Silva G, Navasa M, Bosch J, Chesta J, Pilar Pizcueta M, Casamitjana R, Rivera F, Rodés J. Hemodynamic effects of glucagon in portal hypertension. Hepatology. 1990 Apr;11(4):668-73. doi: 10.1002/hep.1840110421. PMID: 2328958.

Annalisa Berzigotti, Susana Seijo, Enric Reverter & Jaime Bosch (2013) Assessing portal hypertension in liver diseases, Expert Review of Gastroenterology & Hepatology, 7:2, 141-155, DOI: 10.1586/egh.12.83

Erlinger S, Benhamou J. Cirrhosis: Clinical aspects. In: Oxford Textbook of Clinical Hepatology, Mcintyre N, Benhamou J, Rizzetto M, et al (Eds), University Press, Oxford 1991. p.380.

Ellis G, Goldberg DM, Spooner RJ, Ward AM. Serum enzyme tests in diseases of the liver and biliary tree. Am J Clin Pathol. 1978 Aug;70(2):248-58. doi: 10.1093/ajcp/70.2.248. PMID: 696683.

Kanwal F, Singal AG. Surveillance for Hepatocellular Carcinoma: Current Best Practice and Future Direction. Gastroenterology. 2019 Jul;157(1):54-64. doi: 10.1053/j.gastro.2019.02.049. Epub 2019 Apr 12. PMID: 30986389; PMCID: PMC6636644.

Townsend, J. C. M., Beauchamp, R. D., Evers, B. M., & Mattox, K. L. (2016). Sabiston textbook of surgery (20th ed.). Elsevier - Health Sciences Division.

Nagashima K, Irisawa A, Tominaga K, Kashima K, Kunogi Y, Minaguchi T, Izawa N, Yamamiya A, Yamabe A, Hoshi K, Goda K, Iijima M. The Role of Endoscopic Ultrasound for Esophageal Varices. Diagnostics (Basel). 2020 Nov 25;10(12):1007. doi: 10.3390/diagnostics10121007. PMID: 33255736; PMCID: PMC7760989.

Bosch J, Groszmann RJ, Shah VH. Evolution in the understanding of the pathophysiological basis of portal hypertension: How changes in paradigm are leading to successful new treatments. J Hepatol. 2015 Apr;62(1 Suppl):S121-30. doi: 10.1016/j.jhep.2015.01.003. PMID: 25920081; PMCID: PMC4519833.

Sarin SK, Kumar A, Angus PW, et al. Diagnosis and management of acute variceal bleeding: Asian Pacific Association for Study of the Liver recommendations. Hepatol Int. 2011;5(2):607-624. doi:10.1007/s12072-010-9236-9

Avgerinos A, Armonis A, Stefanidis G, Mathou N, Vlachogiannakos J, Kougioumtzian A, et al. Sustained rise of portal pressure after sclerotherapy, but not band ligation, in acute variceal bleeding in cirrhosis. Hepatology. 2004;39:1623–1630. doi: 10.1002/hep.20236.

Azoulay D, Castaing D, Majno P, Saliba F, Ichaï P, Smail A, Delvart V, Danaoui M, Samuel D, Bismuth H. Salvage transjugular intrahepatic portosystemic shunt for uncontrolled variceal bleeding in patients with decompensated cirrhosis. J Hepatol. 2001 Nov;35(5):590-7. doi: 10.1016/s0168-8278(01)00185-4. PMID: 11690704.

Gur I, Diggs BS, Orloff SL. Surgical portosystemic shunts in the era of TIPS and liver transplantation are still relevant. HPB (Oxford). 2014;16(5):481-493. doi:10.1111/hpb.12163

Ede CJ, Ede R, Brand M. Selective versus non‐selective shunts for the prevention of variceal rebleeding. Cochrane Database Syst Rev. 2019;2019(11):CD013471. Published 2019 Nov 7. doi:10.1002/14651858.CD013471

SHERLOCK S, SUMMERSKILL WH, WHITE LP, PHEAR EA. Portal-systemic encephalopathy; neurological complications of liver disease. Lancet. 1954 Sep 4;267(6836):454-7. PMID: 13193045.

(2001). Hepatic Encephalopathy. In Diseases of the Liver and Biliary System (eds S. Sherlock and J. Dooley). https://doi.org/10.1002/9780470986820.ch7

Fitz GJ. Hepatic Encephalopathy. In: Feldman M., Friedsman L.S., Sleisenger MH., eds. Gastrointestinal and Liver disease. Philadelphia: W. B. Saunders. 2002; 1543-9.

Lai D, Gorbach SL, Levitan R. Intestinal microflora in patients with alcoholic cirrhosis: urea-splitting bacteria and neomycin resistance. Gastroenterology. 1972 Feb;62(2):275-9. PMID: 4637986.

Hansen BA, Vilstrup H. Increased intestinal hydrolysis of urea in patients with alcoholic cirrhosis. Scand J Gastroenterol. 1985 Apr;20(3):346-50. doi: 10.3109/00365528509091662. PMID: 4001843.

Ferenci P, Lockwood A, Mullen K, Tarter R, Weissenborn K, Blei AT. Hepatic encephalopathy--definition, nomenclature, diagnosis, and quantification: final report of the working party at the 11th World Congresses of Gastroenterology, Vienna, 1998. Hepatology. 2002 Mar;35(3):716-21. doi: 10.1053/jhep.2002.31250. PMID: 11870389.

Córdoba J, López-Hellín J, Planas M, Sabín P, Sanpedro F, Castro F, Esteban R, Guardia J. Normal protein diet for episodic hepatic encephalopathy: results of a randomized study. J Hepatol. 2004 Jul;41(1):38-43. doi: 10.1016/j.jhep.2004.03.023. PMID: 15246205.

Miglio F, Valpiani D, Rossellini SR, Ferrieri A. Rifaximin, a non-absorbable rifamycin, for the treatment of hepatic encephalopathy. A double-blind, randomised trial. Curr Med Res Opin. 1997;13(10):593-601. doi: 10.1185/03007999709113333. PMID: 9327194.

Williams R, James OF, Warnes TW, Morgan MY. Evaluation of the efficacy and safety of rifaximin in the treatment of hepatic encephalopathy: a double-blind, randomized, dose-finding multi-centre study. Eur J Gastroenterol Hepatol. 2000 Feb;12(2):203-8. doi: 10.1097/00042737-200012020-00012. PMID: 10741936.

Shawcross D, Jalan R. Dispelling myths in the treatment of hepatic encephalopathy. Lancet. 2005 Jan 29-Feb 4;365(9457):431-3. doi: 10.1016/S0140-6736(05)17832-5. PMID: 15680459.

Elkjaer JM, Jensen TU, Emborg J. Flumazenil til behandling af hepatisk encefalopati [Treatment of hepatic encephalopathy with flumazenil]. Ugeskr Laeger. 2000 Aug 28;162(35):4681-4. Danish. PMID: 10986901.

Menon KV, Shah V, Kamath PS. The Budd-Chiari syndrome. N Engl J Med. 2004 Feb 5;350(6):578-85. doi: 10.1056/NEJMra020282. PMID: 14762185.

Valla DC. Hepatic vein thrombosis (Budd-Chiari syndrome). Semin Liver Dis. 2002 Feb;22(1):5-14. doi: 10.1055/s-2002-23202. PMID: 11928075.

Zeitoun G, Escolano S, Hadengue A, Azar N, El Younsi M, Mallet A, Boudet MJ, Hay JM, Erlinger S, Benhamou JP, Belghiti J, Valla D. Outcome of Budd-Chiari syndrome: a multivariate analysis of factors related to survival including surgical portosystemic shunting. Hepatology. 1999 Jul;30(1):84-9. doi: 10.1002/hep.510300125. PMID: 10385643.

Ludwig J, Hashimoto E, McGill DB, van Heerden JA. Classification of hepatic venous outflow obstruction: ambiguous terminology of the Budd-Chiari syndrome. Mayo Clin Proc. 1990 Jan;65(1):51-5. doi: 10.1016/s0025-6196(12)62109-0. PMID: 2296212.

Chawla Y, Kumar S, Dhiman RK, Suri S, Dilawari JB. Duplex Doppler sonography in patients with Budd-Chiari syndrome. J Gastroenterol Hepatol. 1999 Sep;14(9):904-7. doi: 10.1046/j.1440-1746.1999.01969.x. PMID: 10535473.

Millener P, Grant EG, Rose S, Duerinckx A, Schiller VL, Tessler FN, Perrella RR, Ragavendra N. Color Doppler imaging findings in patients with Budd-Chiari syndrome: correlation with venographic findings. AJR Am J Roentgenol. 1993 Aug;161(2):307-12. doi: 10.2214/ajr.161.2.8333368. PMID: 8333368.

Al-Damegh S. Budd-Chiari syndrome: a short radiological review. J Gastroenterol Hepatol. 1999 Nov;14(11):1057-61. doi: 10.1046/j.1440-1746.1999.02010.x. PMID: 10574131.

Noone TC, Semelka RC, Woosley JT, Pisano ED. Ultrasound and MR findings in acute Budd-Chiari syndrome with histopathologic correlation. J Comput Assist Tomogr. 1996 Sep-Oct;20(5):819-22. doi: 10.1097/00004728-199609000-00029. PMID: 8797923.

Tang TJ, Batts KP, de Groen PC, van Hoek B, Haagsma EB, Hop WC, Janssen HL. The prognostic value of histology in the assessment of patients with Budd-Chiari syndrome. J Hepatol. 2001 Sep;35(3):338-43. doi: 10.1016/s0168-8278(01)00131-3. PMID: 11592594.

Tanaka M, Wanless IR. Pathology of the liver in Budd-Chiari syndrome: portal vein thrombosis and the histogenesis of veno-centric cirrhosis, veno-portal cirrhosis, and large regenerative nodules. Hepatology. 1998 Feb;27(2):488-96. doi: 10.1002/hep.510270224. PMID: 9462648.

Janssen HL, Garcia-Pagan JC, Elias E, Mentha G, Hadengue A, Valla DC; European Group for the Study of Vascular Disorders of the Liver. Budd-Chiari syndrome: a review by an expert panel. J Hepatol. 2003 Mar;38(3):364-71. doi: 10.1016/s0168-8278(02)00434-8. PMID: 12586305.

Slakey DP, Klein AS, Venbrux AC, Cameron JL. Budd-Chiari syndrome: current management options. Ann Surg. 2001 Apr;233(4):522-7. doi: 10.1097/00000658-200104000-00007. PMID: 11303134; PMCID: PMC1421281.

Bilbao JI, Pueyo JC, Longo JM, Arias M, Herrero JI, Benito A, Barettino MD, Perotti JP, Pardo F. Interventional therapeutic techniques in Budd-Chiari syndrome. Cardiovasc Intervent Radiol. 1997 Mar-Apr;20(2):112-9. doi: 10.1007/s002709900117. PMID: 9030501.

Raju GS, Felver M, Olin JW, Satti SD. Thrombolysis for acute Budd-Chiari syndrome: case report and literature review. Am J Gastroenterol. 1996 Jun;91(6):1262-3. PMID: 8651186.

Ganger DR, Klapman JB, McDonald V, Matalon TA, Kaur S, Rosenblate H, Kane R, Saker M, Jensen DM. Transjugular intrahepatic portosystemic shunt (TIPS) for Budd-Chiari syndrome or portal vein thrombosis: review of indications and problems. Am J Gastroenterol. 1999 Mar;94(3):603-8. doi: 10.1111/j.1572-0241.1999.00921.x. PMID: 10086638.

Blum U, Rössle M, Haag K, Ochs A, Blum HE, Hauenstein KH, Astinet F, Langer M. Budd-Chiari syndrome: technical, hemodynamic, and clinical results of treatment with transjugular intrahepatic portosystemic shunt. Radiology. 1995 Dec;197(3):805-11. doi: 10.1148/radiology.197.3.7480760. PMID: 7480760.

Molmenti EP, Segev DL, Arepally A, et al. The utility of TIPS in the management of Budd-Chiari syndrome. Ann Surg. 2005;241(6):978-983. doi:10.1097/01.sla.0000164180.77824.12

Rössle M, Olschewski M, Siegerstetter V, Berger E, Kurz K, Grandt D. The Budd-Chiari syndrome: outcome after treatment with the transjugular intrahepatic portosystemic shunt. Surgery. 2004 Apr;135(4):394-403. doi: 10.1016/j.surg.2003.09.005. PMID: 15041963.

Ahn SS, Yellin A, Sheng FC, Colonna JO, Goldstein LI, Busuttil RW. Selective surgical therapy of the Budd-Chiari syndrome provides superior survivor rates than conservative medical management. J Vasc Surg. 1987 Jan;5(1):28-37. PMID: 3795390.

Schattenfroh N, Bechstein WO, Blumhardt G, Langer R, Lobeck H, Langrehr JM, Neuhaus P. Liver transplantation for PNH with Budd-Chiari syndrome. A case report. Transpl Int. 1993;6(6):354-8. doi: 10.1007/BF00335976. PMID: 8297467.

Janssen, Harry LA, et al. "Budd–Chiari syndrome: a review by an expert panel." Journal of hepatology 38.3 (2003): 364-371.

Dulley, Frederico L., et al. "Venocclusive disease of the liver after chemoradiotherapy and autologous bone marrow transplantation." Transplantation 43.6 (1987): 870-873.

Venous Anatomy of the Abdomen and Pelvis. (2021). Retrieved 27 October 2021, from https://radiologykey.com/venous-anatomy-of-the-abdomen-and-pelvis

Pillai AK, Andring B, Patel A, Trimmer C, Kalva SP. Portal hypertension: a review of portosystemic collateral pathways and endovascular interventions. Clin Radiol. 2015 Oct;70(10):1047-59. doi: 10.1016/j.crad.2015.06.077. Epub 2015 Jul 15. PMID: 26188844.

Dülgeroğlu M, Çekmen N. Ortotopik Karaciğer Transplantasyonu Hastalarında Perioperatif Yönetim. J Turk Soc Intens Care 2019;17:176-189

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