FONTAN术后相关性肝病研究进展

摘要/Abstract

摘要： Fontan术式的发展和多学科联合管理使得患有单室生理性先天性心脏病的儿童能够得到及时救治。Fontan术后的常见并发症中包括Fontan相关性肝病,并且几乎100%的患者在青春期会出现临床上无症状的肝纤维化。本文就Fontan相关性肝病的病理生理学机制、临床表现及诊断方法的进展做如下综述,以期为慢性肝病的临床诊治提供借鉴。

关键词: Fontan手术, Fontan相关性肝病, 肝纤维化, 诊断方法

徐强, 王亚年, 吴炎. FONTAN术后相关性肝病研究进展[J]. 肝脏, 2022, 27(6): 624-627.

[1] Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax,1971,26:240-248.
[2] Ghaferi AA, Hutchins GM. Progression of liver pathology in patients undergoing the Fontan procedure: Chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma. J Thorac Cardiovasc Surg,2005,129:1348-1352.
[3] de Leval MR, Kilner P, Gewillig M, et al. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. J Thorac Cardiovasc Surg,1988,96:682-695.
[4] Gewillig M. The Fontan circulation. Heart,2005,91:839-846.
[5] Goldberg DJ, Surrey LF, Glatz AC, et al. Hepatic Fibrosis Is Universal Following Fontan Operation, and Severity is Associated With Time From Surgery: A Liver Biopsy and Hemodynamic Study. J Am Heart Assoc,2017,6.
[6] Mandelbaum I, Burns WH. Pulsatile and Nonpulsatile Blood Flow. JAMA, 1965,191:657-660.
[7] Simonetto DA, Yang HY, Yin M, et al. Chronic passive venous congestion drives hepatic fibrogenesis via sinusoidal thrombosis and mechanical forces. Hepatology,2015,61:648-659.
[8] Hilscher MB, Sehrawat T, Arab JP, et al. Mechanical Stretch Increases Expression of CXCL1 in Liver Sinusoidal Endothelial Cells to Recruit Neutrophils, Generate Sinusoidal Microthombi, and Promote Portal Hypertension. Gastroenterology 2019;157:193-209 e9.
[9] Nagatomo Y, Tang WH. Intersections Between Microbiome and Heart Failure: Revisiting the Gut Hypothesis. J Card Fail,2015,21:973-980.
[10] Sandek A, Bauditz J, Swidsinski A, et al. Altered intestinal function in patients with chronic heart failure. J Am Coll Cardiol,2007,50:1561-1569.
[11] Byrne RD, Weingarten AJ, Clark DE, et al. Sizing Up Fontan Failure: Association with Increasing Weight in Adulthood. Pediatr Cardiol,2021,42:1425-1432.
[12] Allen LA, Felker GM, Pocock S, et al. Liver function abnormalities and outcome in patients with chronic heart failure: data from the Candesartan in Heart Failure: Assessment of Reduction in Mortality and Morbidity (CHARM) program. Eur J Heart Fail,2009,11:170-177.
[13] Sessa A, Allaire M, Lebray P, et al. From congestive hepatopathy to hepatocellular carcinoma, how can we improve patient management? JHEP Rep,2021,3:100249.
[14] Gordon-Walker TT, Bove K, Veldtman G. Fontan-associated liver disease: A review. J Cardiol,2019,74:223-232.
[15] Elder RW, McCabe NM, Hebson C, et al. Features of portal hypertension are associated with major adverse events in Fontan patients: the VAST study. Int J Cardiol, 2013,168:3764-3769.
[16] Farias AQ, Silvestre OM, Garcia-Tsao G, et al. Serum B-type natriuretic peptide in the initial workup of patients with new onset ascites: a diagnostic accuracy study. Hepatology, 2014,59:1043-1051.
[17] Agnoletti G, Ferraro G, Bordese R, et al. Fontan circulation causes early, severe liver damage. Should we offer patients a tailored strategy? Int J Cardiol,2016,209:60-65.
[18] D’Amico G, De Franchis R, Cooperative Study G. Upper digestive bleeding in cirrhosis. Post-therapeutic outcome and prognostic indicators. Hepatology,2003,38:599-612.
[19] Egbe AC, Poterucha JT, Warnes CA, et al. Hepatocellular Carcinoma After Fontan Operation: Multicenter Case Series. Circulation,2018,138:746-748.
[20] Possner M, Gordon-Walker T, Egbe AC, et al. Hepatocellular carcinoma and the Fontan circulation: Clinical presentation and outcomes. Int J Cardiol,2021,322:142-148.
[21] Sterling RK, Lissen E, Clumeck N, et al. Development of a simple noninvasive index to predict significant fibrosis in patients with HIV/HCV coinfection. Hepatology,2006,43:1317-1325.
[22] Baek JS, Bae EJ, Ko JS, et al. Late hepatic complications after Fontan operation; non-invasive markers of hepatic fibrosis and risk factors. Heart,2010,96:1750-1755.
[23] Heuman DM, Mihas AA, Habib A, et al. MELD-XI: a rational approach to "sickest first" liver transplantation in cirrhotic patients requiring anticoagulant therapy. Liver Transpl, 2007,13:30-37.
[24] Byrne RD, Weingarten AJ, Clark DE, et al. More than the heart: Hepatic, renal, and cardiac dysfunction in adult Fontan patients. Congenit Heart Dis,2019,14:765-771.
[25] Amdani S, Simpson KE, Thrush P, et al. Hepatorenal dysfunction assessment with the Model for End-Stage Liver Disease Excluding INR score predicts worse survival after heart transplant in pediatric Fontan patients. J Thorac Cardiovasc Surg, 2022,163:1462-1473 e12.
[26] Wells mL, Fenstad ER, Poterucha JT, et al. Imaging Findings of Congestive Hepatopathy. Radiographics,2016,36:1024-1037.
[27] Wells mL, Venkatesh SK. Congestive hepatopathy. Abdom Radiol (NY), 2018,43:2037-2051.
[28] Friedrich-Rust M, Koch C, Rentzsch A, et al. Noninvasive assessment of liver fibrosis in patients with Fontan circulation using transient elastography and biochemical fibrosis markers. J Thorac Cardiovasc Surg,2008,135:560-567.
[29] Wallihan DB, Podberesky DJ, Marino BS, et al. Relationship of MR elastography determined liver stiffness with cardiac function after Fontan palliation. J Magn Reson Imaging,2014,40:1328-1335.
[30] Silva-Sepulveda JA, Fonseca Y, Vodkin I, et al. Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics. Congenit Heart Dis,2019,14:600-608.
[31] Alsaied T, Possner M, Lubert AM, et al. Relation of Magnetic Resonance Elastography to Fontan Failure and Portal Hypertension. Am J Cardiol,2019,124:1454-1459.
[32] Wu FM, Jonas MM, Opotowsky AR, et al. Portal and centrilobular hepatic fibrosis in Fontan circulation and clinical outcomes. J Heart Lung Transplant,2015;34:883-891.
[33] Schwartz MC, Sullivan LM, Glatz AC, et al. Portal and sinusoidal fibrosis are common on liver biopsy after Fontan surgery. Pediatr Cardiol,2013,34:135-142.
[34] Surrey LF, Russo P, Rychik J, et al. Prevalence and characterization of fibrosis in surveillance liver biopsies of patients with Fontan circulation. Hum Pathol, 2016,57:106-115.
[35] Vaikunth SS, Higgins JP, Concepcion W, et al. Does liver biopsy accurately measure fibrosis in Fontan-associated liver disease? A comparison of liver biopsy pre-combined heart and liver transplant and liver explant post-transplant. Clin Transplant, 2020,34:e14120.

FONTAN术后相关性肝病研究进展

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