门静脉性肺动脉高压的研究现状

殷鑫; 张雨; 邵玥明; 高卉; 秦婷婷; 温晓玉

doi:10.3969/j.issn.1001-5256.2020.01.051

门静脉性肺动脉高压的研究现状

DOI: 10.3969/j.issn.1001-5256.2020.01.051

1. 吉林大学第一医院肝病科
2. 泰安市立医院消化内科

基金项目:

吉林省自然科学基金项目(20190201065JC)；

详细信息

中图分类号: R575.2
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出版历程
- 出版日期: 2020-01-20

Current status of the research on portopulmonary hypertension

Department of Hepatology, The First Hospital of Jilin University, Changchun 130021, China

Research funding:

摘要

摘要:
门静脉性肺动脉高压(POPH)是在门静脉高压基础上发生的肺动脉压力升高,随着病情的进展导致进行性右心功能衰竭并最终死亡。该病发病率低,由于患者早期无症状或非特异性症状,常被临床医生忽视。总结近年来该病的发病机制、诊断及治疗的进展,介绍目前POPH诊断可应用的无创检测方法,探讨根据POPH的严重程度及个体化情况选择恰当的治疗方式,提高临床医生对该病的认识。
- 高血压,肺性 /
- 高血压,门静脉 /
- 诊断 /
- 治疗学
Abstract:
Portopulmonary hypertension(POPH) refers to the increase in pulmonary artery pressure on the basis of portal hypertension,which may lead to progressive right ventricular failure and death with disease progression. POPH has a low incidence rate and is often neglected by clinicians due to a lack of symptoms or the presence of non-specific symptoms in the early stage. This article summarizes the research advances in the pathogenesis,diagnosis,and treatment of POPH in recent years,introduces the noninvasive detection methods currently available for POPH diagnosis,and explores the selection of appropriate treatment methods based on the severity and individualized conditions of patients with POPH,so as to improve the understanding of this disease among clinicians.
- hypertension,pulmonary /
- hypertension,portal /
- diagnosis /
- therapeutics

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参考文献(51)

[1] SIMONNEAU G,GATZOULIS MA,ADATIA I,et al. Updated clinical classification of pulmonary hypertension[J]. J Am Coll Cardiol,2013,62(25 Suppl):34-41.

[2] MANTZ FA Jr,CRAIGE E. Portal axis thrombosis with spontaneous portacaval shunt and resultant cor pulmonale[J]. AMA Arch Pathol,1951,52(1):91-97.

[3] BADESCH DB,RASKOB GE,ELLIOTT CG,et al. Pulmonary arterial hypertension:baseline characteristics from the REVEAL Registry[J]. Chest,2010,137(2):376-387.

[4] MANCUSO L,SCORDATO F,PIERI M,et al. Management of portopulmonary hypertension:new perspectives[J]. World J Gastroenterol,2013,19(45):8252-8257.

[5] RODRIGUEZ-ROISIN R,KROWKA MJ,HERVP,et al. Pulmonary-Hepatic vascular Disorders(PHD)[J]. Eur Respir J,2004,24(5):861-880.

[6] LI J,ZHUANG Q,ZHANG X,et al. Prevalence and prognosis of portopulmonary hypertension in 223 liver transplant recipients[J]. Can Respir J,2018,2018:1-6.

[7] KAWUT SM,KROWKA MJ,TROTTER JF,et al. Clinical risk factors for portopulmonary hypertension[J]. Hepatology,2008,48(1):196-203.

[8] RAMSAY MA,SIMPSON BR,NGUYEN AT,et al. Severe pulmonary hypertension in liver transplant candidates[J]. Liver Transpl Surg,1997,3(5):494-500.

[9] LE PJ,SOUZA R,HERVE P,et al. Portopulmonary hypertension:Survival and prognostic factors[J]. Am J Respir Crit Care Med,2008,178(6):637-643.

[10] SEGRAVES JM,CARTIN-CEBA R,LEISE MD,et al. Relationship between portopulmonary hypertension and splenectomy:Mayo Clinic experience and review of published works[J]. Hepatol Res,2018,48(3):340-346.

[11] RODRGUEZ-VILARRUPLA A,FERNNDEZ M,BOSCH J,et al. Current concepts on the pathophysiology of portal hypertension[J]. Ann Hepatol,2007,6(1):28-36.

[12] RAEVENS S,GEERTS A,van STEENKISTE C,et al. Hepatopulmonary syndrome and portopulmonary hypertension:Recent knowledge in pathogenesis and overview of clinical assessment[J]. Liver Int,2015,35(6):1646-1660.

[13] TALWALKAR JA,SWANSON KL,KROWKA MJ,et al. Prevalence of spontaneous portosystemic shunts in patients with portopulmonary hypertension and effect on treatment[J].Gastroenterology,2011,141(5):1673-1679.

[14] THEVENOT T,WEIL D,di MARTINO V. What is the true relationship between spontaneous portosystemic shunts and portopulmonary hypertension in cirrhotic patients[J]. Gastroenterology,2018,155(5):1647-1648.

[15] LANE KB,MACHADO RD,PAUCIULO MW,et al. Heterozygous germline mutations in BMPR2,encoding a TGF-beta receptor,cause familial primary pulmonary hypertension[J].Nature Genetics,2000,26(1):81-84.

[16] DENG Z,MORSE JH,SLAGER SL,et al. Familial primary pulmonary hypertension(gene PPH1)is caused by mutations in the bone morphogenetic protein receptor-II gene[J]. Am J Hum Genet,2000,67(3):737-744.

[17] NIKOLIC I,YUNG LM,YANG P,et al. Bone morphogenetic protein 9 is a mechanistic biomarker of portopulmonary hypertension[J]. Am J Respir Crit Care Med,2019,199(7):891-902.

[18] POUSADA G,BALOIRA A,VALVERDE D. Mutational screening in genes related with porto-pulmonary hypertension:An analysis of 6 cases[J]. Med Clin(Barc),2017,148(7):310-313.

[19] CHEN H,WANG F,XIAO J,et al. Natural killer cell-mediated immune deficiency or compromise in patients with portopulmonary hypertension[J]. Arch Med Sci,2014,10(5):1055-1056.

[20] ROBERTS KE,FALLON MB,KROWKA MJ,et al. Genetic risk factors for portopulmonary hypertension in patients with advanced liver disease[J]. Am J Respir Crit Care Med,2009,179(9):835-842.

[21] TERAO M,TAKAKI A,MARUYAMA T,et al. Serum oxidative/anti-oxidative stress balance is dysregulated in potentially pulmonary hypertensive patients with liver cirrhosis:A case control study[J]. Intern Med,2015,54(22):2815-2826.

[22] FUSSNER LA,IYER VN,CARTIN-CEBA R,et al. Intrapulmonary vascular dilatations are common in portopulmonary hypertension and may be associated with decreased survival[J].Liver Transpl,2015,21(11):1355-1364.

[23] SIMONNEAU G,MONTANI D,CELERMAJER DS,et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension[J]. Eur Respir J,2019,53(1):1801913.

[24] SWANSON KL,KROWKA MJ. Arterial oxygenation associated with porto-pulmonary hypertension[J]. Chest,2002,121(6):1869-1875.

[25] COLLE IO,MOREAU R,GODINHO E,et al. Diagnosis of portopulmonary hypertension in candidates for liver transplantation:a prospective study[J]. Hepatology,2003,37(2):401-409.

[26] KROWKA MJ,SWANSON KL,FRANTZ RP,et al. Portopulmonary hypertension:Results from a 10-year screening algorithm[J]. Hepatology,2006,44(6):1502–1510.

[27] DUBROCK HM,RODRIGUEZ-LOPEZ JM,LEVARGE BL,et al. Macrophage migration inhibitory factor as a novel biomarker of portopulmonary hypertension[J]. Pulm Circ,2016,6(4):498-507.

[28] LONG L,ORMISTON ML,YANG X,et al. Selective enhancement of endothelial BMPR-II with BMP9 reverses pulmonary arterial hypertension[J]. Nat Med,2015,21(7):777-785.

[29] MIMIDIS KP,VASSILAKOS PI,MASTORAKOU AN,et al.Evaluation of contrast echocardiography and lung perfusion scan in detecting intrapulmonary vascular dilatation in normoxemic patients with early liver cirrhosis[J]. Hepatogastroenterology,1998,45(24):2303-2307.

[30] AWDISH RL,CAJIGAS HR. Early initiation of prostacyclin in portopulmonary hypertension:10 years of a transplant center’s experience[J]. Lung,2013,191(6):593-600.

[31] SITBON O,CHANNICK R,CHIN KM,et al. Selexipag for the treatment of pulmonary arterial hypertension[J]. N Engl J Med,2015,373(26):2522-2533.

[32] SIMONNEAU,GALIN,JANSA P,et al. Long-term results from the EARLY study of bosentan in WHO functional class II pulmonary arterial hypertension patients[J]. Int J Cardiol,2014,172(2):332-339.

[33] CARTIN-CEBA R,SWANSON K,IYER V,et al. Safety and efficacy of ambrisentan for the treatment of portopulmonary hypertension[J]. Chest,2011,139(1):109-114.

[34] PULIDO T,ADZERIKHO I,CHANNICK RN,et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension[J]. N Engl J Med,2013,369(9):809-818.

[35] SITBON O,BOSCH J,COTTREEL E,et al. Macitentan for the treatment of portopulmonary hypertension(PORTICO):A multicentre,randomised,double-blind,placebo-controlled,phase 4 trial[J]. Lancet Respir Med,2019,7(7):594-604.

[36] FISHER JH,JOHNSON SR,CHAU C,et al. Effectiveness of phosphodiesterase-5 inhibitor therapy for portopulmonary hypertension[J]. Can Respir J,2015,22(1):42-46.

[37] STEVELING E,OLSCHEWSKI H,GRIMMINGER F,et al. Sildenafil treatment for portopulmonary hypertension[J]. Eur Respir J,2006,28(3):563-567.

[38] CARTIN-CEBA R,HALANK M,GHOFRANI HA,et al. Riociguat treatment for portopulmonary hypertension:A subgroup analysis from the PATENT-1/-2 studies[J]. Pulm Circ,2018,8(2):2045894018769305.

[39] GHOFRANI HA,GRIMMINGER F,GRUNIG E,et al. Predictors of long-term outcomes in patients treated with riociguat for chronic thromboembolic pulmonary arterial hypertension:Data from the PATENT-2 open-label,randomised,longterm extension trial[J]. Lancet Respir Med,2016,4(5):361-371.

[40] SCHWABL P,BRUSILOVSKAYA K,RIEDL F,et al. The guanylyl cyclase stimulator rloclguat reduces liver fibrosis and portal pressure in cirrhotic rats[J]. J Hepatology,2016,64(2):s140.

[41] VIONNET J,YERLY P,AUBERT JD,et al. Management of severe portopulmonary hypertension with dual oral therapy prior to liver transplantation[J]. Transplantation,2018,102(5):e194.

[42] VIONNET J,YERLY P,AUBERT JD,et al. Management of severe portopulmonary hypertension with dual oral therapy prior to liver transplantation[J]. Transplantation,2018,102(5):e194.

[43] LIU YJ,LI T. An excerpt of international liver transplant society practice guidelines:Diagnosis and management of hepatopulmonary hypertension(2016)[J]. J Clin Hepatol,2016,32(10):1838-1842.(in Chinese)刘以俊，李涛．《2016年国际肝移植学会实践指南:肝肺综合征与门脉性肺动脉高压的诊断与管理》摘译[J]．临床肝胆病杂志，2016,32(10):1838-1842.

[44] SARAGAI Y,TAKAKI A,UMEDA Y,et al. A subclinical high tricuspid regurgitation pressure gradient independent of the mean pulmonary artery pressure is a risk factor for the survival after living donor liver transplantation[J]. BMC Gastroenterol,2018,18(1):62.

[45] REYMOND M,BARBIER L,SALAME E,et al. Does portopulmonary hypertension impede liver transplantation in cirrhotic patients? A French multicentric retrospective study[J]. Transplantation,2018,102(4):616-622.

[46] GRANNAS G,NEIPP M,HOEPER MM,et al. Indications for and outcomes after combined lung and liver transplantation:A single-center experience on 13 consecutive cases[J].Transplantation,2008,85(4):524-531.

[47] ROBALINO BD,MOODIE DS. Association between primary pulmonary hypertension and portal hypertension:Analysis of its pathophysiology and clinical,laboratory and hemodynamic manifestations[J]. J Am Coll Cardiol,1991,17(2):492-498.

[48] LEPAVEC J,SOUZA R,HERVE P,et al. Portopulmonary hypertension:Survival and prognostic factors[J]. Am J Respir Crit Care Med,2008,178(6):637-643.

[49] KAWUT SM,TAICHMAN DB,AHYA VN,et al. Hemodynamics and survival of patients with porto-pulmonary hypertension[J]. Liver Transpl,2005,11(9):1107-1111.

[50] KROWKA MJ,MILLER DP,BARST RJ,et al. Portopulmonary hypertension:A report from the US-based REVEAL Registry[J]. Chest,2012,141(4):906-915.

[51] SWANSON KL,WIESNER RH,NYBERG SL,et al. Survival in porto-pulmonary hypertension:Mayo Clinic experience categorized by treatment subgroups[J]. Am J Transplant,2008,8(11):2445-2453.

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