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Wu ZP, Wang YF, Shi FW, Cao WH, Sun J, Yang L, Ding FP, Hu CX, Kang WW, Han J, Yang RH, Song QK, Jin JW, Shi HB, Ma YM. Predictive models and clinical manifestations of intrapulmonary vascular dilatation and hepatopulmonary syndrome in patients with cirrhosis: Prospective comparative study. World J Gastroenterol 2025; 31:105720. [PMID: 40309225 PMCID: PMC12038555 DOI: 10.3748/wjg.v31.i15.105720] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/12/2025] [Revised: 03/13/2025] [Accepted: 03/26/2025] [Indexed: 04/18/2025] Open
Abstract
BACKGROUND Patients with cirrhosis with hepatopulmonary syndrome (HPS) have a poorer prognosis. The disease has a subtle onset, symptoms are easily masked, clinical attention is insufficient, and misdiagnosis rates are high. AIM To compare the clinical characteristics of patients with cirrhosis, cirrhosis combined with intrapulmonary vascular dilatation (IPVD), and HPS, and to establish predictive models for IPVD and HPS. METHODS Patients with cirrhosis were prospectively screened at a liver-specialized university teaching hospital. Clinical information and blood samples were collected, and biomarker levels in blood samples were measured. Patients with cirrhosis were divided into three groups: Those with pure cirrhosis, those with combined IPVD, and those with HPS based on contrast-enhanced transthoracic echocardiography results and the pulmonary alveolar-arterial oxygen gradient values. Univariate logistic regression and Least Absolute Shrinkage and Selection Operator (LASSO) regression methods were utilized to identify risk factors for IPVD and HPS, and nomograms were constructed to predict IPVD and HPS. RESULTS A total of 320 patients were analyzed, with 101 diagnosed with IPVD, of whom 54 were diagnosed with HPS. There were statistically significant differences in clinical parameters among these three groups of patients. Among the tested biomarkers, sphingosine 1 phosphate, angiopoietin-2, and platelet-derived growth factor BB were significantly associated with IPVD and HPS in patients with cirrhosis. Following LASSO logistic regression screening, prediction models for IPVD and HPS were established. The area under the receiver operating characteristic curve for IPVD prediction was 0.792 (95% confidence interval [CI]: 0.737-0.847), and for HPS prediction was 0.891 (95%CI: 0.848-0.934). CONCLUSION This study systematically compared the clinical characteristics of patients with cirrhosis, IPVD, and HPS, and constructed predictive models for IPVD and HPS based on clinical parameters and laboratory indicators. These models showed good predictive value for IPVD and HPS in patients with cirrhosis. They can assist clinicians in the early prognosis assessment of patients with cirrhosis, ultimately benefiting the patients.
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Affiliation(s)
- Zhi-Peng Wu
- Beijing Institute of Hepatology, Beijing Youan Hospital, Capital Medical University, Beijing 100069, China
| | - Ying-Fei Wang
- Beijing Institute of Hepatology, Beijing Youan Hospital, Capital Medical University, Beijing 100069, China
| | - Feng-Wei Shi
- Beijing Institute of Hepatology, Beijing Youan Hospital, Capital Medical University, Beijing 100069, China
| | - Wen-Hui Cao
- Beijing Institute of Hepatology, Beijing Youan Hospital, Capital Medical University, Beijing 100069, China
| | - Jie Sun
- Department of Respiratory and Critical Care Medicine, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
| | - Liu Yang
- Department of Respiratory and Critical Care Medicine, Beijing Youan Hospital, Capital Medical University, Beijing 100069, China
| | - Fang-Ping Ding
- Department of Respiratory and Critical Care Medicine, Beijing Youan Hospital, Capital Medical University, Beijing 100069, China
| | - Cai-Xia Hu
- Hepatic Disease and Tumor Interventional Treatment Center, Beijing Youan Hospital, Capital Medical University, Beijing 100069, China
| | - Wei-Wei Kang
- Fourth Department of Liver Disease, Beijing Youan Hospital, Capital Medical University, Beijing 100069, China
| | - Jing Han
- Ultrasound and Functional Diagnosis Center, Beijing Youan Hospital, Capital Medical University, Beijing 100069, China
| | - Rong-Hui Yang
- Beijing Institute of Hepatology, Beijing Youan Hospital, Capital Medical University, Beijing 100069, China
| | - Qing-Kun Song
- Division of Clinical Epidemiology and Evidence-Based Medicine, Beijing Youan Hospital, Capital Medical University, Beijing 100069, China
| | - Jia-Wei Jin
- Department of Respiratory and Critical Care Medicine, Beijing Chaoyang Hospital, Capital Medical University, Beijing 100043, China
| | - Hong-Bo Shi
- Beijing Institute of Hepatology, Beijing Youan Hospital, Capital Medical University, Beijing 100069, China
| | - Ying-Min Ma
- Beijing Institute of Hepatology, Department of Respiratory and Critical Care Medicine, Beijing Youan Hospital, Capital Medical University, Beijing 100069, Beijing, China
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2
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Zaka AZ, Mangoura SA, Ahmed MA. New updates on hepatopulmonary syndrome: A comprehensive review. Respir Med 2025; 236:107911. [PMID: 39662637 DOI: 10.1016/j.rmed.2024.107911] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/12/2024] [Revised: 11/28/2024] [Accepted: 12/08/2024] [Indexed: 12/13/2024]
Abstract
Hepatopulmonary syndrome (HPS) is a serious pulmonary vascular complication that causes arterial hypoxemia in the setting of liver disease. HPS has a progressive course and is associated with a two-fold increased risk of mortality relative to cirrhotic patients without HPS. It primarily affects patients with portal hypertension. The key pathological features of HPS include intrapulmonary angiogenesis and vascular dilations (IPVDs). The prevalence of HPS varies widely due to inconsistent diagnostic criteria and a lack of standardized protocols. Despite advances in understanding its pathophysiology, no effective curative treatments for HPS exist. Liver transplantation remains the only definitive treatment, improving survival and altering the disease natural course. This review explores the pathophysiology, clinical features, and therapeutic strategies for HPS, highlighting recent advances in the literature.
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Affiliation(s)
- Andrew Z Zaka
- Department of Medical Pharmacology, Faculty of Medicine, Assiut University, Assiut, 71515, Egypt.
| | - Safwat A Mangoura
- Department of Medical Pharmacology, Faculty of Medicine, Assiut University, Assiut, 71515, Egypt; Department of Pharmacology and Toxicology, Faculty of Pharmacy, Badr University in Cairo (BUC), Badr, Cairo, 11829, Egypt.
| | - Marwa A Ahmed
- Department of Medical Pharmacology, Faculty of Medicine, Assiut University, Assiut, 71515, Egypt
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Singh SA, Shrivastava P, Agarwal A, Nandakumar K, Nasa VK, Premkumar G, Rajakumar A, Panchwagh A, Vohra V, Ranade S, Kumar L, Saraf N, Shah V, Sudhidharan S. LTSI Consensus Guidelines: Preoperative Pulmonary Evaluation in Adult Liver Transplant Recipients. J Clin Exp Hepatol 2023; 13:523-531. [PMID: 37250889 PMCID: PMC10213854 DOI: 10.1016/j.jceh.2022.12.012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/02/2022] [Accepted: 12/20/2022] [Indexed: 05/31/2023] Open
Abstract
The relationship between chronic liver disease and respiratory symptoms and hypoxia is well recognized. Over the last century, three pulmonary complications specific to chronic liver disease (CLD) have been characterized: hepatopulmonary syndrome, portopulmonary hypertension, and hepatic hydrothorax. Apart from that coexisting pulmonary disease like chronic obstructive lung disease and interstitial lung disease also complicate the outcomes after liver transplantation (LT). Assessment for evaluation of underlying pulmonary disorders is essential to improve outcomes in patients with CLD, posted for LT. This consensus guideline of the Liver Transplant Society of India (LTSI) provides a comprehensive review of pulmonary issues in CLD, related and unrelated to underlying liver disease and gives recommendations for pulmonary screening in specific clinical scenarios in adults with chronic liver disease planned for LT. This document also aims to standardize the strategies for preoperative evaluation of these pulmonary issues in this subset of patients. Proposed recommendations were based on selected single case reports, small series, registries, databases, and expert opinion. The paucity of randomized, controlled trials in either of these disorders was noted. Additionally, this review will highlight the lacunae in our current evaluation strategy, challenges faced, and will provide direction to potentially useful futuristic preoperative evaluation strategies.
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Affiliation(s)
- Shweta A. Singh
- Center for Liver & Biliary Sciences, Max Super Speciality Hospital, New Delhi, 110017, India
| | | | - Anil Agarwal
- Liver Transplant Anaesthesia, Fortis Hospital, Noida, India
| | - K. Nandakumar
- Liver Transplant Anaesthesia, Apollo Main Hospital, Greams Road, Chennai, India
| | - Vaibhav K. Nasa
- Center for Liver & Biliary Sciences, Max Super Speciality Hospital, New Delhi, 110017, India
| | | | - Akila Rajakumar
- Dr. Rela Institute and Medical Centre, Chromepet, Chennai, Tamil Nadu, India
| | | | - Vijay Vohra
- Medanta - The Medicity Hospital, Gurugram, India
| | - Sharmila Ranade
- Kokilaben Dhirubhai Ambani Hospital and Medical Research Center, Mumbai, India
| | - Lakshmi Kumar
- Amrita Institute of Medical Sciences & Research Centre, Kochi, India
| | - Neeraj Saraf
- Medanta - The Medicity Hospital, Gurugram, India
| | - V.R. Shah
- Institute of Kidney Disease and Research Centre-ITS, Ahmedabad, Gujarat, India
| | - S. Sudhidharan
- HPB and LT Surgery, Amrita Institute of Medical Sciences and Research Centre, Kochi, Kerala, India
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4
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Bommena S, Gerkin RD, Agarwal S, Raevens S, Glassberg MK, Fallon MB. Diagnosis of Hepatopulmonary Syndrome in a Large Integrated Health System. Clin Gastroenterol Hepatol 2021; 19:2370-2378. [PMID: 33007510 DOI: 10.1016/j.cgh.2020.09.050] [Citation(s) in RCA: 9] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/24/2020] [Revised: 08/26/2020] [Accepted: 09/20/2020] [Indexed: 02/07/2023]
Abstract
BACKGROUND & AIMS Data on the accuracy of the diagnosis of hepatopulmonary syndrome (HPS) in cirrhosis is limited. We evaluated the clinical characteristics of patients with International Classification of Diseases (ICD) codes for hepatopulmonary syndrome (HPS) in a large integrated health system. METHODS A retrospective review of encounters was performed of all patients with ICD-9-CM and/or ICD-10-CM diagnosis of cirrhosis and HPS from 2014-2019 in a multi-state health system. Demographics and cardiopulmonary testing closest to the time of HPS diagnosis were recorded. HPS was defined using standard criteria. RESULTS A total of 42,749 unique individuals with cirrhosis were identified. An ICD diagnosis of HPS was found in 194 patients (0.45%), of which 182 had clinically confirmed cirrhosis. 143 (78.5%) underwent contrast-enhanced transthoracic echocardiography, and 98 (54%) had delayed shunting. Among them, 61 patients had a documented arterial blood gas, with 53 showing abnormal oxygenation (A-a gradient of >15 mm Hg). 12 were excluded due to significant pulmonary function test abnormalities and abnormal oxygenation from other cardiopulmonary diseases. Ultimately, 41 (22.5%) fulfilled the criteria for HPS. When stratifying those with an ICD code diagnosis of HPS into HPS, no HPS and indeterminate HPS groups, based on standard diagnostic criteria for HPS, we found that the confirmed HPS patients had similar complications except for less portopulmonary hypertension, worse gas exchange, less cardiopulmonary disease and were more often diagnosed in transplant centers. CONCLUSIONS The diagnosis of HPS by ICD code is made in an extremely small subset of a sizeable cirrhotic cohort. When made, only a minority of these patients meet diagnostic criteria. Our findings highlight the need for improved education and more effective screening algorithms.
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Affiliation(s)
- Shoma Bommena
- Department of Medicine, University of Arizona College of Medicine-Phoenix, Phoenix, Arizona; Department of Internal Medicine, Banner University Medical Center-Phoenix, Phoenix, Arizona.
| | - Richard D Gerkin
- Department of Medicine, University of Arizona College of Medicine-Phoenix, Phoenix, Arizona; Department of Internal Medicine, Banner University Medical Center-Phoenix, Phoenix, Arizona
| | - Sumit Agarwal
- Department of Medicine, University of Arizona College of Medicine-Phoenix, Phoenix, Arizona; Department of Internal Medicine, Banner University Medical Center-Phoenix, Phoenix, Arizona
| | - Sarah Raevens
- Department of Internal Medicine, Ghent University, Ghent, Belgium
| | - Marilyn K Glassberg
- Department of Medicine, University of Arizona College of Medicine-Phoenix, Phoenix, Arizona; Department of Internal Medicine, Banner University Medical Center-Phoenix, Phoenix, Arizona
| | - Michael B Fallon
- Department of Medicine, University of Arizona College of Medicine-Phoenix, Phoenix, Arizona; Department of Internal Medicine, Banner University Medical Center-Phoenix, Phoenix, Arizona
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Del Valle K, DuBrock HM. Hepatopulmonary Syndrome and Portopulmonary Hypertension: Pulmonary Vascular Complications of Liver Disease. Compr Physiol 2021; 11:3281-3302. [PMID: 34636408 DOI: 10.1002/cphy.c210009] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Pulmonary vascular disease is a frequent complication of chronic liver disease and portal hypertension, affecting up to 30% of patients. There are two distinct pulmonary vascular complications of liver disease: hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH). HPS affects 25% of patients with chronic liver disease and is characterized by intrapulmonary vasodilatation and abnormal arterial oxygenation. HPS negatively impacts quality of life and is associated with a 2-fold increased risk of death compared to controls with liver disease without HPS. Angiogenesis, endothelin-1 mediated endothelial dysfunction, monocyte influx, and alveolar type 2 cell dysfunction seem to play important roles in disease pathogenesis but there are currently no effective medical therapies. Fortunately, HPS resolves following liver transplant (LT) with improvements in hypoxemia. POPH is a subtype of pulmonary arterial hypertension (PAH) characterized by an elevated mean pulmonary arterial pressure and pulmonary vascular resistance in the setting of normal left-sided filling pressures. POPH affects 5% to 6% of patients with chronic liver disease. Although the pathogenesis has not been fully elucidated, endothelial dysfunction, inflammation, and estrogen signaling have been identified as key pathways involved in disease pathogenesis. POPH is typically treated with PAH targeted therapy and may also improve with liver transplantation in selected patients. This article highlights what is currently known regarding the diagnosis, management, pathobiology, and outcomes of HPS and POPH. Ongoing research is needed to improve understanding of the pathophysiology and outcomes of these distinct and often misunderstood pulmonary vascular complications of liver disease. © 2021 American Physiological Society. Compr Physiol 11:1-22, 2021.
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Sharma S, Sonny A, Dalia AA, Karamchandani K. Acute heart failure after liver transplantation: A narrative review. Clin Transplant 2020; 34:e14079. [PMID: 32941661 DOI: 10.1111/ctr.14079] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/30/2020] [Revised: 08/06/2020] [Accepted: 08/27/2020] [Indexed: 11/27/2022]
Abstract
Acute heart failure (AHF) is an under recognized yet potentially lethal complication after liver transplantation (LT) surgery. The increase in incidence of liver transplantation amongst high-risk patients and the leniency in the criteria for transplantation, predisposes these patients to postoperative AHF and the antecedent morbidity and mortality. The inability of conventional preoperative cardiovascular testing to accurately identify patients at risk for post-LT AHF poses a considerable challenge to clinicians caring for these patients. Even if high-risk patients are identified, there is considerable ambiguity in the candidacy for transplantation as well as optimization strategies that could potentially prevent the development of AHF in the postoperative period. The intraoperative and postoperative management of patients who develop AHF is also challenging and requires a well-coordinated multidisciplinary approach. The use of mechanical circulatory support in patients with refractory heart failure has the potential to improve outcomes but its use in this complex patient population can be associated with significant complications and requires a stringent risk-benefit analysis on a case-by-case basis.
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Affiliation(s)
- Sonal Sharma
- Department of Anesthesiology and Perioperative Medicine, Penn State Health Milton S. Hershey Medical Center, Penn State College of Medicine, Hershey, PA, USA
| | - Abraham Sonny
- Department of Anesthesia, Critical Care and Pain Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA
| | - Adam A Dalia
- Department of Anesthesia, Critical Care and Pain Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA
| | - Kunal Karamchandani
- Department of Anesthesiology and Perioperative Medicine, Penn State Health Milton S. Hershey Medical Center, Penn State College of Medicine, Hershey, PA, USA
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7
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Dimitroglou Y, Aggeli C, Alexopoulou A, Mavrogeni S, Tousoulis D. Cardiac Imaging in Liver Transplantation Candidates: Current Knowledge and Future Perspectives. J Clin Med 2019; 8:2132. [PMID: 31817014 PMCID: PMC6947158 DOI: 10.3390/jcm8122132] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/22/2019] [Revised: 11/23/2019] [Accepted: 11/26/2019] [Indexed: 12/12/2022] Open
Abstract
Cardiovascular dysfunction in cirrhotic patients is a recognized clinical entity commonly referred to as cirrhotic cardiomyopathy. Systematic inflammation, autonomic dysfunction, and activation of vasodilatory factors lead to hyperdynamic circulation with high cardiac output and low peripheral vascular resistance. Counter acting mechanisms as well as direct effects on cardiac cells led to systolic or diastolic dysfunction and electromechanical abnormalities, which are usually masked at rest but exposed at stress situations. While cardiovascular complications and mortality are common in patients undergoing liver transplantation, they cannot be adequately predicted by conventional cardiac examination including transthoracic echocardiography. Newer echocardiography indices and other imaging modalities such as cardiac magnetic resonance have shown increased diagnostic accuracy with predictive implications in cardiovascular diseases. The scope of this review was to describe the role of cardiac imaging in the preoperative assessment of liver transplantation candidates with comprehensive analysis of the future perspectives anticipated by the use of newer echocardiography indices and cardiac magnetic resonance applications.
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Affiliation(s)
- Yannis Dimitroglou
- Department of Cardiology, National and Kapodistrian University of Athens Medical School, Hippokration General Hospital, 115 27 Athens, Greece; (C.A.); (D.T.)
| | - Constantina Aggeli
- Department of Cardiology, National and Kapodistrian University of Athens Medical School, Hippokration General Hospital, 115 27 Athens, Greece; (C.A.); (D.T.)
| | - Alexandra Alexopoulou
- Department of Internal Medicine and Research Laboratory, National and Kapodistrian University of Athens Medical School, Hippokration General Hospital, 115 27 Athens, Greece
| | - Sophie Mavrogeni
- Onassis Cardiac Center and National and Kapodistrian University of Athens, 176 74 Athens, Greece;
| | - Dimitris Tousoulis
- Department of Cardiology, National and Kapodistrian University of Athens Medical School, Hippokration General Hospital, 115 27 Athens, Greece; (C.A.); (D.T.)
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8
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Abstract
The most common pulmonary complications of chronic liver disease are hepatic hydrothorax, hepatopulmonary syndrome, and portopulmonary hypertension. Hepatic hydrothorax is a transudative pleural effusion in a patient with cirrhosis and no evidence of underlying cardiopulmonary disease. Hepatic hydrothorax develops owing to the movement of ascitic fluid into the pleural space. Hepatopulmonary syndrome and portopulmonary hypertension are pathologically linked by the presence of portal hypertension; however, their pathophysiologic mechanisms are significantly different. Hepatopulmonary syndrome is characterized by low pulmonary vascular resistance secondary to intrapulmonary vascular dilatations and hypoxemia; portopulmonary hypertension features elevated pulmonary vascular resistance and constriction/obstruction within the pulmonary vasculature.
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Affiliation(s)
- Rodrigo Cartin-Ceba
- Division of Pulmonary and Critical Care Medicine, Mayo Clinic Arizona, 13400 East Shea Boulevard, Scottsdale, AZ 85259, USA.
| | - Michael J Krowka
- Division of Pulmonary and Critical Care Medicine, Mayo Clinic Rochester, 200 1st Street SW, Rochester, MN 55905, USA
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9
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Carrier P, Debette-Gratien M, Jacques J, Loustaud-Ratti V. Cirrhotic patients and older people. World J Hepatol 2019; 11:663-677. [PMID: 31598192 PMCID: PMC6783402 DOI: 10.4254/wjh.v11.i9.663] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/17/2019] [Revised: 06/18/2019] [Accepted: 07/16/2019] [Indexed: 02/06/2023] Open
Abstract
The global population is aging, and so the number of older cirrhotic patients is increasing. Older patients are characterised by a risk of frailty and comorbidities, and age is a risk factor for mortality in cirrhotic patients. The incidence of non-alcoholic fatty liver disease as an aetiology of cirrhosis is increasing, while that of chronic viral hepatitis is decreasing. Also, cirrhosis is frequently idiopathic. The management of portal hypertension in older cirrhotic patients is similar to that in younger patients, despite the greater risk of treatment-related adverse events of the former. The prevalence of hepatocellular carcinoma increases with age, but its treatment is unaffected. Liver transplantation is generally recommended for patients < 70 years of age. Despite the increasing prevalence of cirrhosis in older people, little data are available and few recommendations have been proposed. This review suggests that comorbidities have a considerable impact on older cirrhotic patients.
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Affiliation(s)
- Paul Carrier
- Fédération d’Hépatologie, Centre Hospitalier Universitaire Dupuytren de Limoges, Limoges 87042, France
- Faculté de Médecine et de Pharmacie de Limoges, Rue Docteur Marcland, Limoges 87042, France
| | - Marilyne Debette-Gratien
- Fédération d’Hépatologie, Centre Hospitalier Universitaire Dupuytren de Limoges, Limoges 87042, France
- Faculté de Médecine et de Pharmacie de Limoges, Rue Docteur Marcland, Limoges 87042, France
| | - Jérémie Jacques
- Service de Gastroentérologie, Centre Hospitalier Universitaire Dupuytren de Limoges, Limoges 87042, France
| | - Véronique Loustaud-Ratti
- Fédération d’Hépatologie, Centre Hospitalier Universitaire Dupuytren de Limoges, Limoges 87042, France
- Faculté de Médecine et de Pharmacie de Limoges, Rue Docteur Marcland, Limoges 87042, France.
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10
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Intraoperative anesthetic management of the liver transplant recipient with portopulmonary hypertension. Curr Opin Organ Transplant 2019; 24:121-130. [DOI: 10.1097/mot.0000000000000613] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
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11
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Perioperative Management of Patients with Hepatopulmonary Syndrome. CURRENT TRANSPLANTATION REPORTS 2018. [DOI: 10.1007/s40472-018-0208-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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12
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Zhao H, Tsauo J, Zhang X, Ma H, Weng N, Wang L, Li X. Pulmonary transit time derived from pulmonary angiography for the diagnosis of hepatopulmonary syndrome. Liver Int 2018; 38:1974-1981. [PMID: 29573542 DOI: 10.1111/liv.13741] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/28/2017] [Accepted: 03/12/2018] [Indexed: 02/05/2023]
Abstract
BACKGROUND & AIMS Pulmonary transit time (PTT) is the transit time of blood from the right side of the heart to the left side of the heart. The aim of the present study was to evaluate the role of the PTT derived from pulmonary angiography in the diagnosis of hepatopulmonary syndrome (HPS). METHODS From December 2014 to September 2015, all patients with chronic liver disease and/or portal hypertension undergoing a venous interventional radiologic procedure at our institution were eligible for inclusion in this prospective study. Pulmonary angiography was performed in all patients, and the PTT, which was defined as the time between opacification of the pulmonary trunk and the right border of the left atrium, was determined. RESULTS A total of 53 patients were included, 20 of whom had a positive contrast-enhanced echocardiography result and an elevated alveolar-arterial oxygen gradient were considered to have HPS. PTT was significantly shorter in patients with HPS than in those without [median, 3.34 (interquartile range, 3.01-3.67) seconds vs 4.0 (interquartile range, 3.67-4.17) seconds; P < .001]. The area under the receiver operating characteristic curve of PTT for diagnosing HPS was 0.83 (95% confidence interval, 0.70-0.92). The optimal cut-off value of PTT for diagnosing HPS, based on Youden's index, was 3.55 seconds. The sensitivity, specificity and accuracy of PTT < 3.55 seconds for diagnosing HPS were 70%, 85% and 79% respectively. CONCLUSIONS Pulmonary transit time derived from pulmonary angiography is useful for diagnosing HPS, especially for patients with intracardiac shunts and inadequate echocardiographic windows.
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Affiliation(s)
- He Zhao
- Department of Interventional Therapy, National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- Institute of Interventional Radiology, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Jiaywei Tsauo
- Department of Interventional Therapy, National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Xiaowu Zhang
- Department of Interventional Therapy, National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Huaiyuan Ma
- Institute of Interventional Radiology, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Ningna Weng
- Institute of Interventional Radiology, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Luhua Wang
- Department of Radiation Oncology, National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Xiao Li
- Department of Interventional Therapy, National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- Institute of Interventional Radiology, West China Hospital, Sichuan University, Chengdu, Sichuan, China
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13
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Angeli P, Bernardi M, Villanueva C, Francoz C, Mookerjee RP, Trebicka J, Krag A, Laleman W, Gines P. EASL Clinical Practice Guidelines for the management of patients with decompensated cirrhosis. J Hepatol 2018; 69:406-460. [PMID: 29653741 DOI: 10.1016/j.jhep.2018.03.024] [Citation(s) in RCA: 1766] [Impact Index Per Article: 252.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/28/2018] [Accepted: 03/28/2018] [Indexed: 02/06/2023]
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14
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Cardoso FS, Karvellas CJ. Respiratory Complications Before and After Liver Transplant. J Intensive Care Med 2018; 34:355-363. [PMID: 29886790 DOI: 10.1177/0885066618781526] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Abstract
Respiratory complications before and after liver transplant are common, diverse, and potentially have a negative impact on patient outcomes. In this review, we discuss the most frequent respiratory conditions that patients may develop in the perioperative period. Their prevention and/or treatment may help to maximize the benefit these patients may derive from liver transplant. This review examines diagnostic and therapeutic approaches to these complications for hepatologists, surgeons, and critical care physicians.
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Affiliation(s)
- Filipe S Cardoso
- 1 Gastroenterology and Intensive Care Divisions, Hospital Curry Cabral, Central Lisbon Hospital Center, Nova Medical School, Nova University, Lisbon, Portugal
| | - Constantine J Karvellas
- 2 Division of Gastroenterology (Liver Unit) and Department of Critical Care Medicine, University of Alberta, Edmonton, Canada
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15
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Linecker M, Krones T, Berg T, Niemann CU, Steadman RH, Dutkowski P, Clavien PA, Busuttil RW, Truog RD, Petrowsky H. Potentially inappropriate liver transplantation in the era of the "sickest first" policy - A search for the upper limits. J Hepatol 2018; 68:798-813. [PMID: 29133246 DOI: 10.1016/j.jhep.2017.11.008] [Citation(s) in RCA: 84] [Impact Index Per Article: 12.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/01/2017] [Revised: 10/11/2017] [Accepted: 11/06/2017] [Indexed: 12/11/2022]
Abstract
Liver transplantation has emerged as a highly efficient treatment for a variety of acute and chronic liver diseases. However, organ shortage is becoming an increasing problem globally, limiting the applicability of liver transplantation. In addition, potential recipients are becoming sicker, thereby increasing the risk of losing the graft during transplantation or in the initial postoperative period after liver transplantation (three months). This trend is challenging the model for end-stage liver disease allocation system, where the sickest candidates are prioritised and no delisting criteria are given. The weighting of the deontological demand for "equity", trying to save every patient, regardless of the overall utility; and "efficiency", rooted in utilitarianism, trying to save as many patients as possible and increase the overall quality of life of patients facing the same problem, has to be reconsidered. In this article we are aiming to overcome the widespread concept of futility in liver transplantation, providing a definition of potentially inappropriate liver transplantation and giving guidance on situations where it is best not to proceed with liver transplantation, to decrease the mortality rate in the first three months after transplantation. We propose "absolute" and "relative" conditions, where early post-transplant mortality is highly probable, which are not usually captured in risk scores predicting post-transplant survival. Withholding liver transplantation for listed patients in cases where liver transplant is not deemed clearly futile, but is potentially inappropriate, is a far-reaching decision. Until now, this decision had to be discussed extensively on an individual basis, applying explicit communication and conflict resolution processes, since the model for end-stage liver disease score and most international allocation systems do not include explicit delisting criteria to support a fair delisting process. More work is needed to better identify cases where transplantation is potentially inappropriate and to integrate and discuss these delisting criteria in allocation systems, following a societal debate on what we owe to all liver transplant candidates.
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Affiliation(s)
- Michael Linecker
- Swiss HPB and Transplantation Center, University Hospital Zurich, Switzerland; Department of Surgery and Transplantation, University Hospital Zurich, Switzerland
| | - Tanja Krones
- Division of Clinical Ethics, University Hospital Zurich, Switzerland; Institute of Biomedical Ethics and History of Medicine, University of Zurich, Switzerland
| | - Thomas Berg
- Division of Hepatology, University of Leipzig, Germany
| | - Claus U Niemann
- Department of Anesthesiology, University of California, San Francisco, USA; Department of Surgery, University of California San Francisco, USA
| | - Randolph H Steadman
- Department of Anesthesiology and Perioperative Medicine, Ronald Reagan Medical Center, University of California Los Angeles, Los Angeles, USA
| | - Philipp Dutkowski
- Swiss HPB and Transplantation Center, University Hospital Zurich, Switzerland; Department of Surgery and Transplantation, University Hospital Zurich, Switzerland
| | - Pierre-Alain Clavien
- Swiss HPB and Transplantation Center, University Hospital Zurich, Switzerland; Department of Surgery and Transplantation, University Hospital Zurich, Switzerland
| | - Ronald W Busuttil
- Dumont-UCLA Transplant Center, Ronald Reagan Medical Center, University of California Los Angeles, USA
| | - Robert D Truog
- Center for Bioethics, Harvard Medical School, Boston, USA; Department of Anesthesia, Perioperative and Pain Medicine, Boston Children's Hospital, USA
| | - Henrik Petrowsky
- Swiss HPB and Transplantation Center, University Hospital Zurich, Switzerland; Department of Surgery and Transplantation, University Hospital Zurich, Switzerland.
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Ronot M, Pommier R, Calame P, Purcell Y, Vilgrain V. Computed Tomography. DIAGNOSTIC METHODS FOR CIRRHOSIS AND PORTAL HYPERTENSION 2018:183-210. [DOI: 10.1007/978-3-319-72628-1_13] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/05/2025]
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Dal Bosco A, Boeira Schedler F, Raskopf Colares J, Simões Dias A, Possa Marroni N. Hepatopulmonary Syndrome: Oxidative Stress and Physical Exercise. EUROPEAN MEDICAL JOURNAL 2017. [DOI: 10.33590/emj/10314105] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022] Open
Abstract
Hepatopulmonary syndrome (HPS) may be defined by hepatic disease, gas exchange abnormalities that may lead to hypoxaemia, and the presence of pulmonary vascular dilations. The balance between the many substances involved in vasodilation and vasoconstriction is regulated by the liver; thus, liver damage may generate systemic changes throughout the body. The pulmonary tissue may be damaged by reactive oxygen species or nitric oxide. Dyspnoea is the most frequent pulmonary symptom, caused by tissue damage, and may become worse when an individual exercises. In experimental research the surgical model of bile duct ligation is the optimal model to simulate the typical lung alterations present in HPS, which results in an increase in oxidative stress in hepatic and pulmonary tissues. In liver injury, the muscular system may also be damaged, for example sarcopenia may seriously aggravate cirrhosis and is associated with cirrhotic patient mortality. Muscular changes can be explained by the actions of myostatin and insulin-like growth factor and the increase in body levels of ammonia. As a result of impaired cardiopulmonary and muscular conditions, HPS patients may exhibit a low exercise tolerance, low muscle strength, and low functionality. Liver disease can contribute to HPS oxidative stress and is one of the main factors responsible for the reduction of gas exchange. Physical exercise can be performed as a way of modifying this pathophysiological state. Studies that have investigated physical exercise as a therapy for cirrhosis suggest that this approach may be beneficial for cirrhotic patients, primarily with regard to muscular and cardiorespiratory injuries.
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Affiliation(s)
- Adriane Dal Bosco
- Methodist University Center, Porto Alegre, Brazil; Laboratory of Experimental Hepatology and Gastroenterology, Hospital of Clinics of Porto Alegre, Porto Alegre, Brazil
| | - Filipe Boeira Schedler
- Laboratory of Experimental Hepatology and Gastroenterology, Hospital of Clinics of Porto Alegre, Porto Alegre, Brazil
| | - Josieli Raskopf Colares
- Postgraduate program in medical sciences, Federal University of Rio Grande do Sul, Porto Alegre, Brazil; Laboratory of Experimental Hepatology and Gastroenterology, Hospital of Clinics of Porto Alegre, Porto Alegre, Brazil
| | - Alexandre Simões Dias
- Laboratory of Experimental Hepatology and Gastroenterology, Hospital of Clinics of Porto Alegre, Porto Alegre, Brazil; Federal University of Rio Grande do Sul, Porto Alegre, Brazil
| | - Norma Possa Marroni
- Postgraduate program in medical sciences, Federal University of Rio Grande do Sul, Porto Alegre, Brazil; Postgraduate program in cellular and molecular biology applied to health, Lutheran University of Brazil, Canoas, Brazil; Laboratory of Experimental Hepatology and Gastroenterology, Hospital of Clinics of Porto Alegre, Porto Alegre, Brazil; Federal University of Rio Grande do Sul, Porto Alegre, Brazil
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CXCR2 is involved in pulmonary intravascular macrophage accumulation and angiogenesis in a rat model of hepatopulmonary syndrome. Clin Sci (Lond) 2016; 131:159-168. [DOI: 10.1042/cs20160593] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/03/2016] [Revised: 11/03/2016] [Accepted: 11/22/2016] [Indexed: 12/29/2022]
Abstract
Hepatopulmonary syndrome (HPS) is a lung complication in various liver diseases, with high incidence, poor prognosis and no effective non-surgical treatments in patients with hepatocirrhosis. Therefore, assessing HPS pathogenesis to explore proper therapy strategies is clinically relevant. In the present study, male Sprague–Dawley rats underwent sham operation or common bile duct ligation (CBDL). Two weeks post-surgery, the following groups were set up for 2 weeks of treatment: sham + normal saline, CBDL + CXCR2 antagonist SB225002, CBDL + tumour necrosis factor α (TNF-α) antagonist PTX and CBDL + normal saline groups. Liver and lung tissues were collected after mean arterial pressure (MAP) and portal venous pressure (PVP) measurements. Haematoxylin and eosin (H&E) staining (lung) and Masson staining (liver) were performed for pathological analyses. Finally, pulmonary tissue RNA and total protein were assessed for target effectors. The mRNA and protein levels of CXCR2 were significantly increased in the pulmonary tissue of CBDL rats. What's more, CXCR2 inhibition by SB225002 reduced the expression of CD68 and von Willebrand factor (vWf) in CBDL rats. Importantly, CXCR2 inhibition suppressed the activation of Akt and extracellular signal-regulated kinase (ERK) in CBDL rats. Antagonization of TNF-α with PTX down-regulated the expression of CXCR2. During HPS pathogenesis in rats, CXCR2 might be involved in the accumulation of pulmonary intravascular macrophages and angiogenesis, possibly by activating Akt and ERK, with additional regulation by TNF-α that enhanced pulmonary angiogenesis by directly acting on the pulmonary tissue. Finally, the present study may provide novel targets for the treatment of HPS.
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Abstract
Portopulmonary hypertension (PoPH) refers to the condition that pulmonary arterial hypertension (PAH) occur in the stetting of portal hypertension. The development of PoPH is thought to be independent of the severity of portal hypertension or the etiology or severity of liver disease. PoPH results from excessive vasoconstriction, vascular remodeling, and proliferative and thrombotic events within the pulmonary circulation that lead to progressive right ventricular failure and ultimately to death. Untreated PoPH is associated with a poor prognosis. As PoPH is frequently asymptomatic or symptoms are generally non-specific, patients should be actively screened for the presence of PoPH. Two-dimensional transthoracic echocardiography is a useful non-invasive screening tool, but a definitive diagnosis requires invasive hemodynamic confirmation by right heart catheterization. Despite a dearth of randomized, prospective data, an ever-expanding clinical experience shows that patients with PoPH benefit from therapy with PAH-specific medications including with endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and/or prostanoids. Due to high perioperative mortality, transplantation should be avoided in those patients who have severe PoPH that is refractory to medical therapy.
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Affiliation(s)
- Yong Lv
- a Department of Liver Diseases and Digestive Interventional Radiology , Xijing Hospital of Digestive Diseases, Fourth Military Medical University , Xi'an , China
| | - Guohong Han
- a Department of Liver Diseases and Digestive Interventional Radiology , Xijing Hospital of Digestive Diseases, Fourth Military Medical University , Xi'an , China
| | - Daiming Fan
- b State Key Laboratory of Cancer Biology & Xijing Hospital of Digestive Diseases , Fourth Military Medical University , Xi'an , China
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Goldberg DS, Fallon MB. The Art and Science of Diagnosing and Treating Lung and Heart Disease Secondary to Liver Disease. Clin Gastroenterol Hepatol 2015; 13:2118-27. [PMID: 25934564 PMCID: PMC4618073 DOI: 10.1016/j.cgh.2015.04.024] [Citation(s) in RCA: 27] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/06/2015] [Revised: 04/13/2015] [Accepted: 04/14/2015] [Indexed: 02/07/2023]
Abstract
Patients with chronic liver disease are at risk of extrahepatic complications related to cirrhosis and portal hypertension, as well as organ-specific complications of certain liver diseases. These complications can compromise quality of life, while also increasing morbidity and mortality before and after liver transplantation. Patients with chronic liver disease are at risk for pulmonary complications of hepatopulmonary syndrome and portopulmonary syndrome; the cardiac complication fall under the general concept of cirrhotic cardiomyopathy, which can affect systolic and diastolic function, as well as cardiac conduction. In addition, patients with certain diseases are at risk of lung and/or cardiac complications that are specific to the primary disease (ie, emphysema in α-1-antitrypsin deficiency) or occur with increased incidence in certain conditions (ie, ischemic heart disease associated with nonalcoholic steatohepatitis). This article focuses on the epidemiology, clinical presentation, pathogenesis, treatment options, and role of transplantation for lung and heart diseases secondary to liver disease, while also highlighting select liver diseases that directly affect the lungs and heart.
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Affiliation(s)
- David S Goldberg
- Division of Gastroenterology, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; Center for Clinical Epidemiology and Biostatistics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; Leonard Davis Institute, University of Pennsylvania, Philadelphia, Pennsylvania.
| | - Michael B Fallon
- Division of Gastroenterology, Hepatology, and Nutrition, Department of Internal Medicine, The University of Texas Health Science Center at Houston, Houston, Texas
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21
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Mechanical characteristics of the pulmonary artery in beagle dogs with hepatopulmonary syndrome and portopulmonary hypertension. Biomed Rep 2015; 4:51-54. [PMID: 26870333 DOI: 10.3892/br.2015.526] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/04/2015] [Accepted: 09/10/2015] [Indexed: 02/07/2023] Open
Abstract
The continuous changes in pulmonary hemodynamic properties in hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) have not been fully characterized in large animal models of HPS and PoPH. Beagle dog models of HPS and PoPH were induced by chronic common bile duct ligation and Sephadex microspheres, respectively. The model was validated by catheter examination and pathological analyses, and the hemodynamic characteristics of the models were observed. The results revealed that the cross-sectional area of the blood vessel was significantly increased in HPS models, but it was significantly decreased in the PoPH models. Furthermore, the resistance of pulmonary circulation was elevated in models of HPS, but it was decreased in models of PoPH. The present findings renew the traditional view that pulmonary hypertension is due to the enhanced peripheral resistance.
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Tsauo J, Weng N, Ma H, Jiang M, Zhao H, Li X. Role of Transjugular Intrahepatic Portosystemic Shunts in the Management of Hepatopulmonary Syndrome: A Systemic Literature Review. J Vasc Interv Radiol 2015; 26:1266-1271. [PMID: 26074026 DOI: 10.1016/j.jvir.2015.04.017] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/27/2015] [Revised: 04/20/2015] [Accepted: 04/21/2015] [Indexed: 02/05/2023] Open
Abstract
PURPOSE To evaluate the role of transjugular intrahepatic portosystemic shunt (TIPS) creation in the management of hepatopulmonary syndrome (HPS). MATERIALS AND METHODS A MEDLINE (PubMed) search from January 1990 to April 2015 was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The search was restricted to the English language and human subjects. Inclusion criteria were patients with HPS who underwent TIPS creation for any indication. Exclusion criteria was age < 18 years. RESULTS Ten studies consisting of 12 patients with HPS were included. Eight patients had very severe HPS, 2 had severe HPS, and 2 had moderate HPS. Transjugular intrahepatic portosystemic shunt creation was technically successful in all patients, without complications. Mean portosystemic pressure gradients before and after the procedure were 18.2 mm Hg (range, 10-30 mm Hg) and 6.5 mm Hg (range, 3-15 mm Hg), respectively. The mean duration of follow-up was 9.3 months (range, 0.75-36 mo). Improvement in oxygenation occurred in 9 patients but was not sustained after 4 months in 2 patients. In the remaining 3 patients, oxygenation remained unchanged; it worsened after 4 months in 1 patient. Four patients underwent liver transplantation. Two patients died of multiple organ dysfunction syndrome and 1 died of sepsis. The remaining patients were alive and well at the time of last follow-up. CONCLUSIONS Transjugular intrahepatic portosystemic shunt creation shows promise in the management of HPS. Future prospective studies are warranted.
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Affiliation(s)
- Jiaywei Tsauo
- Institute of Interventional Radiology, West China Hospital, Sichuan University, No. 37, Guoxue Lane, Chengdu 610041, Sichuan, China.; Department of Radiology, West China Hospital, Sichuan University, No. 37, Guoxue Lane, Chengdu 610041, Sichuan, China
| | - Ningna Weng
- Institute of Interventional Radiology, West China Hospital, Sichuan University, No. 37, Guoxue Lane, Chengdu 610041, Sichuan, China.; Department of Gastroenterology, West China Hospital, Sichuan University, No. 37, Guoxue Lane, Chengdu 610041, Sichuan, China
| | - Huaiyuan Ma
- Institute of Interventional Radiology, West China Hospital, Sichuan University, No. 37, Guoxue Lane, Chengdu 610041, Sichuan, China.; Department of Radiology, West China Hospital, Sichuan University, No. 37, Guoxue Lane, Chengdu 610041, Sichuan, China
| | - Mingshan Jiang
- Institute of Interventional Radiology, West China Hospital, Sichuan University, No. 37, Guoxue Lane, Chengdu 610041, Sichuan, China.; Department of Gastroenterology, West China Hospital, Sichuan University, No. 37, Guoxue Lane, Chengdu 610041, Sichuan, China
| | - He Zhao
- Institute of Interventional Radiology, West China Hospital, Sichuan University, No. 37, Guoxue Lane, Chengdu 610041, Sichuan, China.; Department of Gastroenterology, West China Hospital, Sichuan University, No. 37, Guoxue Lane, Chengdu 610041, Sichuan, China
| | - Xiao Li
- Institute of Interventional Radiology, West China Hospital, Sichuan University, No. 37, Guoxue Lane, Chengdu 610041, Sichuan, China..
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Cuadrado A, Díaz A, Iruzubieta P, Salcines JR, Crespo J. Síndrome hepatopulmonar. GASTROENTEROLOGIA Y HEPATOLOGIA 2015; 38:398-408. [DOI: 10.1016/j.gastrohep.2015.02.007] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 11/08/2014] [Revised: 02/01/2015] [Accepted: 02/08/2015] [Indexed: 12/17/2022]
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Velthuis S, Buscarini E, Gossage JR, Snijder RJ, Mager JJ, Post MC. Clinical implications of pulmonary shunting on saline contrast echocardiography. J Am Soc Echocardiogr 2015; 28:255-63. [PMID: 25623000 DOI: 10.1016/j.echo.2014.12.008] [Citation(s) in RCA: 47] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/29/2014] [Indexed: 02/07/2023]
Abstract
Pulmonary right-to-left shunting can be encountered using transthoracic contrast echocardiography (TTCE) with agitated saline. Diseases associated with pulmonary shunting on saline TTCE include hereditary hemorrhagic telangiectasia (HHT), hepatopulmonary syndrome, and some congenital heart defects after partial or complete cavopulmonary anastomosis. Furthermore, small pulmonary shunts on saline TTCE are also documented in a proportion of healthy individuals. Pulmonary shunting carries the risk for severe neurologic complications due to paradoxical embolization. In HHT, additional chest computed tomography is recommended in case of any pulmonary shunt detected on saline TTCE, to evaluate the feasibility for transcatheter embolotherapy of pulmonary arteriovenous malformations. Furthermore, antibiotic prophylaxis is advised in case of any pulmonary shunt on saline TTCE to prevent brain abscesses after procedures with risk for bacteremia. The present review provides an overview of important aspects of pulmonary shunting and its detection using saline TTCE. Furthermore, advances in understanding the clinical implications of different pulmonary shunt grades on saline TTCE are described. It appears that small pulmonary shunts on saline TTCE (grade 1) lack any clinical implication, as these shunts cannot be used as a diagnostic criterion for HHT, are not associated with an increased risk for neurologic complications, and represent pulmonary arteriovenous malformations too small for subsequent endovascular treatment. This implies that additional chest computed tomography could be safely withheld in all persons with only small pulmonary shunts on saline TTCE and sets the stage for further discussion about the need for antibiotic prophylaxis in these subjects. Besides further optimization of the current screening algorithm for the detection of pulmonary arteriovenous malformations in HHT, these observations can be of additional clinical importance in other diseases associated with pulmonary shunting and in those healthy individuals with documented small pulmonary shunts on saline TTCE.
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Affiliation(s)
- Sebastiaan Velthuis
- Department of Cardiology, St Antonius Hospital, Nieuwegein, The Netherlands.
| | | | - James R Gossage
- Department of Medicine, Georgia Regents University, Augusta, Georgia
| | - Repke J Snijder
- Department of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands
| | - Johannes J Mager
- Department of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands
| | - Martijn C Post
- Department of Cardiology, St Antonius Hospital, Nieuwegein, The Netherlands
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Zaky A, Bendjelid K. Appraising cardiac dysfunction in liver transplantation: an ongoing challenge. Liver Int 2015; 35:12-29. [PMID: 24797833 DOI: 10.1111/liv.12582] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/26/2013] [Accepted: 04/26/2014] [Indexed: 12/26/2022]
Abstract
End-stage liver disease (ESLD) is a multisystemic disease that adversely and mutually aggravates other organs such as the heart. Cardiac dysfunction in ESLD encompasses a spectrum of disease that could be aggravated, precipitated or be occurring hand-in-hand with coexisting aetiological factors precipitating cirrhosis. Additionally and more complexly, liver transplantation, the curative modality of ESLD, is responsible for additional intra- and postoperative short- and long-term cardiac morbidity. The phenotypic distinction of the different forms of cardiac dysfunction in ESLD albeit important prognostically and therapeutically is not allowed by the current societal recommendations, due to conceptual, and methodological limitations in the appraisal of cardiac function and structure in ESLD and in designing studies that are based on this appraisal. This review comprehensively discusses the spectrum of cardiac dysfunction in ESLD, discusses the limitations of the current appraisal of cardiac dysfunction in ESLD, and proposes a hypothetical approach for studying cardiac dysfunction in liver transplant candidates.
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Affiliation(s)
- Ahmed Zaky
- Department of Anesthesiology and Critical Care Medicine, University of Alabama at Birmingham, Birmingham, AL, USA
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Anaesthetic and Perioperative Management for Liver Transplantation. ABDOMINAL SOLID ORGAN TRANSPLANTATION 2015. [PMCID: PMC7124066 DOI: 10.1007/978-3-319-16997-2_9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
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Aldenkortt F, Aldenkortt M, Caviezel L, Waeber JL, Weber A, Schiffer E. Portopulmonary hypertension and hepatopulmonary syndrome. World J Gastroenterol 2014; 20:8072-8081. [PMID: 25009379 PMCID: PMC4081678 DOI: 10.3748/wjg.v20.i25.8072] [Citation(s) in RCA: 39] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/28/2013] [Accepted: 04/09/2014] [Indexed: 02/06/2023] Open
Abstract
Portopulmonary hypertension (POPH) and hepatopulmonary syndrome (HPS) are two frequent complications of liver disease, with prevalence among liver transplant candidates of 6% and 10%, respectively. Both conditions result from a lack of hepatic clearance of vasoactive substances produced in the splanchnic territory. Subsequently, these substances cause mainly pulmonary vascular remodeling and some degree of vasoconstriction in POPH with resulting elevated pulmonary pressure and right ventricular dysfunction. In HPS the vasoactive mediators cause intrapulmonary shunts with hypoxemia. Medical treatment is disappointing overall. Whereas liver transplantation (LT) results in the disappearance of HPS within six to twelve months, its effect on POPH is highly unpredictable. Modern strategies in managing HPS and POPH rely on a thorough screening and grading of the disease’s severity, in order to tailor the appropriate therapy and select only the patients who will benefit from LT. The anesthesiologist plays a central role in managing these high-risk patients. Indeed, the important hemodynamic and respiratory modifications of the perioperative period must be avoided through continuation of the preoperatively initiated drugs, appropriate intraoperative monitoring and proper hemodynamic and respiratory therapies.
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Intrapulmonary vascular dilatation evaluated by 99mTc-MAA scintigraphy and its association with portal hypertension in schistosomiasis. PLoS Negl Trop Dis 2014; 8:e2881. [PMID: 24967578 PMCID: PMC4072598 DOI: 10.1371/journal.pntd.0002881] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/21/2013] [Accepted: 04/07/2014] [Indexed: 12/14/2022] Open
Abstract
Background Portal hypertension is responsible for various complications in patients with schistosomiasis, among them intrapulmonary vascular dilations (IPVD). In cirrhotic patients the presence of IPVD is a sign of poor prognosis, but in patients with hepatosplenic schistosomiasis (HSS) there are no studies assessing the significance of this change. The aim of this study was to evaluate the occurrence of IPVD through 99mTc-MAA scintigraphy in patients with HSS and its relationship with clinical, laboratory, endoscopic and ultrasound parameters. Methods Cross-sectional study evaluating 51 patients with HSS. Patients were diagnosed with IPVD when the brain uptake of 99mTc-MAA was higher than 6%. Subsequently, they were divided according to presence (G1) or absence (G2) of IPVD and variables were compared between groups. Results Overall, 51 patients with mean age of 56±12 years were assessed. IPVD was observed in 31 patients (60%). There was no statistically significant differences between groups when clinical, laboratory and endoscopic parameters were compared. Regarding ultrasound parameters, the splenic vein diameter was smaller in G1 (0.9±0.3 cm) compared to G2 (1.2±0.4 cm), p = 0.029. Conclusion In patients with HSS, the occurrence of IPVD by 99mTc-MAA scintigraphy was high and was associated with lower splenic vein diameter, which can be a mechanism of vascular protection against portal hypertension. However, more studies are needed to determine the clinical significance of the early diagnosis and natural evolution of IPVD in this population. Intrapulmonary vascular dilatation (IPVD) is the key event in development of hepatopulmonary syndrome, an arterial oxygenation defect in patients with portal hypertension. IPVD diagnosis can be made by EchoDopplercardiography or 99mTc-macroaggregated albumin scintigraphy (99mTc-MAA), and ethiopatogeny is still unknown. In Northeastern Brazil, hepatosplenic schistossomiasis (HSS) is the main cause of portal hypertension. In cirrhotics, the presence of IPVD influences survival and candidacy for liver transplantation, however, in HSS patients, IPVD has been poorly studied, specially using lung perfusion scan with 99mTc-MAA. Some authors believe that IPVD is common in HSS and in the existence of differences in indirect portal hypertension parameters between patients with and without IPVD. All patients were distributed into two groups according to presence or not of IPVD, and laboratorial, endoscopy and ultrasound tests were perfomed. Occurrence of IPVD was high and was associated with lower splenic vein diameter, which can be a vascular protection mechanism against portal hypertension status.
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Abstract
Hepatopulmonary syndrome (HPS) is a pulmonary complication observed in patients with chronic liver disease and/or portal hypertension, attributable to an intrapulmonary vascular dilatation that induces severe hypoxaemia. Considering the favourable long-term survival of HPS patients as well as the reversal of the syndrome with a functional liver graft, HPS is now an indication for orthotopic liver transplantation (OLT). Consequently, blood gas analysis and imaging techniques should be performed when cirrhotic patients present with shortness of breath as well as when OLT candidates are placed on the transplant waiting list. If the arterial partial pressure of oxygen (PaO2) is more than 10.7 kPa when breathing room air, HPS can be excluded and no other investigation is needed. When the PaO2 when breathing room air is 10.7 kPa or less, contrast-enhanced echocardiography should be performed to exclude pulmonary vascular dilatation. Lung function tests may also help detect additional pulmonary diseases that can contribute to impaired oxygenation. When contrast-enhanced echocardiography is negative, HPS is excluded and no follow-up is needed. When contrast-enhanced echocardiography is positive and PaO2 less than 8 kPa, patients should obtain a severity score that provides them with a reasonable probability of being transplanted within 3 months. In mild-to-moderate HPS (PaO2 8 to 10.6 kPa), periodic follow-up is recommended every 3 months to detect any further deterioration in PaO2. Although no intraoperative deaths have been directly attributed to HPS, oxygenation may worsen immediately following OLT due to volume overload and postoperative infections. Mechanical ventilation is often prolonged with an extended stay in the ICU. A high postoperative mortality (mostly within 6 months) is observed in this group of patients in comparison to non-HPS patients. However, the recovery of an adequate PaO2 within 12 months after OLT explains the similar outcome of HPS and non-HPS patients following OLT over a longer time period.
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Machicao VI, Balakrishnan M, Fallon MB. Pulmonary complications in chronic liver disease. Hepatology 2014; 59:1627-37. [PMID: 24089295 DOI: 10.1002/hep.26745] [Citation(s) in RCA: 123] [Impact Index Per Article: 11.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/15/2013] [Accepted: 09/12/2013] [Indexed: 12/13/2022]
Abstract
The association of chronic liver disease with respiratory symptoms and hypoxia is well recognized. Over the last century, three pulmonary complications specific to chronic liver disease have been characterized: hepatopulmonary syndrome (HPS), portopulmonary hypertension (POPH), and hepatic hydrothorax (HH). The development of portal hypertension is fundamental in the pathogenesis of each of these disorders. HPS is the most common condition, found in 5%-30% of cirrhosis patients, manifested by abnormal oxygenation due to the development of intrapulmonary vascular dilatations. The presence of HPS increases mortality and impairs quality of life, but is reversible with liver transplantation (LT). POPH is characterized by development of pulmonary arterial hypertension in the setting of portal hypertension, and is present in 5%-10% of cirrhosis patients evaluated for LT. Screening for POPH in cirrhosis patients eligible for LT is critical since severe POPH is a relative contraindication for LT. Patients with moderate POPH, who respond adequately to medical therapy, may benefit from LT, although sufficient controlled data are lacking. HH is a transudative pleural effusion seen in 5%-10% of cirrhosis patients, in the absence of cardiopulmonary disease. Diagnosis of HH should prompt consideration for LT, which is the ultimate treatment for HH. Conservative management includes salt restriction and diuretics, with thoracentesis and transjugular intrahepatic portosystemic shunt (TIPS) as second-line therapeutic options.
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Affiliation(s)
- Victor I Machicao
- Division of Gastroenterology, Hepatology and Nutrition, Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX
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Fouad YM, Yehia R. Hepato-cardiac disorders. World J Hepatol 2014; 6:41-54. [PMID: 24653793 PMCID: PMC3953805 DOI: 10.4254/wjh.v6.i1.41] [Citation(s) in RCA: 80] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/22/2013] [Revised: 11/19/2013] [Accepted: 01/06/2014] [Indexed: 02/06/2023] Open
Abstract
Understanding the mutual relationship between the liver and the heart is important for both hepatologists and cardiologists. Hepato-cardiac diseases can be classified into heart diseases affecting the liver, liver diseases affecting the heart, and conditions affecting the heart and the liver at the same time. Differential diagnoses of liver injury are extremely important in a cardiologist's clinical practice calling for collaboration between cardiologists and hepatologists due to the many other diseases that can affect the liver and mimic haemodynamic injury. Acute and chronic heart failure may lead to acute ischemic hepatitis or chronic congestive hepatopathy. Treatment in these cases should be directed to the primary heart disease. In patients with advanced liver disease, cirrhotic cardiomyopathy may develop including hemodynamic changes, diastolic and systolic dysfunctions, reduced cardiac performance and electrophysiological abnormalities. Cardiac evaluation is important for patients with liver diseases especially before and after liver transplantation. Liver transplantation may lead to the improvement of all cardiac changes and the reversal of cirrhotic cardiomyopathy. There are systemic diseases that may affect both the liver and the heart concomitantly including congenital, metabolic and inflammatory diseases as well as alcoholism. This review highlights these hepatocardiac diseases.
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Affiliation(s)
- Yasser Mahrous Fouad
- Yasser Mahrous Fouad, Reem Yehia, Gastroenterology and Hepatology Unit, Endemic Medicine Department, Minia University, Minia 19111, Egypt
| | - Reem Yehia
- Yasser Mahrous Fouad, Reem Yehia, Gastroenterology and Hepatology Unit, Endemic Medicine Department, Minia University, Minia 19111, Egypt
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Saigal S, Choudhary N, Saraf N, Kotecha H, Kakodkar R, Mohanka R, Rastogi A, Menon P, Goja S, Govil D, Vohra V, Soin A. Excellent outcome of living donor liver transplantation in patients with hepatopulmonary syndrome: a single centre experience. Clin Transplant 2013; 27:530-4. [PMID: 23721501 DOI: 10.1111/ctr.12126] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/20/2012] [Indexed: 12/15/2022]
Affiliation(s)
- Sanjiv Saigal
- Medanta the Medicity; Institute of Liver Transplantation and Regenerative Medicine
| | - Narendra Choudhary
- Medanta the Medicity; Institute of Liver Transplantation and Regenerative Medicine
| | - Neeraj Saraf
- Medanta the Medicity; Institute of Liver Transplantation and Regenerative Medicine
| | - Hardik Kotecha
- Medanta the Medicity; Institute of Liver Transplantation and Regenerative Medicine
| | - Rahul Kakodkar
- Medanta the Medicity; Institute of Liver Transplantation and Regenerative Medicine
| | - Ravi Mohanka
- Medanta the Medicity; Institute of Liver Transplantation and Regenerative Medicine
| | - Amit Rastogi
- Medanta the Medicity; Institute of Liver Transplantation and Regenerative Medicine
| | - Palat Menon
- Medanta the Medicity; Institute of Liver Transplantation and Regenerative Medicine
| | - Sanjay Goja
- Medanta the Medicity; Institute of Liver Transplantation and Regenerative Medicine
| | - Deepak Govil
- Medanta the Medicity; Institute of critical care
| | - Vijay Vohra
- Medanta the Medicity; Liver transplant anesthesia; Gurgaon; Haryana; India
| | - Arvinder Soin
- Medanta the Medicity; Institute of Liver Transplantation and Regenerative Medicine
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Porres-Aguilar M, Gallegos-Orozco JF, Garcia H, Aguirre J, Macias-Rodriguez RU, Torre-Delgadillo A. Pulmonary vascular complications in portal hypertension and liver disease: a concise review. REVISTA DE GASTROENTEROLOGÍA DE MÉXICO 2013; 78:35-44. [PMID: 23369639 DOI: 10.1016/j.rgmx.2012.10.004] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 08/18/2012] [Accepted: 10/19/2012] [Indexed: 11/30/2022]
Abstract
Chronic liver disease and/or portal hypertension may be associated with one of the two pulmonary vascular complications: portopulmonary hypertension and hepatopulmonary syndrome. These pulmonary vascular disorders are notoriously underdiagnosed; however, they have a substantial negative impact on survival and require special attention in order to understand their diagnostic approach and to select the best therapeutic options. Portopulmonary hypertension results from excessive vasoconstriction, vascular remodeling, and proliferative and thrombotic events within the pulmonary circulation that lead to progressive right ventricular failure and ultimately to death. On the other hand, abnormal intrapulmonary vascular dilations, profound hypoxemia, and a wide alveolar-arterial gradient are the hallmarks of the hepatopulmonary syndrome, resulting in difficult-to-treat hypoxemia. The aim of this review is to summarize the latest pathophysiologic concepts, diagnostic approach, therapy, and prognosis of portopulmonary hypertension and hepatopulmonary syndrome, as well as to discuss the role of liver transplantation as a definitive therapy in selected patients with these conditions.
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Affiliation(s)
- M Porres-Aguilar
- Department of Internal Medicine, Division of Hospital Medicine, Texas Tech University Health Sciences Center/Paul L. Foster School of Medicine, El Paso, TX, USA.
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Kianifar HR, Khalesi M, Mahmoodi E, Afzal Aghaei M. Pentoxifylline in hepatopulmonary syndrome. World J Gastroenterol 2012; 18:4912-6. [PMID: 23002364 PMCID: PMC3447274 DOI: 10.3748/wjg.v18.i35.4912] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/27/2011] [Revised: 05/10/2012] [Accepted: 06/08/2012] [Indexed: 02/06/2023] Open
Abstract
AIM: To determine the effects of pentoxifylline (PTX) on clinical manifestations and evaluate arterial blood gas data in hepatopulmonary syndrome (HPS) in children.
METHODS: In a pilot study of 10 children with chronic liver disease, who had HPS, 20 mg/kg/d PTX was administered for 3 mo. Clinical data and arterial blood gas parameters were evaluated at baseline, the end of the treatment period, and 3 mo after drug discontinuation.
RESULTS: Six patients could tolerate PTX, while four patients experienced complications. Among patients who could tolerate PTX, there was a significant increase in arterial oxygen pressure (PaO2) (P = 0.02) and oxygen saturation (SaO2) (P = 0.04) and alveolar-arterial oxygen gradient (P = 0.02) after 3 mo of treatment. Significant decreases in PaO2 (P = 0.02) and alveolar-arterial oxygen gradient (P = 0.02) were also seen after drug discontinuation.
CONCLUSION: PTX may improve PaO2, SaO2 and alveolar-arterial oxygen gradient in the early stage of HPS.
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Abstract
The pulmonary involvement concurrent with gastrointestinal (GI) diseases is often clinically subtle. Radiological manifestations might lag behind the respiratory compromise, and only such specialized testing as high resolution computed tomography (HRCT), permeability studies with labelled proteins, or comprehensive pulmonary function tests (PFTs) may be sensitive enough to detect the evolving pathophysiology. Increasing recognition of specific entities, such as immune-mediated alveolitis, will allow implementation of therapies that can significantly improve a patient's prognosis.
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Huo YM, Hua R, Chen W, Sun YW. Clinical study on pulmonary diffusion function in patients with chronic liver disease. J Dig Dis 2010; 11:291-8. [PMID: 20883425 DOI: 10.1111/j.1751-2980.2010.00452.x] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
OBJECTIVE To investigate the influence of chronic liver disease (CLD) on pulmonary diffusion function. METHODS Arterial blood gas analysis, pulmonary function test, contrast-enhanced transthoracic echocardiography and technetium macro-aggregated albumin scanning were performed in 50 cirrhotic patients who underwent surgery on portal hypertension and liver transplantation. The severity of chronic liver disease (CLD) was evaluated by Child-Pugh-Turcotte (CPT) categorization and model for end stage liver disease (MELD) score from October 2008 to July 2009 in our surgical department and organ transplantation center. RESULTS A-aDO(2) was increased with the aggravation of liver dysfunction. The pulmonary diffusion capacity for carbon monoxide (DLCO) differed significantly among the three groups, which was (90.8 ± 7.3)% in CPT A group, (82.8 ± 10.8)% in CPT B group, and (73.5 ± 8.3)% in CPT C group. A-aDO(2) correlated positively with CPT (r= 0.581, P= 0.000) as well as the MELD score (r= 0.696, P= 0.000), whereas DLCO was negatively correlated with CPT (r=-0.630, P= 0.000) and the MELD score (r=-0.708, P= 0.000). CONCLUSION DLCO can be useful in the detection of pulmonary vascular abnormality of CLD. The MELD score may be a better criterion than the CPT score in assessing intrapulmonary vascular damage of CLD patients.
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Affiliation(s)
- Yan Miao Huo
- Department of General Surgery, Renji Hospital, Shanghai Jiaotong University School of Medicine, China
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Gonzalez LF, Leal D, Vidal CA, Zuluaga D, Ariza F, Giraldo C. Síndrome hepatopulmonar y trasplante hepático. COLOMBIAN JOURNAL OF ANESTHESIOLOGY 2009. [DOI: 10.1016/s0120-3347(09)74003-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022] Open
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Minemura M, Tajiri K, Shimizu Y. Systemic abnormalities in liver disease. World J Gastroenterol 2009; 15:2960-2974. [PMID: 19554648 PMCID: PMC2702103 DOI: 10.3748/wjg.15.2960] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/09/2009] [Revised: 05/23/2009] [Accepted: 05/30/2009] [Indexed: 02/06/2023] Open
Abstract
Systemic abnormalities often occur in patients with liver disease. In particular, cardiopulmonary or renal diseases accompanied by advanced liver disease can be serious and may determine the quality of life and prognosis of patients. Therefore, both hepatologists and non-hepatologists should pay attention to such abnormalities in the management of patients with liver diseases.
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Gonzalez LF, Leal D, Vidal CA, Zuluaga D, Ariza F, Giraldo C. Síndrome hepatopulmonar y trasplante hepático. COLOMBIAN JOURNAL OF ANESTHESIOLOGY 2008. [DOI: 10.1016/s0120-3347(08)64009-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022] Open
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Liver transplantation for pulmonary vascular complications of pediatric end-stage liver disease. J Pediatr Surg 2008; 43:1813-20. [PMID: 18926213 DOI: 10.1016/j.jpedsurg.2008.04.002] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/26/2007] [Revised: 03/31/2008] [Accepted: 04/01/2008] [Indexed: 11/20/2022]
Abstract
PURPOSE Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPH) are poorly understood pulmonary complications of end-stage liver disease (ESLD). We present a case series of children with HPS and PPH. METHODS After institutional review board approval, query of our medical database identified children 0 to 18 years of age with ESLD diagnosed with HPS or PPH. Data were collected via chart review. RESULTS We identified 7 children with either HPS (n = 5) or PPH (n = 2). Patients with HPS presented with progressive dyspnea over a mean of 7 months (range, 4-12 months) at a mean of 13 years (range, 5-17 years) of age. Pulmonary shunting by albumin perfusion scan averaged 41% (range, 20%-66%) with an initial mean resting SpO(2) of 88% (range, 84%-94%) and mean SpO(2) during exertion of 79% (range, 60%-89%). Four patients required supplemental O(2) and, upon United Network for Organ Sharing (UNOS) appeal, received pediatric model for ESLD (or Child-Pugh) score exceptions, enabling them to undergo orthotopic liver transplant (OLT) within 1-2 months. The fifth patient was initially rejected by the UNOS regional review board, but 6 months of worsening hypoxemia led to OLT 2 months after successful UNOS appeal. All patients with HPS undergoing OLT experienced complete resolution of hypoxemia within 8 months. Both children with PPH were treated with intravenous epoprostenol, which lowered or stabilized mean pulmonary artery pressure and bridged them to OLT within 7 months of listing. Overall, there were no pulmonary complications; however, 1 patient with PPH expired shortly after OLT. The remaining patients are alive at a median follow-up of 27 months (range, 6-96 months). CONCLUSION Hepatopulmonary syndrome and PPH are uncommon complications of ESLD in children. Epoprostenol can bridge PPH patients to OLT. OLT leads to rapid resolution of HPS and PPH and currently represents the only successful treatment for these children.
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Ferreira PP, Camara EJN, Paula RLPD, Zollinger CC, Cavalcanti AR, Bittencourt PL. Prevalence of hepatopulmonary syndrome in patients with decompensated chronic liver disease and its impact on short-term survival. ARQUIVOS DE GASTROENTEROLOGIA 2008; 45:34-7. [DOI: 10.1590/s0004-28032008000100007] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/04/2007] [Accepted: 08/15/2007] [Indexed: 01/28/2023]
Abstract
BACKGROUND: Hepatopulmonary syndrome is reported to occur in 4% to 32% of the patients with chronic liver disease and is associated with poor liver function and shortened patient survival before and after liver transplantation. AIMS: To assess the frequency of hepatopulmonary syndrome in Brazilian patients with decompensated chronic liver disease and to investigate its impact on patient survival. METHODS: One hundred and thirty patients (101 males, mean age 61 ± 12 years) with decompensated chronic liver disease were evaluated for the presence of hepatopulmonary syndrome. The diagnosis of hepatopulmonary syndrome was considered in the presence of alveolar arterial oxygen gradient of more than 15 mm Hg and of pulmonary vascular dilatation assessed by contrast enhanced echocardiography. RESULTS: Hepatopulmonary syndrome was observed in 21 (16%) patients. The presence of hepatopulmonary syndrome was significantly associated with severity of liver disease assessed by the MELD (Model for End-Stage Liver Disease) score, but not with in hospital mortality after admission due to decompensated chronic liver disease. CONCLUSIONS: Hepatopulmonary syndrome occurs in 16% of patients with chronic liver disease and is associated with disease severity according to the MELD score. Short term mortality following decompensation of chronic liver disease was not associated with hepatopulmonary syndrome.
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Sakai T, Planinsic RM, Mathier MA, de Vera ME, Venkataramanan R. Initial experience using continuous intravenous treprostinil to manage pulmonary arterial hypertension in patients with end-stage liver disease. Transpl Int 2008; 22:554-61. [PMID: 19175541 DOI: 10.1111/j.1432-2277.2008.00830.x] [Citation(s) in RCA: 47] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
Abstract
Treprostinil is a prostacyclin analog and has been used on idiopathic pulmonary arterial hypertension (PAH). There is only limited clinical experience using treprostinil to manage PAH in patients with end-stage liver disease (ESLD). We report three ESLD patients with PAH, who were treated with continuous intravenous treprostinil. A 59-year-old woman with ESLD secondary to alcoholic hepatitis had portopulmonary hypertension with mean pulmonary arterial pressure (mPAP) of 44 mmHg and transpulmonary gradient (TPG) of 23 mmHg. Treprostinil at 45 ng/kg/min for 6 months decreased mPAP to 23 (TPG to 8). A 53-year-old man had ESLD secondary to alcoholic hepatitis with PAH caused by multiple pulmonary embolisms (mPAP of 32 and TPG of 23). Treprostinil at 36 ng/kg/min for 3 months decreased mPAP to 23 and TPG to 14. Both patients underwent uneventful liver transplantation. A 48-year-old man had ESLD secondary to hepatitis C and portopulmonary hypertension with mPAP of 60 and TPG of 44. Two years after intravenous treprostinil at 106 ng/kg/min, his mPAP decreased to 44 and TPG to 30. These results demonstrate that for a selected group of ESLD patients with PAH, a continuous intravenous infusion of treprostinil appears to be safe and effective.
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Affiliation(s)
- Tetsuro Sakai
- Department of Anesthesiology, University of Pittsburgh Medical Center, Pittsburgh, PA 15213, USA
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Pastor CM, Schiffer E. Therapy Insight: hepatopulmonary syndrome and orthotopic liver transplantation. ACTA ACUST UNITED AC 2007; 4:614-21. [PMID: 17978818 DOI: 10.1038/ncpgasthep0965] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/09/2007] [Accepted: 08/23/2007] [Indexed: 02/06/2023]
Abstract
Hepatopulmonary syndrome (HPS)--a pulmonary complication observed in patients who have chronic liver disease and/or portal hypertension--is attributed to intrapulmonary vascular dilatation and induces severe hypoxemia. HPS is mainly detected when patients are included on the waiting list for orthotopic liver transplantation (OLT) and can be diagnosed by blood gas analysis, transthoracic contrast-enhanced echocardiography or body scan with (99m)Tc-labeled macroaggregated albumin perfusion. When the partial pressure of arterial oxygen (PaO(2)) is >or=80 mmHg, it is unlikely that the patient has HPS. When the PaO(2) is <80 mmHg, imaging techniques should be used to confirm or exclude pulmonary vascular dilatation. When a diagnosis of HPS is confirmed, knowing the degree of hypoxemia is crucial for optimum patient management. Patients who have a PaO(2) >or=50 mmHg but <60 mmHg should be prioritized for OLT. This procedure is not indicated for patients with a PaO(2) between 60 mmHg and 80 mmHg, although follow-up every 3 months is recommended to detect any deterioration of the PaO(2). A PaO(2) of <50 mmHg might preclude OLT, because mortality and morbidity after OLT are greatly increased in these patients.
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Affiliation(s)
- Catherine M Pastor
- Laboratoire de Physiopathologie Hépatique et Imagerie Moléculaire, Hôpitaux Universitaires de Genève, 1205 Geneva, Switzerland.
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46
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Reichen J. Signs and Symptoms of Liver Disease. TEXTBOOK OF HEPATOLOGY 2007:441-450. [DOI: 10.1002/9780470691861.ch5a] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Fix OK, Bass NM, De Marco T, Merriman RB. Long-term follow-up of portopulmonary hypertension: effect of treatment with epoprostenol. Liver Transpl 2007; 13:875-85. [PMID: 17539008 DOI: 10.1002/lt.21174] [Citation(s) in RCA: 101] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
Moderate to severe portopulmonary hypertension (PPHTN) increases the risks of orthotopic liver transplantation (OLT). Epoprostenol is an effective treatment of PPHTN, but long-term effects on pulmonary hemodynamics or liver function in PPHTN are poorly defined. We sought to describe the long-term effects of treatment with or without epoprostenol on pulmonary hemodynamics, liver biochemistries, and survival in patients with moderate to severe PPHTN at a single center. A large retrospective cohort was identified with moderate to severe PPHTN diagnosed before OLT. Baseline and follow-up pulmonary hemodynamics and liver biochemistries were compared and outcomes assessed. Nineteen patients were treated with epoprostenol and 17 were not treated with epoprostenol. There were significant improvements in mean pulmonary artery pressure (MPAP, 48.4-36.1 mm Hg; P < 0.0001), pulmonary vascular resistance (PVR, 632-282 dynes . s . cm(-5); P < 0.0001), and cardiac output (5.7 to 7.7 L/min; P = 0.0009) with epoprostenol after a median of 15.4 months. Liver biochemistries did not change significantly, and survival did not seem to differ between the 2 groups (hazard ratio, 0.85; P = 0.77). In the epoprostenol group, patients who survived had greater absolute changes in MPAP, transpulmonary gradient, and PVR than those who died. Two patients in the epoprostenol group successfully underwent OLT. Long-term epoprostenol therapy greatly improves pulmonary hemodynamics in patients with PPHTN. Liver biochemistries are not greatly changed. Survival seemed not to differ between treatment groups. A minority of patients treated with epoprostenol will improve sufficiently to undergo OLT.
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Affiliation(s)
- Oren K Fix
- Division of Gastroenterology, Department of Medicine, University of California, San Francisco, San Francisco, CA, USA.
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Ashfaq M, Chinnakotla S, Rogers L, Ausloos K, Saadeh S, Klintmalm GB, Ramsay M, Davis GL. The impact of treatment of portopulmonary hypertension on survival following liver transplantation. Am J Transplant 2007; 7:1258-64. [PMID: 17286619 DOI: 10.1111/j.1600-6143.2006.01701.x] [Citation(s) in RCA: 112] [Impact Index Per Article: 6.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/25/2023]
Abstract
Pulmonary hypertension in the setting of cirrhosis and portal hypertension is known as portopulmonary hypertension (PPHTN). Moderate or severe PPHTN is uncommon, but has a poor prognosis and is considered to be a contraindication to liver transplantation. We assessed the impact of vasodilation therapy on pulmonary hemodynamics and outcome after liver transplant in these patients. Eighty-six patients evaluated for liver transplant between 1997 and 2005 had an estimated right ventricular systolic pressure >40 mm Hg or a clinical suspicion of PPHTN. Right heart catheterization confirmed PPHTN in 30 patients (ten mild, eight moderate, and 12 severe). Sixteen of the 20 with moderate-to-severe pulmonary hypertension (mPAP >or= 35) were otherwise considered suitable liver transplant candidates and were treated with vasodilation therapy. mPAP fell to less than 35 mm Hg in 12 patients (75%) and 11 of them then underwent orthotopic liver transplantation. One- and five-year survivals in the transplanted patients were 91% and 67%, respectively. Nine of 11 were off vasodilator therapy after a median of 9.2 months following transplantation. None of the patients who failed vasodilator therapy survived (median survival, 8 months). Effective pharmacologic control of PPHTN before liver transplant is associated with excellent posttransplant survival that is similar to patients transplanted for other indications.
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Affiliation(s)
- M Ashfaq
- Department of Medicine, Baylor Regional Transplant Institute, Baylor University Medical Center, Dallas, TX, USA
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49
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Møller S, Krag A, Henriksen JH, Bendtsen F. Pathophysiological aspects of pulmonary complications of cirrhosis. Scand J Gastroenterol 2007; 42:419-27. [PMID: 17454850 DOI: 10.1080/00365520601151695] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Affiliation(s)
- Søren Møller
- Department of Clinical Physiology 239, Hvidovre Hospital, DK-2650 Hvidovre, Denmark.
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50
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Schiffer E, Majno P, Mentha G, Giostra E, Burri H, Klopfenstein CE, Beaussier M, Morel P, Hadengue A, Pastor CM. Hepatopulmonary syndrome increases the postoperative mortality rate following liver transplantation: a prospective study in 90 patients. Am J Transplant 2006; 6:1430-7. [PMID: 16686767 DOI: 10.1111/j.1600-6143.2006.01334.x] [Citation(s) in RCA: 90] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/25/2023]
Abstract
Hepatopulmonary syndrome (HPS) is a frequent pulmonary complication of patients with end-stage liver diseases. HPS is diagnosed by hypoxemia and pulmonary vascular dilatation and is an independent risk factor of mortality. Orthotopic liver transplantation (OLT) is the only factor that modifies the natural course of HPS. Once patients with HPS have been transplanted, their long-term survival rate is similar to transplanted patients without HPS. Consequently, HPS is an indication of OLT whatever the severity of hypoxemia. However, besides the favorable long-term survival of HPS patients with OLT, a high postoperative mortality (mostly within 6 months) has been suggested. The aim of our study was to analyze the incidence of HPS and postoperative outcome after OLT in 90 consecutive patients. All patients were prospectively included and had blood gas analysis to detect HPS. Patients with hypoxemia had contrast echocardiography to confirm HPS. Nine patients had HPS with a 50 </= PaO(2)</= 70 mmHg. Among them 3 (33%) died while the mortality rate was 9.2% in the group without HPS (7 over 76 patients). In the HPS patients who survived, the syndrome completely recovered within 6 months. In conclusion, our study shows a high postoperative mortality rate following OLT even though the preoperative PaO(2) was >50 mmHg in all HPS patients transplanted.
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Affiliation(s)
- E Schiffer
- Service d'Anesthésiologie, Département APSI, Hopitaux Universitaires de Geneve, 1205 Geneva, Switzerland.
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