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Sayadi A, Duhaut L, Robert F, Savale L, Coilly A. [Hepatopulmonary syndrome]. Rev Med Interne 2024; 45:156-165. [PMID: 37005097 DOI: 10.1016/j.revmed.2023.03.008] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/10/2022] [Revised: 03/07/2023] [Accepted: 03/17/2023] [Indexed: 04/03/2023]
Abstract
The hepatopulmonary syndrome (HPS) is one of the lung diseases associated with cirrhosis and portal hypertension. It should be discussed for any dyspnea in cirrhotic patients. HPS is a pulmonary vascular disease characterized by intrapulmonary vascular dilatations (IPVD). The pathogenesis is complex and seems to rely on communications between the portal and pulmonary circulations. The diagnosis is based on a triad of liver disease and portal hypertension, evidence of IPVDs, and impaired gas exchange (alveolar-arterial oxygen difference [A-aO2]≥15mmHg). HPS impairs prognosis (23% survival at 5years) and patients' quality of life. Liver transplantation (LT) allows regression of IPDVD in almost 100% of cases, normalization of gas exchange and improves survival with a 5-year post-LT survival between 76 and 87%. It is the only curative treatment, indicated in patients with severe HPS, defined by an arterial partial pressure of oxygen (PaO2) below 60mmHg. When LT is not indicated or feasible, long-term oxygen therapy may be proposed as a palliative treatment. A better understanding of the pathophysiological mechanisms is needed to improve the therapeutic possibilities in a near future.
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Affiliation(s)
- A Sayadi
- UMR-S 1193, hôpital Paul-Brousse, centre hépato-biliaire, université Paris-Saclay, AP-HP, 94800 Villejuif, France
| | - L Duhaut
- UMR-S 1193, hôpital Paul-Brousse, centre hépato-biliaire, université Paris-Saclay, AP-HP, 94800 Villejuif, France
| | - F Robert
- Inserm UMR_S 999, 94270 Le Kremlin-Bicêtre, France
| | - L Savale
- Inserm UMR_S 999, 94270 Le Kremlin-Bicêtre, France; Service de pneumologie, hôpital Bicêtre, université Paris-Saclay, AP-HP, 94270 Le Kremlin-Bicêtre, France
| | - A Coilly
- UMR-S 1193, hôpital Paul-Brousse, centre hépato-biliaire, université Paris-Saclay, AP-HP, 94800 Villejuif, France.
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2
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Koc ÖM, Aslan D, Kramer M, Verbeek J, Van Malenstein H, van der Merwe S, Monbaliu D, Vos R, Verleden GM, Pirenne J, Nevens F. Outcomes of liver transplantation for hepatopulmonary syndrome in patients with concomitant respiratory disease. Clin Transplant 2024; 38:e15171. [PMID: 37897208 DOI: 10.1111/ctr.15171] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/12/2023] [Revised: 09/05/2023] [Accepted: 10/17/2023] [Indexed: 10/29/2023]
Abstract
BACKGROUND & AIMS Concomitant respiratory disease is a common finding in patients with hepatopulmonary syndrome (HPS). Among patients who underwent liver transplantation (LT) for HPS, we compared characteristics and outcome of patients with versus without concomitant respiratory disease. METHODS This single center retrospective observational study included patients with HPS who underwent LT between 1999 and 2020. RESULTS During the study period, 32 patients with HPS received a LT; nine (28%) with concomitant respiratory disease of whom one required a combined lung-liver transplantation. Patients with concomitant respiratory disease had higher PaCO2 (38 vs. 33 mm Hg, p = .031). The 30-day postoperative mortality was comparable, but the estimated cumulative probability of resolution of oxygen therapy after LT in HPS patients with versus those without concomitant respiratory disease was lower: 63% versus 91% at 12 months and 63% versus 100% at 18 months (HR 95% CI .140-.995, p = .040). In addition to the presence of concomitant respiratory disease (p = .040), history of smoking (p = .012), and high baseline 99mTcMAA shunt fraction (≥20%) (p = .050) were significantly associated with persistent need of oxygen therapy. The 5-year estimated cumulative probability of mortality in patients with concomitant respiratory disease was worse: 50% versus 23% (HR 95% CI .416-6.867, p = .463). CONCLUSIONS The presence of a concomitant respiratory disease did not increase the short-term postoperative mortality after LT in patients with HPS. However, it resulted in a longer need for oxygen therapy.
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Affiliation(s)
- Özgür M Koc
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Maastricht University Medical Center, Maastricht, The Netherlands
- Faculty of Medicine and Life Sciences, Hasselt University, Hasselt, Belgium
| | - Devrim Aslan
- Department of Gastroenterology and Hepatology, University Hospitals KU Leuven, Leuven, Belgium
| | - Matthijs Kramer
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Maastricht University Medical Center, Maastricht, The Netherlands
| | - Jef Verbeek
- Department of Gastroenterology and Hepatology, University Hospitals KU Leuven, Leuven, Belgium
| | - Hannah Van Malenstein
- Department of Gastroenterology and Hepatology, University Hospitals KU Leuven, Leuven, Belgium
| | - Schalk van der Merwe
- Department of Gastroenterology and Hepatology, University Hospitals KU Leuven, Leuven, Belgium
| | - Diethard Monbaliu
- Department of Abdominal Transplantation Surgery, University Hospitals KU Leuven, Leuven, Belgium
| | - Robin Vos
- Department of Respiratory Diseases, University Hospitals KU Leuven, Leuven, Belgium
| | - Geert M Verleden
- Department of Respiratory Diseases, University Hospitals KU Leuven, Leuven, Belgium
| | - Jacques Pirenne
- Department of Abdominal Transplantation Surgery, University Hospitals KU Leuven, Leuven, Belgium
| | - Frederik Nevens
- Department of Gastroenterology and Hepatology, University Hospitals KU Leuven, Leuven, Belgium
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Xu EJ, Zarm AM, Lecker SH, Hoenig MP. Acid Base Disorders in Cirrhosis. ADVANCES IN KIDNEY DISEASE AND HEALTH 2023; 30:336-342. [PMID: 37657880 DOI: 10.1053/j.akdh.2023.04.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/12/2022] [Revised: 02/14/2023] [Accepted: 04/14/2023] [Indexed: 09/03/2023]
Abstract
Metabolic and respiratory acid-base disorders are common in individuals with liver disease and cirrhosis. The most common disorder is respiratory alkalosis, which may be related to dyspnea or respiratory stimulation. Primary metabolic disorders are less common. Although the liver plays a role in metabolism of amino acids and generation of acid from dietary sources, it does not play a role in the regulation of pH. Instead, metabolic disorders may arise from alterations in normal metabolism or from medications, particularly diuretics and osmotic laxatives, used in the treatment of these complex patients. Understanding the mechanistic underpinnings of these disorders can aid in the management of individuals with liver disease in the hospital and in outpatient settings.
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Affiliation(s)
- Eric J Xu
- Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA
| | - Ayaa M Zarm
- Department of Nephrology, Oschner Medical Center, New Orleans, LA
| | - Stewart H Lecker
- Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA
| | - Melanie P Hoenig
- Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA.
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4
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Rodríguez-Roisin R. Hepatopulmonary Syndrome: A Forgotten Liver-induced Lung Vascular Disorder. Arch Bronconeumol 2023; 59:137-138. [PMID: 36609108 DOI: 10.1016/j.arbres.2022.12.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/19/2022] [Accepted: 12/19/2022] [Indexed: 12/24/2022]
Affiliation(s)
- Robert Rodríguez-Roisin
- Universitat de Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic, Barcelona, Spain.
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5
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Raza MH, Kwon Y, Kobierski P, Misra AC, Lim A, Goldbeck C, Etesami K, Kohli R, Emamaullee J. Model for End-Stage Liver Disease/Pediatric End-Stage Liver Disease exception policy and outcomes in pediatric patients with hepatopulmonary syndrome requiring liver transplantation. Liver Transpl 2023; 29:134-144. [PMID: 37160070 PMCID: PMC9868062 DOI: 10.1002/lt.26548] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/18/2022] [Revised: 07/11/2022] [Accepted: 07/14/2022] [Indexed: 01/29/2023]
Abstract
Hepatopulmonary syndrome (HPS) is associated with increased waitlist mortality in liver transplantation (LT) candidates. Children with HPS are granted Model for End-Stage Liver Disease (MELD)/Pediatric End-Stage Liver Disease (PELD) exception points for waitlist prioritization in the United States based on criterion developed for adults. In this study, the impact of this MELD/PELD exception policy on post-LT survival in children was examined. A retrospective cohort of patients aged younger than 18 years with a MELD/PELD exception request who underwent LT between 2007 and 2018 were identified in the Scientific Registry of Transplant Recipients. Patients were stratified by waitlist partial pressure of arterial oxygen (PaO 2 ) to assess risk factors for waitlist mortality and post-LT survival. Among 3082 pediatric LT recipients included in the study, 124 patients (4%) received MELD/PELD exception points for HPS. Patients with HPS were a median age of 9 years (interquartile range: 6, 12 years), 54.8% were girls, and 54% were White. Most patients (87.9%) were listed with laboratory MELD/PELD scores <15. Waitlist mortality for patients with HPS exception points was rare and not different from patients without HPS. When stratified by pre-LT PaO 2 , hypoxemia severity was not associated with differences in 1-, 3-, or 5-year survival rates after LT ( p = 0.13). However, patients with HPS showed a slightly lower survival rate at 5 years compared with patients without HPS (88.7% vs. 93.4%; p = 0.04). MELD/PELD exceptions for children with HPS mitigated waitlist mortality, and recipients with HPS experienced excellent 5-year survival after LT, although slightly lower than in patients without HPS. Unlike adults with HPS, the severity of pre-LT hypoxemia in children does not impact post-LT survival. These data suggest that adult criteria for granting MELD/PELD exception points may not appropriately capture HPS severity in pediatric patients. Further prospective multicenter studies to examine the risk factors predicting negative survival outcomes in children with HPS are warranted.
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Affiliation(s)
- Muhammad H Raza
- Keck School of Medicine , University of Southern California , Los Angeles , California , USA
| | - Yong Kwon
- Keck School of Medicine , University of Southern California , Los Angeles , California , USA.,Division of Hepatobiliary and Abdominal Transplant Surgery, Department of Surgery , University of Southern California , Los Angeles , California , USA.,Liver Transplant Program , Children's Hospital-Los Angeles , Los Angeles , California , USA
| | - Pierre Kobierski
- Keck School of Medicine , University of Southern California , Los Angeles , California , USA
| | - Asish C Misra
- Keck School of Medicine , University of Southern California , Los Angeles , California , USA.,Division of Hepatobiliary and Abdominal Transplant Surgery, Department of Surgery , University of Southern California , Los Angeles , California , USA.,Liver Transplant Program , Children's Hospital-Los Angeles , Los Angeles , California , USA
| | - Angelina Lim
- Keck School of Medicine , University of Southern California , Los Angeles , California , USA
| | - Cameron Goldbeck
- Division of Hepatobiliary and Abdominal Transplant Surgery, Department of Surgery , University of Southern California , Los Angeles , California , USA
| | - Kambiz Etesami
- Keck School of Medicine , University of Southern California , Los Angeles , California , USA.,Division of Hepatobiliary and Abdominal Transplant Surgery, Department of Surgery , University of Southern California , Los Angeles , California , USA.,Liver Transplant Program , Children's Hospital-Los Angeles , Los Angeles , California , USA
| | - Rohit Kohli
- Keck School of Medicine , University of Southern California , Los Angeles , California , USA.,Liver Transplant Program , Children's Hospital-Los Angeles , Los Angeles , California , USA.,Department of Pediatrics , University of Southern California , Los Angeles , California , USA
| | - Juliet Emamaullee
- Keck School of Medicine , University of Southern California , Los Angeles , California , USA.,Division of Hepatobiliary and Abdominal Transplant Surgery, Department of Surgery , University of Southern California , Los Angeles , California , USA.,Liver Transplant Program , Children's Hospital-Los Angeles , Los Angeles , California , USA
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6
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Gandhi KD, Taweesedt PT, Sharma M, Surani S. Hepatopulmonary syndrome: An update. World J Hepatol 2021; 13:1699-1706. [PMID: 34904039 PMCID: PMC8637683 DOI: 10.4254/wjh.v13.i11.1699] [Citation(s) in RCA: 6] [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: 05/16/2021] [Revised: 06/25/2021] [Accepted: 08/31/2021] [Indexed: 02/06/2023] Open
Abstract
Hepatopulmonary syndrome (HPS) is characterized by defects in oxygenation caused by intra-pulmonary vasodilation occurring because of chronic liver disease, portal hypertension, or congenital portosystemic shunts. Clinical implications of portal hypertension are very well-known, however, awareness of its effect on multiple organs such as the lungs are less known. The presence of HPS in chronic liver disease is associated with increased mortality. Medical therapies available for HPS have not been proven effective and definitive treatment for HPS is mainly liver transplantation (LT). LT improves mortality for patients with HPS drastically. This article provides a review on the definition, clinical presentation, diagnosis, and management of HPS.
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Affiliation(s)
- Kejal D Gandhi
- Department of Internal Medicine, Medstar Washington Hospital Center/Georgetown University, Washigton, DC 20010, United States
| | - Pahnwat Tonya Taweesedt
- Department of Medicine, Corpus Christi Medical Center, Corpus Christi, TX 78412, United States
| | - Munish Sharma
- Department of Medicine, Corpus Christi Medical Center, Corpus Christi, TX 78412, United States
| | - Salim Surani
- Department of Medicine, Texas A&M University, Bryan, TX 78413, United States
- Department of Anesthesiology, Mayo Clinic, Rochester, MN 55905, United States.
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7
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Murphy C, Arad D. Case Report: Hepatopulmonary syndrome as the first clinical manifestation of cirrhosis in a patient with underlying chronic lung disease. F1000Res 2019; 7:1175. [PMID: 31354935 PMCID: PMC6635985 DOI: 10.12688/f1000research.15434.2] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 05/08/2019] [Indexed: 11/20/2022] Open
Abstract
An 86 year old woman with multiple chronic lung diseases (including chronic obstructive pulmonary disease, bronchiectasis, and untreated mycobacterium avium-intracellulare) presented with two weeks of increased shortness of breath, notably worse when seated as compared to when lying down. After treatments focused on her known conditions did not resolve her dyspnea, the differential diagnosis was broadened and she was found to have evidence of cirrhosis on imaging. As a result of this new diagnosis, transthoracic echocardiography and arterial blood gas analysis were performed and together yielded the diagnosis of hepatopulmonary syndrome. We describe a rare presentation of hepatopulmonary syndrome manifesting as a patient’s first clinical evidence of suspected cirrhosis, a diagnosis made difficult by this patient’s numerous other lung diseases which muddied the picture.
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Affiliation(s)
- Charles Murphy
- Internal Medicine, Montefiore Medical Center, Bronx, Bronx, NY, 10029, USA
| | - Danit Arad
- Internal Medicine, Montefiore Medical Center, Bronx, Bronx, NY, 10029, USA
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8
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Cryptogenic Cirrhosis and Hepatopulmonary Syndrome in a Boy with Hepatic Hemangioma in Botswana: A Case Report and Review of the Literature. Case Rep Pediatr 2018; 2017:7940365. [PMID: 29410927 PMCID: PMC5749285 DOI: 10.1155/2017/7940365] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/24/2017] [Revised: 10/09/2017] [Accepted: 10/31/2017] [Indexed: 11/17/2022] Open
Abstract
Hepatic hemangiomas are considered to be the most common benign tumors of the liver. They are often found incidentally while investigating for other causes of liver disease. Hemangiomas that are less than 10 cm are not expected to cause any problems. Typically, they do not enlarge and, apart from regular follow-up, no definitive treatment is indicated. This is a posthumous case report of a male child with a medium-sized hemangioma from infancy, complicated by cryptogenic cirrhosis and hepatopulmonary syndrome. It demonstrates the challenges of managing a child with such complicated conditions in a resource-limited setting.
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9
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Herborn J, Parulkar S. Anesthetic Considerations in Transplant Recipients for Nontransplant Surgery. Anesthesiol Clin 2017; 35:539-553. [PMID: 28784225 DOI: 10.1016/j.anclin.2017.04.009] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
As solid organ transplantation increases and patient survival improves, it will become more common for these patients to present for nontransplant surgery. Recipients may present with medical problems unique to the transplant, and important considerations are necessary to keep the transplanted organ functioning. A comprehensive preoperative examination with specific focus on graft functioning is required, and the anesthesiologist needs pay close attention to considerations of immunosuppressive regimens, blood product administration, and the risk benefits of invasive monitoring in these immunosuppressed patients. This article reviews the posttransplant physiology and anesthetic considerations for patients after solid organ transplantation.
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Affiliation(s)
- Joshua Herborn
- Department of Anesthesiology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
| | - Suraj Parulkar
- Department of Anesthesiology, Northwestern University Feinberg School of Medicine, 251 East Huron Street, F5-704, Chicago, IL 60611, USA
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10
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International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension. Transplantation 2017; 100:1440-52. [PMID: 27326810 DOI: 10.1097/tp.0000000000001229] [Citation(s) in RCA: 291] [Impact Index Per Article: 36.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Two distinct pulmonary vascular disorders, hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) may occur as a consequence of hepatic parenchymal or vascular abnormalities. HPS and POPH have major clinical implications for liver transplantation. A European Respiratory Society Task Force on Pulmonary-Hepatic Disorders convened in 2002 to standardize the diagnosis and guide management of these disorders. These International Liver Transplant Society diagnostic and management guidelines are based on that task force consensus and should continue to evolve as clinical experience dictates. Based on a review of over 1000 published HPS and POPH articles identified via a MEDLINE search (1985-2015), clinical guidelines were based on, selected single care reports, small series, registries, databases, and expert opinion. The paucity of randomized, controlled trials in either of these disorders was noted. Guidelines are presented in 5 parts; I. Definitions/Diagnostic criteria; II. Hepatopulmonary syndrome; III. Portopulmonary hypertension; IV. Implications for liver transplantation; and V. Suggestions for future clinical research.
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11
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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 may induce severe hypoxemia. Microvascular dilation and angiogenesis in the lung have been identified as pathologic features that drive gas exchange abnormalities in experimental HPS. Pulse oximetry is a useful screening test for HPS, which can guide subsequent use of arterial blood gases. Contrast-enhanced echocardiography, perfusion lung scanning, and pulmonary arteriography are three currently used diagnostic imaging modalities that identify the presence of intrapulmonary vascular abnormalities. The presence of HPS increases mortality and impairs quality of life, but is reversible with liver transplantation. No medical therapy is established as effective for HPS. At the present time, liver transplantation is the only available treatment for HPS.
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Affiliation(s)
- Yong Lv
- Department of Liver Disease, Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China,
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12
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DuBrock HM, Bankier AA, Silva M, Litmanovich DE, Curry MP, Washko GR. Pulmonary Vessel Cross-sectional Area before and after Liver Transplantation: Quantification with Computed Tomography. Acad Radiol 2015; 22:752-9. [PMID: 25770631 DOI: 10.1016/j.acra.2015.01.018] [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: 12/04/2014] [Revised: 01/23/2015] [Accepted: 01/25/2015] [Indexed: 10/23/2022]
Abstract
RATIONALE AND OBJECTIVES Pulmonary vascular complications of liver disease have a substantial impact on morbidity and mortality in patients who undergo liver transplant. The effect of liver transplantation on the pulmonary vasculature in patients without pulmonary vascular disease, however, has not been described. This study was undertaken to characterize the regional effect of liver transplant on the cross-sectional area (CSA) of pulmonary vessels. MATERIALS AND METHODS We performed a single-center, retrospective, cohort study of patients who had a liver transplant between 2002 and 2012 and who had chest computed tomography scans within 1 year before and after transplant. Using ImageJ software, we measured the CSA of small pulmonary vessels (0-5 mm(2)) and the total lung CSA to calculate the percent CSA of pulmonary vessels <5 mm (%CSA<5) at the level of the aortic arch, carina, and right inferior pulmonary vein (RIPV). Pretransplant and posttransplant, %CSA<5 were compared, and associations of pretransplant %CSA<5 with clinical parameters were measured. RESULTS There was a significant decrease in %CSA<5 at the level of the RIPV (0.19% [interquartile range {IQR}, 0.15-0.26] before vs. 0.15% [IQR, 0.12-0.21] after; P = .0003), with a median change of -16.2% (IQR, -39.3 to 3.9) posttransplant. Changes at the level of the aortic arch and carina were not significant. Pretransplant RIPV %CSA<5 was not significantly correlated with severity of liver disease or oxygenation but was inversely correlated with percent change in %CSA<5 (r = -0.39; P = .0039). CONCLUSIONS This is the first study to describe a significant regional change in the pulmonary vessels of patients without known pulmonary vascular disease who undergo liver transplant.
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Does general anesthesia with inhalation anesthetics worsen hypoxemia in patients with end-stage liver disease and an intrapulmonary shunt? Transplant Proc 2011; 43:1665-8. [PMID: 21693254 DOI: 10.1016/j.transproceed.2011.03.056] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/28/2010] [Revised: 03/11/2011] [Accepted: 03/22/2011] [Indexed: 01/12/2023]
Abstract
Hepatopulmonary syndrome (HPS) is common among patients with end-stage liver disease (ESLD); however, the effects of general anesthesia on oxygenation capacity have not been studied in these patients. The aim of this study was to evaluate the effects of general anesthesia with inhalation anesthetics on oxygenation parameters according to intrapulmonary shunt grade in patients undergoing liver transplantation. Fifty-eight liver transplant recipients were divided into 2 groups according to the intrapulmonary shunt grade as determined using preoperative echocardiography using the microbubble-syringe technique. Patients in the 'no shunt' group (n = 44) had either no detectable or a mild shunt, whereas those in the "shunt" group (n = 14) displayed moderate to severe changes. Arterial blood gas analysis was performed twice for each patient: preoperatively and 30 minutes after induction of general anesthesia. We calculated arterial oxygen partial pressure-to-FiO(2) ratio (PaO(2)/FiO(2)), alveolar-arterial oxygen difference (A-aDO(2)), age-corrected A-aDO(2), A-aDO(2)-to-inspiratory oxygen fraction ratio (AaDO(2)/FiO(2)), and alveolar oxygen partial pressure-to-PaO(2) ratio (PAO(2)/PaO(2)). In the preoperative period, the PaO(2) was lower in the shunt compared with the no shunt group (77.8 ± 24.3 vs 92.9 ± 14.5, respectively; P = .016), as was the PaO(2)/FiO(2). A-aDO(2), age-corrected A-aDO(2), A-aDO(2)/FiO(2), and PAO(2)/PaO(2) were all greater in the shunt group preoperatively. After induction of general anesthesia, all parameters increased in both groups, but the differences between the 2 groups were no longer significant. Patients with ESLD who underwent liver transplantation with a moderate to severe intrapulmonary shunt showed lower preoperative oxygenation capacities than those without a shunt or with a mild shunt. General anesthesia decreased oxygenation capacity in all patients, but the differences between the 2 groups were no longer significant after induction.
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14
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Rapid progression from hepatopulmonary syndrome to portopulmonary hypertension in an adolescent female with hypopituitarism. J Pediatr Gastroenterol Nutr 2010; 50:334-6. [PMID: 19668006 DOI: 10.1097/mpg.0b013e3181a1c496] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
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Thevenot T, Pastor CM, Cervoni JP, Jacquelinet C, Nguyen-Khac E, Richou C, Heyd B, Vanlemmens C, Mantion G, Di Martino V, Cadranel J. [Hepatopulmonary syndrome]. GASTROENTEROLOGIE CLINIQUE ET BIOLOGIQUE 2009; 33:565-579. [PMID: 19481392 DOI: 10.1016/j.gcb.2009.03.013] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 02/25/2008] [Revised: 08/12/2008] [Accepted: 03/12/2009] [Indexed: 12/17/2022]
Abstract
Hepatopulmonary syndrome is characterized by the presence of portal hypertension with or without cirrhosis, an increased alveolar-arterial oxygen partial pressure difference greater than or equal to 15 mm Hg, and dilated pulmonary capillaries. Hepatopulmonary syndrome is found in up to 20% of patients with cirrhosis and should be considered in any patient who develops dyspnea or hypoxemia. Contrast echocardiography is enough to make the diagnosis of hepatopulmonary syndrome. The exact pathophysiology of hepatopulmonary syndrome remains unknown but nitric oxide is an important factor underlying hepatopulmonary syndrome. Hypoxemia progressively deteriorates and worsens the prognosis of cirrhotic patients. Hypoxemic patients must be controlled regularly to optimise the timing of liver transplantation. Indeed, a preoperative PaO(2) of less than or equal to 50 mm Hg alone or in combination with an isotopic shunt fraction greater than or equal to 20% are the strongest predictors of postoperative mortality. There are currently no effective medical therapies for hepatopulmonary syndrome but garlic powder and iloprost inhalation demonstrate clinical improvements in the pre- and in the post-transplant period.
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Affiliation(s)
- T Thevenot
- Service d'Hépatologie et de Soins Intensifs Digestifs, Hôpital Universitaire Jean-Minjoz, 25030 Besançon, France.
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Deberaldini M, Arcanjo ABB, Melo E, da Silva RF, Felício HCC, Arroyo PC, Duca WJ, Cordeiro JA, da Silva RCMA. Hepatopulmonary syndrome: morbidity and survival after liver transplantation. Transplant Proc 2009; 40:3512-6. [PMID: 19100426 DOI: 10.1016/j.transproceed.2008.08.134] [Citation(s) in RCA: 37] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/09/2007] [Revised: 07/23/2008] [Accepted: 08/12/2008] [Indexed: 02/07/2023]
Abstract
Hepatopulmonary Syndrome (HPS) is a triad of liver disease, intrapulmonary vascular dilatation (IPVD), and arterial deoxygenation. Orthotopic liver transplantation (OLT) constitutes the only effective treatment; however, adverse outcomes have been reported. The aim of this study was to evaluate the early morbidity and short- and long-term survival after OLT for patients with and without HPS. We studied 59 transplant recipients divided into 2 groups: with HPS (HPS group n = 25) and without HPS (control group, n = 34) before the OLT. IPVD was diagnosed using transthoracic contrast-enhanced echocardiography. Arterial deoxygenation was defined as PA-a,O(2) >or= 15 mm Hg. The HPS and control groups were homogeneous regarding age (P = .36; 43.8 +/- 12.2 vs 46.9 +/- 13.5), gender (P = .47), male/female ratio (68%:32% and 78%:22%, respectively), and severity of liver disease. The PaO(2) was significantly lower (74.9 +/- 12.1 vs 93 +/- 6.4 mm Hg; P < .001) and the PA-a,O(2) was significantly higher in the HPS group (30.3 +/- 10.6 vs 11.0 +/- 7.0; P < .001). The percentage of severe (n = 3) and very severe (n = 1) hypoxemia was 16%. There were no significant differences between HPS and control groups regarding short- (68% vs 77%; P = .27) and long-term survival (60% vs 64%; P = .67) as well as among patients with mild, moderate, severe, or very severe HPS and the control group (P = .53). Also, intensive care unit (ICU) stay (7.0 vs 5.5; P = .41), duration of mechanical ventilation (38.0 vs 27.5; P = .43), reintubation rate (32.0% vs 23.5%; P = .45), and early postoperative complications (P = .72) were not different. In conclusion, there were no significant differences regarding the outcomes of OLT for patients with versus without HPS related to early morbidity or short- and long-term survival.
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Affiliation(s)
- M Deberaldini
- Department of Medicine I, Medical School of São José do Rio Preto - FAMERP, Sao Paulo, Brazil
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17
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Affiliation(s)
- Roberto Rodríguez-Roisin
- Servei de Pneumologia (Institut del Tòrax), Hospital Clínic, Institut d'Investigacions Biomédiques August Pi i Sunyer (IDIBAPS), Ciber Enfermedades Respiratorias, and the University of Barcelona, Barcelona, Spain.
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18
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Sulieman BM, Hunsicker LG, Katz DA, Voigt MD. OPTN policy regarding prioritization of patients with hepatopulmonary syndrome: does it provide equitable organ allocation? Am J Transplant 2008; 8:954-64. [PMID: 18416736 DOI: 10.1111/j.1600-6143.2007.02124.x] [Citation(s) in RCA: 33] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/25/2023]
Abstract
United Network for Organ Transplantation (UNOS) policy 3.6.4.5.1 provides exception points to patients diagnosed with hepatopulmonary syndrome (HPS) to compensate for their reported increased mortality risk. We compared pre- and posttransplant and overall outcomes in 255 patients receiving exception points under this policy (HPS policy patients) with 32 358 nonexception control patients listed in the model for end-stage liver disease (MELD) era to determine whether the intent of the policy is being met. Overall, 92.5% of HPS policy patients versus 45.5% of controls had been transplanted, 5.1% versus 31.2% remained on waiting list and 1.5% versus 14.1% had died while awaiting transplant (p < 0.0001 for each comparison). Relative risk (RR) of death for HPS policy patients compared to controls was 0.158 (confidence interval [CI]: 0.059-0.420, p = 0.0002) pretransplant, and 0.827 (CI: 0.587-1.170, p = 0.28) posttransplant. Overall (combined waitlist and posttransplant) RR of death was 0.514 (CI: 0.374-0.707, p = 0.00004) compared with controls. After adjustment for laboratory MELD, overall RR was 0.807 (CI: 0.587-1.110, p = 0.19), indicating that HPS policy patients' mortality risk would be similar to that of controls had they been listed with their laboratory MELD score. HPS policy patients have a significant pretransplant survival advantage over standard liver transplant candidates because of the exception points awarded, and have similar posttransplant survival. Better criteria for diagnosing and grading of HPS are required.
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Affiliation(s)
- B M Sulieman
- Department of Internal Medicine, Divisions of Gastroenterology and Hepatology, Carver College of Medicine, University of Iowa, Iowa City, IA, USA
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19
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Zhang HY, Han DW, Su AR, Zhang LT, Zhao ZF, Ji JQ, Li BH, Ji C. Intestinal endotoxemia plays a central role in development of hepatopulmonary syndrome in a cirrhotic rat model induced by multiple pathogenic factors. World J Gastroenterol 2007; 13:6385-95. [PMID: 18081228 PMCID: PMC4205458 DOI: 10.3748/wjg.v13.i47.6385] [Citation(s) in RCA: 22] [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] [Indexed: 02/06/2023] Open
Abstract
AIM: To characterize the correlation between severity of hepatopulmonary syndrome (HPS) and degree of hepatic dysfunction, and to explore how intestinal endotoxemia (IETM) affects the development of HPS in cirrhotic rats.
METHODS: Male Wister rats were fed with a diet containing maize flour, lard, cholesterol, and alcohol and injected subcutaneously with CCl4 oil solution every two days for 8 wk to induce typical cirrhosis and development of HPS. The animals were also given a nitric oxide (NO) production inhibitor, Nω-nitro-L-arginine methyl ester (L-NAME) intraperitoneally, and an iNOS inhibitor, aminoguanidine hydrochloride (AG) via gavage daily from the end of the 4th wk to the end of the 6th or 8th wk, or a HO-1 inhibitor, zinc protoporphyrin (ZnPP) intraperitoneally 12 h prior to killing. Blood, liver and lung tissues were sampled.
RESULTS: Histological deterioration of the lung paralleled to that of the liver in the cirrhotic rats. The number of pulmonary capillaries was progressively increased from 6.1 ± 1.1 (count/filed) at the 4th wk to 14.5 ± 2.4 (count/filed) at the 8th wk in the cirrhotic rats. Increased pulmonary capillaries were associated with increased blood levels of lipopolysaccharide (LPS) (0.31 ± 0.08 EU/mL vs control 0.09 ± 0.03 EU/mL), alanine transferase (ALT, 219.1 ± 17.4 U/L vs control 5.9 ± 2.2 U/L) and portal vein pressure. Compared with normal control animals, the number of total cells in bronchoalveolar lavage fluid (BALF) of the cirrhotic rats at the 8th wk was not changed, but the number of macrophages and the ratio of macrophages to total cells were increased by nearly 2-fold, protein expression of inducible nitric oxide synthase (iNOS) and endothelial nitric oxide synthase (eNOS) started to increase significantly at the 4th wk, and reached its peak at the 8th wk in the lung of cirrhotic rats. The increase of iNOS expression appeared to be quicker than that of eNOS. NO2-/NO3- was also increased, which was correlated to the increase of iNOS (r = 0.7699, P < 0.0001) and eNOS (r = 0.5829, P < 0.002). mRNA expression of eNOS and iNOS was highly consistent with their protein expression.
CONCLUSION: Progression and severity of HPS as indicated by both increased pulmonary capillaries and histological changes are closely associated with LPS levels and progression of hepatic dysfunction as indicated by increased levels of ALT and portal vein pressure. Intestinal endotoxemia plays a central role in the development of HPS in the cirrhotic rat model by inducing NO and/or CO.
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20
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Liang CD, Ko SF, Chen CL, Concejero AM. Impact of Mild and Moderate Intrapulmonary Shunting in Children with End-Stage Liver Disease Undergoing Liver Transplantation. World J Surg 2007; 31:1474-9. [PMID: 17505855 DOI: 10.1007/s00268-007-9063-x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2007] [Revised: 02/06/2007] [Accepted: 03/04/2007] [Indexed: 01/06/2023]
Abstract
AIM The aim of this study was to evaluate the impact of mild and moderate intrapulmonary shunting (IPS) in children with end-stage liver disease (ESLD) undergoing liver transplantation. MATERIALS AND METHODS A total of 73 patients (38 male, 35 female; mean age 31.5 +/- 35.2 months, range 6-180 months) with ESLD and subsequent liver transplantation were enrolled. Based on contrast echocardiography, patients without IPS were assigned to group 1 (n = 57), and patients with mild or moderate IPS were assigned to group 2 (n = 17). The preoperative age, body weight, O(2) saturation, length of hospital stay, duration of mechanical ventilation, postoperative complications, and 1-year survival rate were compared between groups. RESULTS The overall incidence of IPS and 1-year survival rate were 23.3% (17/73) and 96% (70/73), respectively. There were significant differences between group 1 and group 2 patients regarding age at transplant (35.9 vs. 16.6 months, p = 0.002) and body weight (12.6 vs. 8.5 kg, p = 0.002). There were no other statistically significant differences between the two groups. CONCLUSION Although children with mild and moderate IPS were younger at the time of transplantation and had significantly lower body weight than those without IPS, the presence of mild and moderate IPS in children with ESLD did not affect the overall outcome of liver transplantation.
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Affiliation(s)
- Chi-Di Liang
- Liver Transplantation Program, Section of Pediatric Cardiology, Department of Pediatrics, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Chang Gung University College of Medicine, 123 Ta-Pei Road, Niao Sung, Kaohsiung 833, Taiwan
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21
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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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22
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Gohh RY, Warren G. The Preoperative Evaluation of the Transplanted Patient for Nontransplant Surgery. Surg Clin North Am 2006; 86:1147-66, vi. [PMID: 16962406 DOI: 10.1016/j.suc.2006.07.001] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
Abstract
With the improved success of solid-organ transplantation, there has been an increased willingness to transplant individuals previously felt to be unsuitable for such procedures. Factors such as age and various medical comorbidities are no longer considered contraindications to transplantation, and hence, an increasing number of recipients may require medical care not specifically related to the transplant. After transplantation, many of these patients may require elective or emergent surgery, making it important for all surgeons to be familiar with the factors that may influence surgical outcomes in this population, asa well asa factors that affect postoperative care. Most transplant centres use a team approach to manage these complex patients, relying on medical professionals experienced in their care and management. Close interaction with the transplant team is likely the single most important step in preparing the transplanted patient for surgery and managing their postoperative care.
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Affiliation(s)
- Reginald Y Gohh
- Division of Renal Diseases, Rhode Island Hospital, Brown University School of Medicine, 593 Eddy Street, APC-921, Providence, Rhode Island 02903, USA.
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23
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Martínez-Palli G, Gómez FP, Barberà JA, Navasa M, Roca J, Rodríguez-Roisin R, Burgos F, Gistau C. Sustained low diffusing capacity in hepatopulmonary syndrome after liver transplantation. World J Gastroenterol 2006; 12:5878-83. [PMID: 17007057 PMCID: PMC4100672 DOI: 10.3748/wjg.v12.i36.5878] [Citation(s) in RCA: 27] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
AIM: To study the presence of sustained low diffusing capacity (DLCO) after liver transplantation (LT) in patients with hepatopulmonary syndrome (HPS).
METHODS: Six patients with mild-to-severe HPS and 24 without HPS who underwent LT were prospectively followed before and after LT at mid-term (median, 15 mo). HPS patients were also assessed at long-tem (median, 86 mo).
RESULTS: Before LT, HPS patients showed lower PaO2 (71 ± 8 mmHg), higher AaPO2 (43 ± 10 mmHg) and lower DLCO (54% ± 9% predicted), due to a combination of moderate-to-severe ventilation-perfusion (VA/Q) imbalance, mild shunt and diffusion limitation, than non-HPS patients (94 ± 4 mmHg and 19 ± 3 mmHg, and 85% ± 3% predicted, respectively) (P < 0.05 each). Seven non-HPS patients had also reduced DLCO (70% ± 4% predicted).
At mid- and long-term after LT, compared to pre-LT, HPS patients normalized PaO2 (91 ± 3 mmHg and 87 ± 5 mmHg), AaPO2 (14 ± 3 mmHg and 23 ± 5 mmHg) and all VA/Q descriptors (P < 0.05 each) without changes in DLCO (53% ± 8% and 56% ± 7% predicted, respectively). Post-LT DLCO in non-HPS patients with pre-LT low DLCO was unchanged (75% ± 6% predicted).
CONCLUSION: While complete VA/Q resolution in HPS indicates a reversible functional disturbance, sustained low DLCO after LT also present in some non-HPS patients, points to persistence of sub-clinical liver-induced pulmonary vascular changes.
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Abstract
The transplant recipient has traded a life-threatening illness for a chronically immunosuppressed state. Subsequent anesthetic management for non-transplant surgical procedures may be challenging. The anesthesia provider must be aware of the degree of post-transplant organ dysfunction and alter anesthesia techniques accordingly. This article reviews the anesthetic concerns for patients who have undergone a variety of organ transplants.
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Affiliation(s)
- Mark T Keegan
- Division of Critical Care, Department of Anesthesiology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA
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25
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Mazzeo AT, Lucanto T, Santamaria LB. Hepatopulmonary syndrome: a concern for the anesthetist? Pre-operative evaluation of hypoxemic patients with liver disease. Acta Anaesthesiol Scand 2004; 48:178-86. [PMID: 14995940 DOI: 10.1111/j.0001-5172.2004.00282.x] [Citation(s) in RCA: 27] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/23/2022]
Abstract
Liver cirrhosis and other chronic hepatic diseases are followed in a subset of affected patients by gas exchange abnormalities resulting from a syndrome called hepatopulmonary syndrome (HPS). The structural basis of this clinical entity is an alteration of pulmonary vasculature resulting in abnormal vasodilatation and mismatching of ventilation and perfusion of the lung. Dilatation of the capillary bed near the gas exchange area is the most important factor implicated; it precludes O2 molecules diffusing to the centrum of the dilated vessels to oxygenate venous blood. Contrast (microbubbles) echocardiography and lung perfusion scan are, respectively, the screening tests with the highest sensitivity and specificity for HPS diagnosis. Because of the high morbidity and mortality of HPS, clinicians have been trying to understand the pathophysiology of pulmonary vasodilatation in the hope that the process can be reversed pharmacologically or surgically. An imbalance between production and clearance of vasoactive circulating substances has been implicated in the pathogenesis of HPS with glucagon and nitric oxide among the principal responsible factors. To date various molecules have been implicated for therapy but without definitive positive results. Liver transplantation remains the only real therapy for HPS, and resolution of gas exchange defects outlines the possible functional reversible nature of vascular abnormalities of this syndrome. The need to perform surgery under general anesthesia for hepatic and extrahepatic procedures in patients with HPS is followed by an increased peri-operative risk. The authors emphasize the role of pre-operative clinical evaluation for proper patient management during the peri-operative period.
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Affiliation(s)
- A T Mazzeo
- Department of Neuroscience, Psychiatric and Anesthesiological Sciences, Section of Anesthesiology and Intensive Care, University Hospital Policlinico G. Martino, Via Consolare Valeria, 98121 Messina, Italy.
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26
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Abstract
Liver transplantation is the accepted treatment for patients with end-stage liver disease or intractable symptoms secondary to primary biliary cirrhosis (PBC), and has proven survival benefit. Indications for transplantation are an unacceptable quality of life or anticipated death in less than 1 year. Although there are a number of prognostic models, serum bilirubin provides the simplest guide to transplantation timing. Those grafted for PBC are at greater risk of developing chronic rejection, and are less likely to be successfully weaned from immunosuppression than those grafted for other indications. Following transplantation, antimitochondrial antibodies persist and histological features of recurrent PBC may be seen in the allograft in up to 50% by 10 years; however, at least in the medium-term, this rarely causes clinical problems.
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27
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Zhang XJ, Katsuta Y, Akimoto T, Ohsuga M, Aramaki T, Takano T. Intrapulmonary vascular dilatation and nitric oxide in hypoxemic rats with chronic bile duct ligation. J Hepatol 2003; 39:724-30. [PMID: 14568253 DOI: 10.1016/s0168-8278(03)00430-6] [Citation(s) in RCA: 33] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/16/2023]
Abstract
BACKGROUND/AIMS Nitric oxide (NO) has been suggested as the major cause of pulmonary vascular dilatation and hypoxemia in hepatopulmonary syndrome (HPS). The aim of this study was to assess the effect of NO on arterial oxygenation in rats with common bile duct ligation (CBDL rats), a model of HPS. METHODS Arterial blood gases were measured in 44 CBDL rats and 44 Sham rats under unrestrained conditions. Intrapulmonary shunting was assessed with (141)Ce-labeled microspheres (15-mum diameter) and serum nitrate/nitrite levels were measured by HPLC. The effect of NOS inhibition on A-aDO(2) was studied using L-NAME. RESULTS A decrease of PaO(2) below 82.7 mmHg (the mean value-2sigma in Sham rats) was seen in 43% of CBDL rats. Intrapulmonary shunting was greater in CBDL rats than in Sham rats (P<0.001). A correlation between the extent of shunting and A-aDO(2) was found in all animals studied (r=0.89, P<0.001, n=16). Serum levels of nitrate/nitrite increased significantly across the lungs, and the increase was significantly correlated with A-aDO(2) in the total population of animals studied. Administration of L-NAME to CBDL rats achieved a significant improvement of A-aDO(2). CONCLUSIONS These results suggest that pulmonary vascular dilatation due to NO leads to hypoxemia in CBDL rats.
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Affiliation(s)
- Xue-Jun Zhang
- First Department of Internal Medicine, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan.
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28
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Abstract
We report a case where an embolization therapy was successfully performed for diffusely dilated pulmonary vessels in a patient with hepatopulmonary syndrome (HPS) as a palliative treatment before liver transplantation. To our knowledge, this therapeutic approach has been tried only for arteriovenous malformation type HPS, and not for diffuse dilatation [Chest 91 (1987) 920].
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Affiliation(s)
- Jung Kyu Ryu
- Department of Diagnostic Radiology, Kyung Hee University Hospital, Hoeki-Dong #1, Dongdaemun-Ku, Seoul 130-702, South Korea
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29
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Mohamed R, Freeman JW, Guest PJ, Davies MK, Neuberger JM. Pulmonary gas exchange abnormalities in liver transplant candidates. Liver Transpl 2002; 8:802-808. [PMID: 12200782 DOI: 10.1053/jlts.2002.33746] [Citation(s) in RCA: 26] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Abnormal diffusing capacity is the commonest pulmonary dysfunction in liver transplant candidates, but severe hypoxemia secondary to hepatopulmonary syndrome and significant pulmonary hypertension are pulmonary vascular manifestations of cirrhosis that may affect the perioperative course. We prospectively assessed the extent of pulmonary dysfunction in patients referred for liver transplantation. A total of 57 consecutive patients with chronic liver disease were evaluated. All patients had a chest radiograph, standing arterial blood gas on room air, pulmonary function testing, and Doppler echocardiogram. Those patients with arterial hypoxaemia (PaO(2) < 10 kPa) also underwent (99m)Tc-macroaggregated albumin lung scan, and nine patients had agitated normal saline injection during echocardiography to define further the existence of pulmonary vascular dilatation. Reduced diffusing capacity for carbon monoxide less than 75% of the predicted value was found in 29 of 57 (51%) patients. Although elevated alveolar-arterial oxygen tension difference was detected in 35% (20/57) of the patients, only four (7%) patients had hypoxemia. We were unable to find evidence of intrapulmonary vascular dilatation either on the lung scan or saline-enhanced echocardiography in any of these patients. Reduction in diffusing capacity for carbon monoxide was noted in 75% (18/24) of patients who were transplanted for primary biliary cirrhosis and was accompanied by widened alveolar-arterial oxygen tension in 10 out of 18 (56%) of patients. This study shows that in liver transplant candidates, diffusion impairment and widened alveolar-arterial oxygen tension difference were frequently detected, especially in patients with primary biliary cirrhosis.
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30
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Santamaria F, Sarnelli P, Celentano L, Farina V, Vegnente A, Mansi A, Montella S, Vajro P. Noninvasive investigation of hepatopulmonary syndrome in children and adolescents with chronic cholestasis. Pediatr Pulmonol 2002; 33:374-9. [PMID: 11948983 DOI: 10.1002/ppul.10088] [Citation(s) in RCA: 19] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/19/2023]
Abstract
Early detection of hepatopulmonary syndrome (HPS) may be delayed because of invasiveness of the diagnostic procedures. In this pilot study, we prospectively investigated the usefulness of determining transcutaneous O(2) tension after 100% O(2) (TcPO(2)100) breathing using a transcutaneous hyperoxia test (THT) in 11 children with chronic cholestasis and without primary cardiopulmonary disease. These patients also underwent alveolar-arterial O(2) gradient testing (AaDO(2)) at an inspired oxygen fraction (FiO(2)) of 0.21, lung scintiscan, and contrast transthoracic echocardiography (TTE). Three of them had a liver transplantation because of the downhill course of their liver disease and respiratory status. THT transcutaneous O(2) tension at 21% FiO(2) (TcPO(2)21) was 75 +/- 13 mm Hg, and increased to 488 +/- 106 mmHg after 100% O(2) breathing (TcPO(2)100). Both mean values were not significantly different from those found in 8 age-matched controls (P = 0.9 and P = 0.5, respectively). However, one patient, in spite of her stable liver function, showed an abnormal TcPO(2)21 and TcPO(2)100 (45 mmHg and 210 mmHg, respectively). This same subject was also the only patient with abnormalities of AaDO(2) (54.2 mm Hg; normal value, < 20 mm Hg), lung scintiscan (brain/lung ratio of technetium-99 fixation (B/L SI) = 9, normal value < 1), and TTE, suggesting intrapulmonary vasodilatations and shunts. Given the clinical development of cyanosis and platypnea, all criteria for HPS were fulfilled, and timing of her liver transplantation was therefore accelerated. This resulted in HPS regression. In children with chronic cholestasis, repeated transcutaneous bedside measurements are a rapid and reliable noninvasive test for characterizing the severity of abnormal oxygenation, and may prove useful also in liver posttransplantation monitoring.
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31
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Nakamura CT, Ng GY, Paton JY, Keens TG, Witmer JC, Bautista-Bolduc D, Woo MS. Correlation between digital clubbing and pulmonary function in cystic fibrosis. Pediatr Pulmonol 2002; 33:332-8. [PMID: 11948977 DOI: 10.1002/ppul.10082] [Citation(s) in RCA: 18] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
The correlation between digital clubbing and certain pulmonary function derangements (hypoxemia and FEV(1)) was previously described. However, the relationship between digital clubbing and other measures of pulmonary function or the presence of liver disease in patients with cystic fibrosis (CF) is poorly defined. Hence we compared the digital clubbing index (CI: ratio of distal phalangeal depth to interphalangeal depth) of 100 patients with CF (43 males, 57 females; mean age, 15.7 +/- 7.3 years) with that of 100 age- and gender-matched healthy controls. Digital clubbing was defined as a CI > or = 1.00 (mean + 2.6 SD; 99% of normal subjects). The CI and its relationship to pulmonary function and to liver disease was then evaluated in the CF patients. Digital clubbing was present in 75/100 (75%) of CF patients but was absent in all controls (P < 0.0001). In CF patients, CI was inversely correlated with PaO(2) (r = -0.555; P < 0.001), FEV(1) (r = -0.499; P < 0.001), and FEF(25-75%) (r = -0.404; P < 0.001), and was positively correlated with RV (r = 0.285; P < 0.05) and the slope of phase 3 of single-breath nitrogen washout (SP3N(2)) (r = 0.532; P < 0.01). There was no significant correlation between CI and age (r = 0.020; P = 0.84), TLC (r = -0.097; P = 0.34), PaCO(2) (r = 0.167; P = 0.10), or history of liver disease (P = 0.08). We conclude that in CF, the degree of digital clubbing is related to degree of hypoxemia, airways obstruction, hyperinflation, and nonuniformity of ventilation.
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Affiliation(s)
- Craig T Nakamura
- Division of Pediatric Pulmonology, Childrens Hospital Los Angeles, Keck School of Medicine of the University of Southern California, Los Angeles, California 90027, USA
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32
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Martínez GP, Barberà JA, Visa J, Rimola A, Paré JC, Roca J, Navasa M, Rodés J, Rodriguez-Roisin R. Hepatopulmonary syndrome in candidates for liver transplantation. J Hepatol 2001; 34:651-7. [PMID: 11434610 DOI: 10.1016/s0168-8278(00)00108-2] [Citation(s) in RCA: 162] [Impact Index Per Article: 6.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
BACKGROUND Hepatopulmonary syndrome (HPS) has been defined as a clinical triad, including chronic liver disease, gas exchange defects (increased alveolar-arterial PO2 difference irrespective of the presence of arterial hypoxemia), and widespread intrapulmonary vascular dilatations. We determined the incidence and the clinical and pulmonary functional characteristics of HPS in candidates for orthotopic liver transplantation (OLT) and tested their predicted accuracy. METHODS We studied 80 patients with cirrhosis prospectively, and carried out contrast-enhanced (CE) echocardiography and lung function tests, including ventilation-perfusion (V(A)/Q) distributions. RESULTS Fourteen patients had HPS (incidence, 17.5%). Patients with HPS (49 +/- 12 (+/-SD) years) had more cutaneous spiders, finger clubbing and dyspnea (P < 0.05 each) and a lower diffusing capacity (DLCO, 56 +/- 18% predicted; P < 0.001) than non-HPS patients (n = 66). Mild to moderate V(A)/Q inequalities and increased intrapulmonary shunt were predominant in HPS patients, but oxygen diffusion impairment was observed in those with hypoxemia (n = 8) only. The DLCO showed a considerable area under the receiver operating characteristic curve (0.89). CONCLUSIONS HPS in cirrhotic patient candidates for OLT shows a high incidence and these patients present with distinctive clinical and functional features compared with non-HPS individuals. The presence of a low DLCO may be of help for the diagnosis of HPS.
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Affiliation(s)
- G P Martínez
- Servei de Anestesiologia i Reanimació, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Hospital Clinic, Universitat de Barcelona, Spain
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33
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Nelson RS, Eichinger MR. Role of nitric oxide (NO) in pulmonary dysfunction associated with experimental cirrhosis. RESPIRATION PHYSIOLOGY 2001; 126:65-74. [PMID: 11311311 DOI: 10.1016/s0034-5687(00)00227-9] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/17/2022]
Abstract
We examined the functional role of nitric oxide (NO) and nitric oxide synthase (NOS) isoforms in the pulmonary dysfunction seen in cirrhosis. Lungs were isolated from control and carbon tetrachloride (CCl(4))-induced cirrhotic rats and perfused at constant flow with a whole blood mixture. Ventilation with hypoxic gas resulted in attenuated hypoxic pulmonary vasoconstriction (HPV) in lungs from cirrhotic animals. Administration of the non-selective NOS inhibitor N-omega-Nitro-L-Arginine (L-NNA) resulted in HPV responses that were not different between groups. However, inhibition of inducible nitric oxide synthase (iNOS) did not restore cirrhotic HPV responses. Lungs from cirrhotic rats demonstrated enhanced endothelial-dependent vasodilation to vasopressin when preconstricted with hypoxia but not when preconstricted with thromboxane mimetic. Western blot analysis failed to demonstrate differences in pulmonary endothelial NOS (eNOS) or iNOS levels between groups. Our data suggest that, while NO may play a role in mediating the reduced pulmonary vasoreactivity observed in cirrhosis, other vasoactive factors are likely also important modulators of the pulmonary dysfunction seen in this disease.
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Affiliation(s)
- R S Nelson
- Department of Anesthesiology, University of New Mexico Health Sciences Center, Albuquerque, NM 87131, USA
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Regev A, Yeshurun M, Rodriguez M, Sagie A, Neff GW, Molina EG, Schiff ER. Transient hepatopulmonary syndrome in a patient with acute hepatitis A. J Viral Hepat 2001; 8:83-6. [PMID: 11155156 DOI: 10.1046/j.1365-2893.2001.00270.x] [Citation(s) in RCA: 68] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
The hepatopulmonary syndrome is defined as the triad of liver disease, hypoxaemia and intrapulmonary vascular dilatation. This syndrome has been described in patients with liver cirrhosis, noncirrhotic portal hypertension, and fulminant hepatic failure, however, there are no previous descriptions of hepatopulmonary syndrome in patients with acute nonfulminant viral hepatitis. We report a 47-year-old, previously healthy man that presented with acute hepatitis A, and developed progressive dyspnoea, platypnoea and orthodeoxia with no evidence of parenchymal or thromboembolic lung disease. PaO2 on room air was 58 mmHg, O2 saturation was 88% and alveolar-arterial O2 gradient was 62%. During his hospitalization serum albumin level decreased to 3.1 g/dl and prothrombin time was prolonged to 16.8 s, however, he remained alert with no signs of hepatic encephalopathy. Contrast echocardiography revealed left heart chamber opacification 3-4 cardiac cycles after the opacification of the right heart chamber, consistent with hepatopulmonary syndrome. During the following days there was a gradual improvement in the patient's condition, with resolution of his dyspnoea and gradual increase of PaO2. Repeat contrast echocardiography and PaO2 determinations, 3 weeks later, were normal. On long-term follow-up the patient remained asymptomatic with normal liver function tests and normal O2 saturation. This report indicates that hepatopulmonary syndrome may be a transient manifestation of acute hepatitis A in the absence of fulminant liver failure.
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Affiliation(s)
- A Regev
- Center for Liver Diseases, Division of Hepatology, University of Miami School of Medicine, Miami, FL 33136, USA
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Abstract
The hepatopulmonary syndrome is a triad of liver disease, increased alveolar-arterial oxygen gradient and intrapulmonary vascular dilatations. Manifestations include orthodeoxia, platypnoea and hyperdynamic circulation. Intrapulmonary vascular abnormalities, perhaps mediated by nitric oxide, cause hypoxaemia by shunting, a perfusion-diffusion defect, and ventilation-perfusion mismatching. Contrast-enhanced echocardiography is the method of choice for demonstrating pulmonary vascular abnormalities, although perfusion lung scanning is a more specific and sensitive test. Angiography is best reserved for patients with poor response to 100% oxygen and defines whether vascular dilatations are of the diffuse 'spongy' type or, less commonly, discrete arteriovenous communications amenable to embolization. About 80% of patients with the hepatopulmonary syndrome eventually have improved oxygenation after liver transplantation, thereby making worsening hypoxaemia the primary indication for transplantation in many instances. Nevertheless, severe hypoxaemia carries a peri-operative mortality of 30% and reliable predictors of successful outcome after transplantation remain to be determined.
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Affiliation(s)
- L S Aboussouan
- Division of Pulmonary and Critical Care Medicine, Wayne State University School of Medicine, Harper Hospital, 3-Hudson, 3990 John R, Detroit, MI 48201, USA
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Bachmann-Brandt S, Bittner I, Neuhaus P, Frei U, Schindler R. Plasma levels of endothelin-1 in patients with the hepatorenal syndrome after successful liver transplantation. Transpl Int 2000. [DOI: 10.1111/j.1432-2277.2000.tb01010.x] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
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Abstract
Increasing numbers of individuals leading normal lives have transplanted organs. They may appear in any hospital for treatment of trauma or general diseases. Common anaesthesia methods can be used for these patients, but safe conduct of anaesthesia requires knowledge of the immunosuppression, risk factors, and altered physiology or drug actions. This article reviews the anaesthesia-related literature on patients with transplanted organs.
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Affiliation(s)
- H J Toivonen
- Department of Anaesthesia, University of Helsinki, Finland.
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Taler SJ, Textor SC, Canzanello VJ, Schwartz L, Porayko MK, Wiesner RH, Krom RA. Hypertension after liver transplantation: a predictive role for pretreatment hemodynamics and effects of isradipine on the systemic and renal circulations. Am J Hypertens 2000; 13:231-9. [PMID: 10777026 DOI: 10.1016/s0895-7061(99)00171-5] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022] Open
Abstract
Hypertension developing after liver transplantation during immunosuppression with cyclosporine A reflects an unusual hemodynamic transition from peripheral vasodilation to systemic and renal vasoconstriction. Although dihydropyridine calcium channel blockers are often administered for their efficacy in promoting vasodilation, some liver transplant recipients report marked symptomatic intolerance to these agents. In the present study we examined systemic and renal responses to isradipine using systemic (thoracic bioimpedance) and renal hemodynamic measurements in 15 liver transplant recipients studied at the time of initial diagnosis of posttransplant hypertension and after 3 months of treatment. Circadian blood pressure patterns were examined by overnight ambulatory blood pressure monitoring before and during antihypertensive therapy. During isradipine administration, blood pressure decreased from 151 +/- 3/91 +/- 2 to 130 +/-3/81 +/- 2 mm Hg (P < .01) without change in renal blood flow (406 +/- 43 to 425 +/- 52 mL/min/1.73m2, P = NS) or renal vascular resistance index (25,674 +/-3312 to 20,520 +/- 2311 dynes x sec x cm(-5)/m2, P = NS). Pre-treatment differences in systemic vascular tone persisted during treatment and predicted the tendency for symptomatic tachycardia and flushing, predominantly in those with hyperdynamic circulations. Twice daily dosing of isradipine was associated with partial and significant restoration of the nocturnal decrease in blood pressure (systolic blood pressure decreased 5.5%, normal 13%), usually absent early after transplantation. Our results demonstrate the ability of hemodynamic measurements to predict the symptomatic response to antihypertensive therapy in the posttransplant setting.
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Affiliation(s)
- S J Taler
- Department of Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
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Glattki GP, Maurer C, Satake N, Lange R, Metz K, Wettstein M, Costabel U. [Hepatopulmonary syndrome]. MEDIZINISCHE KLINIK (MUNICH, GERMANY : 1983) 1999; 94:505-12. [PMID: 10544613 DOI: 10.1007/bf03044942] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
ANAMNESIS We report about a 60-year-old woman with hepatopulmonary syndrome. The patient suffered from progressive dyspnea and liver cirrhosis following viral hepatitis C, acquired through blood transfusion 30 years ago. EXAMINATION RESULTS Remarkable were clinical signs of chronic liver disease (cutaneous spider naevi, palmar erythema) and signs of chronic respiratory failure (clubbing) with platypnea. Arterial pO2 was 33 mm Hg under 41 O2/min nasal oxygen supplement and improved to 74 mm Hg under 81 O2/min. Arterial pO2 deteriorated in upright position (orthodeoxia). Contrast enhanced two-dimensional transthoracic echocardiography showed a delayed microbubble opacification in the left heart chambers (without atrial or ventricular septal defect), suggesting pulmonary vascular dilatations. Perfusion scan with macroaggregated albumin labelled with technetium-99m showed uptake over thyroid, kidneys, liver, and spleen suggesting an abnormal passage through the pulmonary vascular bed. The radiological examination with chest radiography and high resolution computer tomography revealed no signs of interstitial lung disease. The pulmonary artery angiography showed neither anatomical shunts nor malformations. DIAGNOSIS The final diagnosis was hepatopulmonary syndrome Type 1. TREATMENT/FOLLOW-UP: The patient underwent liver transplantation with following good function of the liver graft. After 6 months the patient presented in a much better general condition, but with persisting severe hypoxemia whereas the spider naevi disappeared completely. One year after the liver transplantation in the perfusion scan the uptake over thyroid, kidneys, liver, and spleen was absent. The orthodeoxia and hypoxemia improved evidently. From that we conclude that the pulmonary vascular dilatations diminished after liver transplantation.
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Affiliation(s)
- G P Glattki
- Abteilung für Pneumologie/Allergologie, Ruhrlandklinik Essen-Heidhausen
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Kostopanagiotou G, Smyrniotis V, Arkadopoulos N, Theodoraki K, Papadimitriou L, Papadimitriou J. Anesthetic and Perioperative Management of Adult Transplant Recipients in Nontransplant Surgery. Anesth Analg 1999. [DOI: 10.1213/00000539-199909000-00013] [Citation(s) in RCA: 42] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/24/2023]
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Kostopanagiotou G, Smyrniotis V, Arkadopoulos N, Theodoraki K, Papadimitriou L, Papadimitriou J. Anesthetic and perioperative management of adult transplant recipients in nontransplant surgery. Anesth Analg 1999; 89:613-22. [PMID: 10475290 DOI: 10.1097/00000539-199909000-00013] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/24/2023]
Affiliation(s)
- G Kostopanagiotou
- Anesthesiology Unit, Aretaieion Hospital, University of Athens School of Medicine, Greece.
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Martinez G, Barberà JA, Navasa M, Roca J, Visa J, Rodriguez-Roisin R. Hepatopulmonary syndrome associated with cardiorespiratory disease. J Hepatol 1999; 30:882-9. [PMID: 10365816 DOI: 10.1016/s0168-8278(99)80143-3] [Citation(s) in RCA: 86] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/30/2023]
Abstract
BACKGROUND/AIMS Hepatopulmonary syndrome is defined as a clinical triad including chronic liver disease, abnormal pulmonary gas exchange resulting ultimately in profound arterial hypoxaemia, and evidence of intrapulmonary vascular dilatations. We report five patients with liver cirrhosis diagnosed with hepatopulmonary syndrome who had associated chronic obstructive or restrictive respiratory diseases. METHODS Clinical, radiographic and constrast-enhanced echocardiographic findings, and systemic and pulmonary haemodynamic and gas exchange, including ventilation-perfusion distributions, measurements were assessed in all five patients. RESULTS Echocardiography was consistent with the presence of intrapulmonary vasodilation without intracardiac abnormalities, and high resolution computed tomographic scan features were compatible with clinical (3 cases) or histopathological diagnoses (2 cases) of associated respiratory disorders. The most common prominent functional findings were moderate to severe arterial hypoxaemia, caused by moderately to severely increased intrapulmonary shunting and/or mild to moderate low ventilation-perfusion areas, and hypocarbia along with an increased cardiac output and a low pulmonary artery pressure and vascular resistance. CONCLUSIONS These functional characteristics, classically reported in the setting of clinically stable, uncomplicated hepatopulmonary syndrome, conform to a distinctively unique, chronic gas exchange pattern. Equally important, these pulmonary haemodynamic-gas exchange hallmarks are not influenced by the co-existence of chronic cardiorespiratory disease states. These data may have clinical relevance for elective indication of hepatic transplantation in patients with life-threatening hepatopulmonary syndrome.
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Affiliation(s)
- G Martinez
- Institut Clínic de Malalties Digestives, Departament de Medicina, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Hospital Clínic, Universitat de Barcelona, Spain
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Lee KN, Lee HJ, Shin WW, Webb WR. Hypoxemia and liver cirrhosis (hepatopulmonary syndrome) in eight patients: comparison of the central and peripheral pulmonary vasculature. Radiology 1999; 211:549-53. [PMID: 10228541 DOI: 10.1148/radiology.211.2.r99ma46549] [Citation(s) in RCA: 72] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
PURPOSE To evaluate the pulmonary vasculature in patients with hepatopulmonary syndrome. MATERIALS AND METHODS Conventional computed tomographic (CT) scans in eight patients with hepatopulmonary syndrome were retrospectively evaluated to compare the diameters of the pulmonary trunk, right and left main pulmonary arteries, and peripheral pulmonary vasculature in the right posterior basal segment with those in eight healthy subjects and in four patients with normoxemic cirrhosis. With thin-section CT, the ratio of segmental arterial diameter to adjacent bronchial diameter in the right lower lobe in four patients with hepatopulmonary syndrome was compared with that in four patients with normoxemic cirrhosis. RESULTS In patients with hepatopulmonary syndrome, the peripheral pulmonary vasculature was significantly dilated compared with that in control subjects and in patients with normoxemic cirrhosis (P = .002); however, the central pulmonary arteries were not significantly dilated (P > .05). At thin-section CT, the ratio of segmental arterial diameter to adjacent bronchial diameter was significantly greater than that in patients with normoxemic cirrhosis (P < .05). CONCLUSION In patients with hepatopulmonary syndrome, the peripheral pulmonary vasculature is significantly dilated. Dilatation of the peripheral pulmonary vasculature may be helpful in the diagnosis of hepatopulmonary syndrome.
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Affiliation(s)
- K N Lee
- Department of Diagnostic Radiology, Dong-A University College of Medicine, Seo-Ku, Pusan, Korea
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44
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Abstract
The hepatopulmonary syndrome is a disease entity seen in association with liver failure and other disease entities. It is a devastating consequence of liver failure that results in a significant morbidity for affected patients. Currently, there are no identified medications that ameliorate the symptoms of hypoxemia in this disease state. Recent research, however, has begun to unravel the pathobiology of the vascular dilations that arise in the lungs of patients with liver failure. In this article, a compendium of current knowledge is presented, as well as the contemporary methods for identifying and treating patients.
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Affiliation(s)
- V L Scott
- Thomas E. Starzl Transplantation Institute, Pittsburgh, Pennsylvania, USA
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Mimidis KP, Karatza C, Spiropoulos KV, Toulgaridis T, Charokopos NA, Thomopoulos KC, Margaritis VG, Nikolopoulou VN. Prevalence of intrapulmonary vascular dilatations in normoxaemic patients with early liver cirrhosis. Scand J Gastroenterol 1998; 33:988-992. [PMID: 9759957 DOI: 10.1080/003655298750027038] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
BACKGROUND The aim of this study was to determine the prevalence of intrapulmonary vascular dilatations (IPVD) in normoxaemic patients with early liver cirrhosis and to compare their occurrence in progressive alcoholic versus postviral hepatic insufficiency. METHODS Pulmonary function tests and arterial blood gas measurements were performed in 75 consecutive patients with cirrhosis of alcoholic and postviral aetiology. Contrast-enhanced echocardiography was used to identify IPVD. RESULTS All patients were grade A or B in accordance with the Child-Pugh modified classification. Arterial blood gas analyses showed normoxaemia in all patients. Eight of 75 patients (10.7%) had a positive contrast echocardiogram, all with a decreased diffusion capacity (D1CO < 75% of the expected value). The abnormality was more prominent with advancing stage of liver failure (4.5% in grade A versus 19.4% in grade B; P < 0.05) and more common in patients with alcoholic cirrhosis (17.5% in alcoholic versus 2.9% in postviral cirrhosis; P < 0.05). CONCLUSION In normoxaemic patients with early liver cirrhosis subclinical pulmonary vasodilatation, as assessed with contrast echocardiography, can occur. The finding is more prominent in alcoholic cirrhosis and possibly reflects an advancing degree of liver insufficiency.
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Affiliation(s)
- K P Mimidis
- Dept. of Internal Medicine, University Hospital, Patras, Greece
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Tromm A, Micklefield GH, Hüppe D. [Bronchopulmonary manifestations of gastroenterologic and hepatic diseases]. MEDIZINISCHE KLINIK (MUNICH, GERMANY : 1983) 1997; 92:720-5. [PMID: 9483915 DOI: 10.1007/bf03044668] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Topics of this review are the bronchopulmonary manifestations of gastroesophageal reflux disease, cirrhosis of the liver and chronic inflammatory bowel diseases. About 20% of patients with chronic obstructive airway disease show evidence of gastroesophageal reflux disease. Reflux bronchoconstriction seems to be of greater importance than microaspiration. First studies show the positive effects of acid inhibition by proton pump inhibitors on pulmonary symptoms. Hepatorenal syndrome is characterized by arterial hypoxemia with PaO2-values < 70 mm Hg. Different mediators (endotoxins, amines, polypeptides or allergens) are discussed. Furthermore, elevated levels of prostacycline, atrial natriuretic factor and platelet activating factor have been described. Recently published studies focused on the role of nitric oxide (NO). Patients with cirrhosis of the liver show a higher rate of a pathologically elevated airway resistance which might be induced by a reduced histamine clearance. Ascites leads to reversible restrictive airway disease. Bronchopulmonary manifestations in chronic inflammatory bowel diseases include obstructive and restrictive airway diseases, vascular or serosal changes and show low clinical evidence. In contrast, pathological changes of the common function tests were found in 30 to 50%. These findings may be induced by circulating immune complexes, vasculitis, increased permeability or a combined immune reaction of both, the bronchial and intestinal mucosa. Undesired effects of salicylates should be taken into account. This review shows that bronchopulmonary manifestations in diseases of the Gl-tract or the liver are more common than usually known and should be taken into clinical consideration.
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Affiliation(s)
- A Tromm
- Abteilung für Gastroenterologie und Hepatologie, Bergmannsheil-Universitätsklinik Bochum
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Vachiéry F, Moreau R, Hadengue A, Gadano A, Soupison T, Valla D, Lebrec D. Hypoxemia in patients with cirrhosis: relationship with liver failure and hemodynamic alterations. J Hepatol 1997; 27:492-5. [PMID: 9314126 DOI: 10.1016/s0168-8278(97)80353-4] [Citation(s) in RCA: 57] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
BACKGROUND/AIMS The relationship between hypoxemia, liver failure and the hemodynamic alterations in cirrhosis are unknown. This study examined the relationship between arterial hypoxemia, the severity of liver disease and hyperkinetic circulation in patients with cirrhosis. METHODS Arterial blood gases, the severity of cirrhosis (Child-Pugh score), and splanchnic and systemic hemodynamics were measured in 120 patients with cirrhosis and without cardiopulmonary disease. Hypoxemia was considered to be present when PaO2 was < or = 70 mmHg. RESULTS Seventeen patients had hypoxemia (14%). Hypoxemic patients had significantly lower pulmonary vascular resistance and a significantly higher alveolar-arterial oxygen gradient, Child-Pugh score and hepatic venous pressure gradient than non-hypoxemic patients. Cardiac index and right atrial and pulmonary pressures did not significantly differ between the two groups. CONCLUSIONS Hypoxemia occurs mainly in patients with severe liver disease and is associated with pulmonary vasodilation.
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Affiliation(s)
- F Vachiéry
- Laboratoire d'Hémodynamique Splanchnique et de Biologie Vasculaire, Unité de Recherches de Physiopathologie Hépatique (INSERM U-24), Hôpital Beaujon, Clichy, France
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Abstract
The hepatopulmonary syndrome occurs in subjects with chronic liver disease and/or portal hypertension who develop intrapulmonary vasodilation resulting in arterial deoxygenation. Clinical and basic science studies investigating the pathophysiology of HPS are presented. A diagnostic algorithm is provided using contrast echocardiography, the lung perfusion scan, and pulmonary angiography. Medical therapy and experience with liver transplantation are reviewed.
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Affiliation(s)
- G A Abrams
- UAB Liver Center, Division of Gastroenterology and Hepatology, University of Alabama at Birmingham, Birmingham, Alabama 35294-0007, USA
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49
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Uemoto S, Inomata Y, Egawa H, Satomura K, Kiuchi T, Okajima H, Asonuma K, Sano K, Uyama S, Tanaka K. Effects of hypoxemia on early postoperative course of liver transplantation in pediatric patients with intrapulmonary shunting. Transplantation 1997; 63:407-14. [PMID: 9039932 DOI: 10.1097/00007890-199702150-00014] [Citation(s) in RCA: 38] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Abstract
Nine pediatric patients (mean age, 10 years) with biliary atresia, who had hypoxemia related to intrapulmonary shunting, underwent living related liver transplantation. The effects of hypoxemia during the early postoperative period after liver transplantation on cardiopulmonary and renal function, as well as on transplanted liver, were analyzed. Based on the degree of shunt ratio calculated by technetium-99m macroaggregated albumin scintigraphy, the nine patients were included in the moderate group (shunt ratio under 40%, n=4) or the severe group (shunt ratio over 40%, n=5). Partial pressure of arterial oxygen was maintained at normal range in the moderate group, while that in the severe group persistently had very low values (<50 mmHg), in spite of a high degree of oxygen supply. However, all patients in the severe group maintained stable cardiopulmonary vital signs, including systemic blood pressure, heart rate, respiratory rate, and cardiac index. They also demonstrated stable renal function. None of the patients died of cardiopulmonary or renal insufficiency after transplantation, but three patients died of portal vein thrombosis, sepsis, and intracranial hemorrhage (one each). The minimal adverse effect of hypoxemia on the transplanted liver was confirmed by a rapid increase of arterial ketone body ratio, low peak values (under 200 IU/L) of aspartate aminotransferase, and a steady decrease of serum total bilirubin. Four patients encountered surgical complications, including two bile leaks from the cut liver surface, two leaks from bilioenteric anastomosis, and one intestinal perforation. Six patients suffered from bacterial infections, including four wound infections, three right subphrenic abscesses, one cholangitis, and two systemic sepses. All patients in the moderate group recovered from hypoxemia, but four of five patients in the severe group have not recovered during the follow-up period between 4 and 9 months. It was concluded that the adverse effects of hypoxemia on cardiopulmonary and renal function and transplanted liver were minimal, so that patients with severe hypoxemia could tolerate the stress of liver transplantation without special management. However, the high incidence of surgical complication and infection suggested the adverse effects of hypoxemia on wound healing and resistance to bacteria infection.
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Affiliation(s)
- S Uemoto
- Department of Transplantation Immunology, Faculty of Medicine, Kyoto University, Japan
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50
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Krowka MJ, Porayko MK, Plevak DJ, Pappas SC, Steers JL, Krom RA, Wiesner RH. Hepatopulmonary syndrome with progressive hypoxemia as an indication for liver transplantation: case reports and literature review. Mayo Clin Proc 1997; 72:44-53. [PMID: 9005286 DOI: 10.4065/72.1.44] [Citation(s) in RCA: 149] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Abstract
In the hepatopulmonary syndrome (HPS), a pulmonary vascular complication of liver disease, severe hypoxemia due to pulmonary vascular dilatation can be extremely debilitating. Determining whether patients with advanced liver disease and HPS should be considered for liver transplantation is difficult. We describe three patients with progressive and severe hypoxemia who underwent successful liver transplantation and had resolution of their arterial hypoxemia. In these patients, the progressive pulmonary deterioration accelerated the need and was considered an indication for liver transplantation rather than being considered an absolute or relative contraindication. In addition, we review the literature on 81 pediatric and adult patients with HPS who underwent liver transplantation and specifically highlight mortality, morbidity, syndrome resolution, and prognostic factors. Posttransplantation mortality (16%) was associated with the severity of hypoxemia (mean arterial oxygen tension [PaO2] in 68 survivors was 54.2 +/- 13.2 mm Hg and in 13 nonsurvivors was 44.7 +/- 7.7 mm Hg; P<0.03). Patients with a pretransplantation PaO2 of 50 mm Hg or lower had significantly more frequent mortality (30%) in comparison with those with a PaO2 greater than 50 mm Hg (4%; P<0.02). Pulmonary recommendations that address the severity of hypoxemia and candidacy for liver transplantation are discussed.
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Affiliation(s)
- M J Krowka
- Division of Pulmonary and Critical Care Medicine, Mayo Clinic Rochester, Minnesota 55905, USA
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