|
1
|
McGlothlin D, Granton J, Klepetko W, Beghetti M, Rosenzweig EB, Corris P, Horn E, Kanwar M, McRae K, Roman A, Tedford R, Badagliacca R, Bartolome S, Benza R, Caccamo M, Cogswell R, Dewachter C, Donahoe L, Fadel E, Farber HW, Feinstein J, Franco V, Frantz R, Gatzoulis M, Hwa (Anne) Goh C, Guazzi M, Hansmann G, Hastings S, Heerdt P, Hemnes A, Herpain A, Hsu CH, Kerr K, Kolaitis N, Kukreja J, Madani M, McCluskey S, McCulloch M, Moser B, Navaratnam M, Radegran G, Reimer C, Savale L, Shlobin O, Svetlichnaya J, Swetz K, Tashjian J, Thenappan T, Vizza CD, West S, Zuckerman W, Zuckermann A, De Marco T. ISHLT CONSENSUS STATEMENT: Peri-operative Management of Patients with Pulmonary Hypertension and Right Heart Failure Undergoing Surgery. J Heart Lung Transplant 2022; 41:1135-1194. [DOI: 10.1016/j.healun.2022.06.013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/31/2022] [Accepted: 06/13/2022] [Indexed: 10/17/2022] Open
|
|
2
|
Soulaidopoulos S, Goulis I, Cholongitas E. Pulmonary manifestations of chronic liver disease: a comprehensive review. Ann Gastroenterol 2020; 33:237-249. [PMID: 32382226 PMCID: PMC7196609 DOI: 10.20524/aog.2020.0474] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/22/2019] [Accepted: 12/06/2019] [Indexed: 12/14/2022] Open
Abstract
Hepatopulmonary syndrome (HPS) and porto-pulmonary hypertension (PoPH) represent relatively common pulmonary vascular complications of advanced liver disease. Despite distinct differences in their pathogenetic background, both clinical states are characterized by impaired arterial oxygenation and limited functional status, and are associated with increased pre-transplantation mortality. Accumulation of ascitic fluid in the pleural cavity, known as hepatic hydrothorax (HH), is another frequent manifestation of decompensated cirrhosis, which may cause severe respiratory dysfunction, depending on the volume of the effusion, the rapidity of its development and its resistance to therapeutic measures. Orthotopic liver transplantation constitutes the only effective treatment able to resolve the pulmonary complications of liver disease. A prioritization policy for liver transplantation has evolved over the past years regarding advanced stages of HPS, yielding favorable outcomes regarding post-transplantation survival and HPS resolution. In contrast, severe PoPH is associated with poor post-transplantation survival. Hence, liver transplantation is recommended only for patients with PoPH and an acceptable reduction in pulmonary pressure values, after receiving PoPH-targeted vasodilating therapy. This review focuses on basic pathogenetic and diagnostic principles and discusses the current therapeutic approaches regarding HPS, PoPH, and HH.
Collapse
Affiliation(s)
- Stergios Soulaidopoulos
- First Department of Cardiology, Hippokration General Hospital, National and Kapodistrian University of Athens (Stergios Soulaidopoulos)
| | - Ioannis Goulis
- Fourth Department of Internal Medicine, Hippokration General Hospital, Medical School of Aristotle University of Thessaloniki (Ioannis Goulis)
| | - Evangelos Cholongitas
- First Department of Internal Medicine, Laiko General Hospital, Medical School of National and Kapodistrian University of Athens (Evangelos Cholongitas), Greece
| |
Collapse
|
|
3
|
Iqbal S, Smith KA, Khungar V. Hepatopulmonary Syndrome and Portopulmonary Hypertension: Implications for Liver Transplantation. Clin Chest Med 2017; 38:785-795. [PMID: 29128026 DOI: 10.1016/j.ccm.2017.08.002] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) represent serious pulmonary complications of advanced liver diseases. Orthotopic liver transplantation (OLT) is capable of completely resolving the underlying abnormalities associated with HPS. On the other hand, post-OLT response in patients with PoPH is less predictable, although heavily influenced by pre-OLT mean pulmonary arterial pressure. It remains the case that the opportunity to reverse 2 potentially fatal organ dysfunctions in the liver and the lung make HPS and PoPH more than worthy for further clinical investigations.
Collapse
Affiliation(s)
- Shaz Iqbal
- Department of Medicine, General Internal Medicine Division, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104, USA
| | - Kerri Akaya Smith
- Department of Medicine, Pulmonary, Allergy, and Critical Care Division, Perelman School of Medicine, University of Pennsylvania, 3400 Spruce Street, 834 West Gates Building, Philadelphia, PA 19104, USA
| | - Vandana Khungar
- Department of Medicine, Division of Gastroenterology and Hepatology, Perelman School of Medicine, University of Pennsylvania, 3400 Spruce Street 2 Dulles, Philadelphia, PA 19104, USA.
| |
Collapse
|
|
4
|
Abstract
Portopulmonary hypertension (POPH) is a form of pulmonary arterial hypertension occurring in the setting of portal hypertension with or without hepatic cirrhosis. The presence of both portal and pulmonary vascular disease contributes to complicated hemodynamics and therapeutic challenges, though the severities do not appear to correlate directly. Diagnosis of POPH, and distinction from the commonly observed hyperdynamic state of end-stage liver disease, is typically accomplished with an initial screening transthoracic echocardiogram, followed by right heart catheterization for confirmation of hemodynamic parameters. Though few studies have directly evaluated use in POPH, pulmonary artery-directed therapy is the cornerstone of management, along with consideration of liver transplantation. Perioperative and long-term outcomes are variable, but uniformly worse in the setting of uncontrolled pulmonary pressures. Risk stratification and optimal patient selection for these interventions are areas of ongoing investigation.
Collapse
|
|
5
|
|
|
6
|
Abstract
Perioperative pulmonary hypertension can originate from an established disease or acutely develop within the surgical setting. Patients with increased pulmonary vascular resistance are consequently at greater risk for complications. Despite the various specific therapies available, the ideal therapeutic approach in this patient population is not currently clear. This article describes the basic principles of perioperative pulmonary hypertension and reviews the different classes of agents used to promote pulmonary vasodilation in the surgical setting.
Collapse
|
|
7
|
Kähler CM, Graziadei I, Vogelsinger H, Desole S, Cima K, Vogel W. Successful treatment of portopulmonary hypertension with the selective endothelin receptor antagonist Sitaxentan. Wien Klin Wochenschr 2011; 123:248-52. [DOI: 10.1007/s00508-011-1540-4] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/12/2010] [Accepted: 11/24/2010] [Indexed: 01/09/2023]
|
|
8
|
Halank M, Ewert R, Seyfarth HJ, Hoeffken G. Portopulmonary hypertension. J Gastroenterol 2006; 41:837-47. [PMID: 17048047 DOI: 10.1007/s00535-006-1879-x] [Citation(s) in RCA: 17] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/29/2005] [Accepted: 07/25/2006] [Indexed: 02/04/2023]
Abstract
Portopulmonary hypertension (PPHT) is defined as precapillary pulmonary hypertension accompanied by hepatic disease or portal hypertension. Pulmonary hypertension results from excessive pulmonary vascular remodeling and vasoconstriction. These histological alterations have been indistinguishable from those of other forms of pulmonary arterial hypertension. Factors involved in the pathogenesis of PPHT include volume overload, hyperdynamic circulation, and circulating vasoactive mediators. The disorder has a substantial impact on survival and requires focused treatment. Liver transplantation in patients with moderate to severe PPHT is associated with a significantly reduced survival rate. The best medical treatment for patients with PPHT is controversial; most authors currently regard continuous intravenous application of prostacyclin as the treatment of choice for patients with severe PPHT. There is only very limited reported experience with inhaled prostacyclin or its analog, iloprost. Increasing evidence of the efficacy of the endothelin-receptor antagonist bosentan and of the phosphodiesterase-5 inhibitor sildenafil is emerging in highly selected patients with PPHT. In the future, a combination therapy of the above-mentioned agents might become a therapeutic option. Other agents such as beta-blockers seem to be harmful to patients with moderate to severe portopulmonary hypertension. Up-to-date, randomized, double-blind, controlled clinical trials are lacking and are needed urgently.
Collapse
Affiliation(s)
- Michael Halank
- Carl Gustav Carus University Dresden, Internal Medicine I, Fetscherstr. 74, 01307 Dresden, Germany
| | | | | | | |
Collapse
|
|
9
|
Ramsay MA. Portopulmonary Hypertension and Hepatopulmonary Syndrome, and Liver Transplantation. Int Anesthesiol Clin 2006; 44:69-82. [PMID: 16832207 DOI: 10.1097/01.aia.0000210800.60630.ac] [Citation(s) in RCA: 17] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/15/2023]
Affiliation(s)
- Michael A Ramsay
- Department of Anesthesiology, Baylor University Medical Center, Dallas, TX 75246, USA
| |
Collapse
|
|
10
|
Sandifer BL, Brigham KL, Lawrence EC, Mottola D, Cuppels C, Parker RE. Potent effects of aerosol compared with intravenous treprostinil on the pulmonary circulation. J Appl Physiol (1985) 2005; 99:2363-8. [PMID: 16141385 DOI: 10.1152/japplphysiol.00083.2005] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
Abstract
Inhaled vasodilator therapy for pulmonary hypertension may decrease the systemic side effects commonly observed with systemic administration. Inhaled medications only reach ventilated areas of the lung, so local vasodilation may improve ventilation-perfusion matching and oxygenation. We compared the effects of intravenous vs. aerosolized treprostinil on pulmonary and systemic hemodynamics in an unanesthetized sheep model of sustained acute pulmonary hypertension. Acute, stable pulmonary hypertension was induced in instrumented unanesthetized sheep by infusing a PGH(2) analog, U-44069. The sheep were then administered identical doses of treprostinil either intravenously or by aerosol. Systemic and pulmonary hemodynamics were recorded during each administration. Both intravenous and aerosol delivery of treprostinil reduced pulmonary vascular resistance and pulmonary arterial pressure, but the effect was significantly greater with aerosol delivery (P < 0.05). Aerosol delivery of treprostinil had minimal effects on systemic hemodynamics, whereas intravenous delivery increased heart rate and cardiac output and decreased left atrial pressure and systemic blood pressure. Aerosol delivery of the prostacyclin analog treprostinil has a greater vasodilatory effect in the lung with minimal alterations in systemic hemodynamics compared with intravenous delivery of the drug. We speculate that this may result from treprostinil stimulated production of vasodilatory mediators from pulmonary epithelium.
Collapse
Affiliation(s)
- Brett L Sandifer
- Division of Pulmonary, Allergy, and Critical Care, Emory University School of Medicine, Atlanta, GA 30322, USA
| | | | | | | | | | | |
Collapse
|
|
11
|
Inhalative Vasodilatatoren in der kardiochirurgischen Intensivmedizin. ZEITSCHRIFT FUR HERZ THORAX UND GEFASSCHIRURGIE 2005. [DOI: 10.1007/s00398-005-0497-x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
|
|
12
|
Abstract
OBJECTIVE Inhaled nitric oxide has gained an established place in the management of pulmonary hypertension. However, cost, potential toxicity, and the lack of positive outcome data with inhaled nitric oxide therapy has generated interest in alternative inhaled, selective pulmonary vasodilators. This article describes those alternatives that have been studied to date. DESIGN Literature review of inhaled, selective pulmonary vasodilators other than nitric oxide. METHODS A review of the molecular mechanisms, potential side effects, and the studies to date in both animal models and clinical studies describing the physiologic effects of alternative agents to inhaled nitric oxide. CONCLUSION There are a number of available agents that have comparable physiologic effects as inhaled nitric oxide. The best studied of these are the inhaled prostanoids (prostacyclin and iloprost), and there is growing interest in novel therapies such as phosphodiesterase inhibitors and neuropeptides.
Collapse
Affiliation(s)
- Stuart M Lowson
- Department of Anesthesiology, University of Virginia Health Services Foundation, Charlottesville, VA, USA
| |
Collapse
|
|
13
|
Badesch DB, Abman SH, Ahearn GS, Barst RJ, McCrory DC, Simonneau G, McLaughlin VV. Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004; 126:35S-62S. [PMID: 15249494 DOI: 10.1378/chest.126.1_suppl.35s] [Citation(s) in RCA: 356] [Impact Index Per Article: 17.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/28/2023] Open
Abstract
Pulmonary arterial hypertension (PAH) is often difficult to diagnose and challenging to treat. Untreated, it is characterized by a progressive increase in pulmonary vascular resistance leading to right ventricular failure and death. The past decade has seen remarkable improvements in therapy, driven largely by the conduct of randomized controlled trials. Still, the selection of most appropriate therapy is complex, and requires familiarity with the disease process, evidence from treatment trials, complicated drug delivery systems, dosing regimens, side effects, and complications. This chapter will provide evidence-based treatment recommendations for physicians involved in the care of these complex patients. Due to the complexity of the diagnostic evaluation required, and the treatment options available, it is strongly recommended that consideration be given to referral of patients with PAH to a specialized center.
Collapse
Affiliation(s)
- David B Badesch
- University of Colorado Health Sciences Center, Denver, CO 80262, USA.
| | | | | | | | | | | | | |
Collapse
|
|
14
|
Altintas E, Akkus N, Gen R, Helvaci MR, Sezgin O, Oguz D. Effects of terlipressin on systolic pulmonary artery pressure of patients with liver cirrhosis: An echocardiographic assessment. World J Gastroenterol 2004; 10:2278-80. [PMID: 15259082 PMCID: PMC4724967 DOI: 10.3748/wjg.v10.i15.2278] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/15/2022] Open
Abstract
AIM: Portopulmonary hypertension is a serious complication of chronic liver disease. Our aim was to search into the effect of terlipressin on systolic pulmonary artery pressure among cirrhotic patients.
METHODS: Twelve patients (6 males and 6 females) with liver cirrhosis were recruited in the study. Arterial blood gas samples were obtained in sitting position at rest. Contrast enhanced echocardiography and measurements of systolic pulmonary artery pressure were performed before and after the intravenous injection of 2 mg terlipressin.
RESULTS: Of 12 patients studied, the contrast enhanced echocardiography was positive in 5, and the positive findings in contrast enhanced echocardiography were reversed to normal in two after terlipressin injection. The mean systolic pulmonary artery pressure was 25.5 ± 3.6 mmHg before terlipressin injection, and was 22.5 ± 2.5 mmHg after terlipressin (P = 0.003). The systolic pulmonary artery pressure was above 25 mmHg in seven of these 12 patients. After the terlipressin injection, systolic pulmonary artery pressure was < 25 mmHg in four of these cases (58.3% vs 25%, P = 0.04).
CONCLUSION: Terlipressin can decrease the systolic pulmonary artery pressure in patients with liver cirrhosis.
Collapse
Affiliation(s)
- Engin Altintas
- Mersin Universitesi Tip Fakultesi Hastanesi Ic Hastaliklari A.D., 33079 Mersin, Turkiye.
| | | | | | | | | | | |
Collapse
|
|
15
|
Affiliation(s)
- O Sitbon
- Centre des Maladies Vasculaires Pulmonaires, Service de Pneumologie et Réanimation, UPRES EA2705, Université Paris Sud, Assistance Publique, Hôpitaux de Paris, Hôpital Antoine Béclère, 157, rue de la Porte de Trivaux, 92141 Clamart
| |
Collapse
|
|
16
|
Sanchez O. [Treatment of porto-pulmonary hypertension]. GASTROENTEROLOGIE CLINIQUE ET BIOLOGIQUE 2004; 28 Spec No 2:B169-78. [PMID: 15150509 DOI: 10.1016/s0399-8320(04)95252-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 04/29/2023]
Affiliation(s)
- Olivier Sanchez
- Service de Pneumologie et Soins Intensifs, Hôpital Européen Georges Pompidou, 20, rue Leblanc, 75908 Paris, Cedex 15
| |
Collapse
|
|
17
|
Sitbon O. [Treatment of porto pulmonary hypertension]. GASTROENTEROLOGIE CLINIQUE ET BIOLOGIQUE 2004; 28 Spec No 2:B312-6. [PMID: 15150527 DOI: 10.1016/s0399-8320(04)95270-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 04/29/2023]
Affiliation(s)
- Olivier Sitbon
- Centre des Maladies Vasculaires Pulmonaires, Service de Pneumologie et Réanimation, UPRES EA2705, Université Paris Sud, Assistance Publique, Hôpitaux de Paris, Hôpital Antoine Béclère, 157, rue de la Porte de Trivaux, 92141 Clamart
| |
Collapse
|
|
18
|
Abstract
Pulmonary involvement is common in patients with portal hypertension and can manifest in diverse manners. Changes in pulmonary arterial resistance, manifesting either as the hepatopulmonary syndrome or portopulmonary hypertension (PPHTN), have been increasingly recognized in these patients in recent years. This review summarizes the clinicopathologic features, diagnostic criteria, as well as the latest concepts in the pathogenesis and management of PPHTN, which is defined as an elevated pulmonary artery pressure in the setting of an increased pulmonary vascular resistance and a normal wedge pressure in a patient with portal hypertension.
Collapse
Affiliation(s)
- Rohit Budhiraja
- Pulmonary and Critical Care Division, Department of Medicine, Tufts-New England Medical Center, Tufts University School of Medicine, Boston, MA, USA
| | | |
Collapse
|
|
19
|
Kleen M, Zwissler B. Intra-operative use of inhaled vasodilators: are there indications? Curr Opin Anaesthesiol 2002; 15:79-83. [PMID: 17019188 DOI: 10.1097/00001503-200202000-00012] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
The US Food and Drug Administration and European authorities have recently approved inhaled nitric oxide for the treatment of neonates with hypoxic respiratory failure associated with pulmonary hypertension. In addition to this highly specific condition, there is an increasing 'off-label' use of inhaled nitric oxide and other inhaled vasodilators in the perioperative setting. Potential indications include right heart failure as a result of acute pulmonary hypertension in cardiac and non-cardiac surgery, the prevention of reperfusion injury in lung transplantation, the treatment of hypoxaemia during single-lung ventilation, and more recently, the treatment of sickle cell crisis.
Collapse
Affiliation(s)
- Martin Kleen
- Department of Anesthesiology, University of Munich, 81366 Munich, Germany.
| | | |
Collapse
|