Pilot study of pentoxifylline in hepatopulmonary syndrome

Tanikella, Rajasekhar; Philips, George; Faulk, Dorothy K.; Kawut, Steven M.; Fallon, Michael B.

doi:10.1002/lt.21482

Cited by 81 publications

(41 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…By virtue of its ability to block tumor necrosis factor-a and macrophage-derived NO synthesis, pentoxifylline has garnered attention, as rodent studies suggested potential benefit in experimental HPS (63,64). However, two small studies in patients with HPS reached conflicting conclusions (65,66). Occasionally, patients may present with large pulmonary arteriovenous malformations that are amenable to angiographic embolization, which can at least transiently improve oxygenation (67); however, this is the rare exception.…”

Section: Treatmentmentioning

confidence: 99%

Pulmonary Vascular Complications of Liver Disease

Fritz

Fallon²,

Kawut

2013

Am J Respir Crit Care Med

Self Cite

View full text Add to dashboard Cite

Hepatopulmonary syndrome and portopulmonary hypertension are two pulmonary vascular complications of liver disease. The pathophysiology underlying each disorder is distinct, but patients with either condition may be limited by dyspnea. A careful evaluation of concomitant symptoms, the physical examination, pulmonary function testing and arterial blood gas analysis, and echocardiographic, imaging, and hemodynamic studies is crucial to establishing (and distinguishing) these diagnoses. Our understanding of the pathobiology, natural history, and treatment of these disorders has advanced considerably over the past decade; however, the presence of either still increases the risk of morbidity and mortality in patients with underlying liver disease. There is no effective medical treatment for hepatopulmonary syndrome. Although liver transplantation can resolve hepatopulmonary syndrome, there appears to be worse survival even with transplantation. Liver transplantation poses a very high risk of death in those with significant portopulmonary hypertension, where targeted medical therapies may improve functional status and allow successful transplantation in a small number of select patients.Keywords: hepatopulmonary syndrome; hypertension; pulmonary; liver cirrhosis; pulmonary circulationThe pulmonary circulation may be affected by pathogenic processes arising within the liver and portal venous system. Reports of abnormalities of the pulmonary vasculature found in association with coexisting chronic liver disease were first published in the 1950s (1, 2). Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) are both associated with portal hypertension and/or liver disease and in simplest terms are related to excessive pulmonary microvascular dilation (in HPS) or vasoconstriction/vascular obstruction in pulmonary resistance vessels (in PoPH). The fact that these seemingly disparate conditions can occur within the same patient population (and even simultaneously or sequentially in the same patient) suggests the role of disease modifiers that determine the particular pulmonary vascular phenotype. These important pulmonary complications both commonly present with dyspnea, yet have unique pathophysiologies, diagnostic modalities, approaches to treatment, and distinct implications regarding liver transplantation (LT). A thorough understanding of the approach to evaluation and treatment of patients with cirrhosis and possible pulmonary vascular disease is imperative to minimize sequelae and to ensure appropriate management of the underlying liver disease.HEPATOPULMONARY SYNDROME Definition HPS is a disorder of altered gas exchange due to abnormal capillary dilatation and/or arteriovenous fistulae within the pulmonary vascular bed (3). The European Respiratory Society Task Force on Pulmonary-Hepatic Vascular Disorders has defined HPS by the presence of (1) Use of the A-a gradient criterion for abnormal gas exchange captures cases of HPS that would not otherwise meet the Pa O 2 criterion (e.g., due to cirrhosis-in...

show abstract

Section: Treatmentmentioning

confidence: 99%

Pulmonary Vascular Complications of Liver Disease

Fritz

Fallon²,

Kawut

2013

Am J Respir Crit Care Med

Self Cite

View full text Add to dashboard Cite

show abstract

“…An open-label, singlearm clinical trial failed to show any improvement in Pa,O 2 or PAa,O 2 after 6 weeks of pentoxifylline. However, the study included only nine patients and not all of them were able to fulfil the intended follow-up or adherence to therapy, mostly due to adverse effects of pentoxifylline [96]. More recently, a blinded randomised crossover study did not show any benefit in PA-a,O 2 after 4 weeks of norfloxacin, as compared with placebo [97].…”

Section: Review: Poph and Hps M Porres-aguilar Et Almentioning

confidence: 99%

Portopulmonary hypertension and hepatopulmonary syndrome: a clinician-oriented overview

Porres‐Aguilar

Altamirano²,

Torre-Delgadillo³

et al. 2012

European Respiratory Review

105

118

View full text Add to dashboard Cite

Liver disease and portal hypertension can be associated with pulmonary vascular complications, including portopulmonary hypertension (POPH), characterised by an elevated mean pulmonary artery pressure secondary to an increased pulmonary vascular resistance, and hepatopulmonary syndrome (HPS), characterised by hypoxaemia due to pulmonary vasodilatation and shunting.Although clear diagnostic guidelines exist for both conditions on the basis of echocardiography, right heart catheterisation and arterial blood gases, there is considerable variation between centres regarding diagnosis and management of these conditions. Awareness of evaluation and management algorithms for POPH and HPS are critical for optimisation of outcomes in patients with these conditions. Key aspects of management of POPH and HPS include identification of patients likely to benefit from liver transplantation (LTx) and management before and after LTx. Although both disorders may improve after LTx, severe forms of POPH represent a contraindication to LTx.Novel approaches to the treatment of POPH and HPS offer new management options that may expand the pool of transplantable patients and improve overall outcomes.

show abstract

“…Agents such as pentoxifylline (a nonspecific phosphodiesterase inhibitor that blocks the synthesis of tumor necrosis factor alpha), methylene blue (an inhibitor of guanylate cyclase), and endothelin B receptor blockers have been tried in a small number of studies with variable results. [12][13][14][15] The treatment of choice for hepatopulmonary syndrome is liver transplantation, with a 5-y survival rate after liver transplantation of 76%. 16 In contrast, the 5-y survival of patients who do not undergo liver transplantation is 23%, with a median survival of 24 months.…”

Section: Discussionmentioning

confidence: 99%

Coil Embolization of Pulmonary Arteries as a Palliative Treatment of Diffuse Type I Hepatopulmonary Syndrome

et al. 2014

View full text Add to dashboard Cite

Hepatopulmonary syndrome is a serious complication of liver disease. Type I hepatopulmonary syndrome is associated with diffuse dilatation of the pulmonary vasculature, leading to severe hypoxemia. Liver transplantation is the treatment of choice for this condition. There are limited options for those who are not candidates for liver transplantation. We present the case of a patient who presented with severe hypoxemia requiring F IO 2 of 0.95 with P aO 2 of 59 mm Hg. Workup revealed 33% intrapulmonary right-to-left shunt. A pulmonary angiogram showed diffuse dilatation of the pulmonary arteries, especially in the lower lobes. The patient was diagnosed with type I hepatopulmonary syndrome. He was not a candidate for liver transplantation. The patient underwent sequential coil embolizations of the lower lobe pulmonary arteries. He was discharged home on 2 L of supplemental oxygen. This case demonstrates that coil embolization of dilated pulmonary arteries is a potential palliative treatment for patients with diffuse type I hepatopulmonary syndrome.

show abstract

Pilot study of pentoxifylline in hepatopulmonary syndrome

Cited by 81 publications

References 15 publications

Pulmonary Vascular Complications of Liver Disease

Pulmonary Vascular Complications of Liver Disease

Portopulmonary hypertension and hepatopulmonary syndrome: a clinician-oriented overview

Coil Embolization of Pulmonary Arteries as a Palliative Treatment of Diffuse Type I Hepatopulmonary Syndrome

Contact Info

Product

Resources

About