Hepatopulmonary Syndrome: Morbidity and Survival After Liver Transplantation

Deberaldini, Maristela; Arcanjo, Ana Beatriz Braga; Melo, Enaldo Vieira de; Silva, R.F. da; Felício, Helen Catharine Camarero de; Arroyo, P.C.; Duca, W.J.; Cordeiro, José Antônio; Silva, R.C.M.A. da

doi:10.1016/j.transproceed.2008.08.134

Cited by 44 publications

(28 citation statements)

References 23 publications

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“…For comparative purposes, our 30‐day mortality post‐LT (11%) is comparable to other series (N > 5 cases) reported by other investigators4‐17 (Table 4). Importantly, no intraoperative deaths were noted in this or any other study.…”

Section: Discussionsupporting

confidence: 88%

Hepatopulmonary syndrome: Favorable outcomes in the MELD exception era

et al. 2013

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Hepatopulmonary syndrome (HPS) is a pulmonary vascular disorder occurring as a consequence of advanced liver disease, characterized by hypoxemia due to intrapulmonary vascular dilatations. HPS independently increases mortality, regardless of the cause or severity of liver disease. Liver transplantation (LT) improves survival in HPS. We present the largest consecutive series of HPS patients specifically addressing long-term survival relative to the degree of hypoxemia and the era in which LT was conducted. We evaluated 106 HPS patients at the Mayo Clinic from 1986 through 2010. Survival was assessed using Kaplan-Meier methodology. LT was accomplished in 49 HPS patients. Post-LT survival (1, 3, 5, and 10 years) did not differ between groups based on baseline partial pressure of arterial oxygen (PaO 2 ) obtained at the time of HPS diagnosis. Improvements in overall survival at 1, 3, and 5 years post-LT in those HPS patients transplanted after January 1 2002 (n 5 28) (92%, 88%, and 88%, respectively) as compared with those transplanted prior to that time (n 5 21) (71%, 67%, and 67%, respectively) did not reach statistical significance (5-year P 5 0.09). Model for Endstage Liver Disease (MELD) exception to facilitate LT was granted to 21 patients since January 1 2002 with post-LT survival of 19/21 patients and one wait-list death. Conclusion: Long-term outcome after LT in HPS is favorable, with a trend towards improved survival in the MELD exception era since 2002 as compared to earlier HPS transplants. Survival after LT was not associated with PaO 2 levels at the time of HPS diagnosis. (HEPATOLOGY 2013;57:2427-2435 See Editorial on Page 2097 H epatopulmonary syndrome (HPS) is a pulmonary vascular disorder characterized by a clinical triad of hepatic dysfunction (usually with portal hypertension), arterial hypoxemia, and intrapulmonary vascular dilatations. HPS is not uncommon and affects between 5%-32% of individuals being assessed for liver transplantation (LT), depending on the criteria used to define arterial hypoxemia. 1 LT is the only therapy that has been shown to consistently and significantly improve or resolve HPS.Mortality associated with HPS, however, can be significant and not necessarily related to the severity of liver disease as measured by MELD (Model for Endstage Liver Disease) or CTP (Child-Turcotte-Pugh) scores. For those HPS patients with partial pressure of arterial oxygen (PaO 2 ) <60 mmHg, a MELD exception has been granted in an attempt to improve survival for those with no other contraindications to LT. 2 In the pre-MELD exception era (up to 2002), we had previously shown that HPS has a poor prognosis, with a 5-year survival of 23% without LT as compared to a 76% survival with LT. 3 In this report we present additional and extended 10-year survival data for our cohort of 106 patients with HPS, including 49 patients who underwent LT since the inception of our LT program. We aimed to further define the role of arterial hypoxemia as measured by baseline PaO 2 (at the time of HPS diagnosis) in ...

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Section: Discussionsupporting

confidence: 88%

Hepatopulmonary syndrome: Favorable outcomes in the MELD exception era

et al. 2013

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“…These findings led to the concept of a “transplant window” for patients with HPS, in which patients with PO2 less than 60 mmHg are prioritized for transplant, while those with more severe hypoxia are excluded because of their poor post‐transplant prognosis. However, it should be noted that other, albeit retrospective, studies evaluating outcomes following liver transplantation found that a preoperative diagnosis of HPS did not affect long‐term mortality . Furthermore, more recent clinical experience would suggest that outcomes are improving with specialized postoperative care, particularly in the early post‐transplant period .…”

Section: Prognosis and Treatmentmentioning

confidence: 95%

Hepatopulmonary syndrome: Update on recent advances in pathophysiology, investigation, and treatment

Grace

Angus

2013

J of Gastro and Hepatol

104

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Hepatopulmonary syndrome (HPS) is an important cause of dyspnea and hypoxia in the setting of liver disease, occurring in 10-30% of patients with cirrhosis. It is due to vasodilation and angiogenesis in the pulmonary vascular bed, which leads to ventilationperfusion mismatching, diffusion limitation to oxygen exchange, and arteriovenous shunting. There is evidence, primarily from animal studies, that vasodilation is mediated by a number of endogenous vasoactive molecules, including endothelin-1 and nitric oxide (NO). In experimental HPS, liver injury stimulates release of endothelin-1 and results in increased expression of ETB receptors on pulmonary endothelial cells, leading to upregulation of endothelial NO synthase (eNOS) and subsequent increased production of NO, which causes vasodilation. In addition, increased phagocytosis of bacterial endotoxin in the lung not only promotes stimulation of inducible NO synthase, which increases NO production, but also contributes to intrapulmonary accumulation of monocytes, which may stimulate angiogenesis via vascular endothelial growth factor pathway. Despite these insights into the pathogenesis of experimental HPS, there is no established medical therapy, and liver transplantation remains the main treatment for symptomatic HPS, although selected patients may benefit from other surgical or radiological interventions. In this review, we focus on recent advances in our understanding of the pathophysiology of HPS, and discuss current approaches to the investigation and treatment of this condition.

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“…Although HPS typically develops in the presence of cirrhosis or portal hypertension, HPS may also occur in the setting of acute and chronic hepatitis without established portal hypertension (46)(47)(48) and in those with vascular abnormalities that limit hepatic venous outflow to the lung (Abernathy malformation, cavopulmonary shunts) (49,50). Studies have not shown a reliable association between the severity of hepatic impairment and presence or severity of HPS (18,43,(51)(52)(53); thus, patients may present with HPS at any point in the course of their liver disease. Similarly, age and sex do not appear to predispose patients with liver disease to HPS.…”

Section: Epidemiologymentioning

confidence: 99%

Pulmonary Vascular Complications of Liver Disease

Fritz

Fallon²,

Kawut

2013

Am J Respir Crit Care Med

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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...

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Hepatopulmonary Syndrome: Morbidity and Survival After Liver Transplantation

Cited by 44 publications

References 23 publications

Hepatopulmonary syndrome: Favorable outcomes in the MELD exception era

Hepatopulmonary syndrome: Favorable outcomes in the MELD exception era

Hepatopulmonary syndrome: Update on recent advances in pathophysiology, investigation, and treatment

Pulmonary Vascular Complications of Liver Disease

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