Hepatopulmonary syndrome caused by sarcoidosis of the liver treated with transjugular intrahepatic portosystemic shunt

Nistal, Markus; Pace, Andrea; Klose, Hans; Benten, Daniel; Lohse, Ansgar W.

doi:10.1136/thoraxjnl-2011-201193

Cited by 11 publications

(3 citation statements)

References 5 publications

(4 reference statements)

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“…Eleven studies met the inclusion criteria (14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24), of which one was excluded because it included patients aged o 18 years (24). Finally, 10 studies, consisting of nine case reports and one case series, were included (14)(15)(16)(17)(18)(19)(20)(21)(22)(23).…”

Section: Resultsmentioning

confidence: 99%

Role of Transjugular Intrahepatic Portosystemic Shunts in the Management of Hepatopulmonary Syndrome: A Systemic Literature Review

Tsauo

Weng

et al. 2015

Journal of Vascular and Interventional Radiology

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Section: Resultsmentioning

confidence: 99%

Role of Transjugular Intrahepatic Portosystemic Shunts in the Management of Hepatopulmonary Syndrome: A Systemic Literature Review

Tsauo

Weng

et al. 2015

Journal of Vascular and Interventional Radiology

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“…Early case reports suggested that portal decompression with transjugular intrahepatic shunt (TIPS) placement improved gas exchange and shunt fraction in HPS . However, more recent case reports have been disappointing, and therefore the role of TIPS in the management of HPS remains unproven. Intra‐arterial coil embolization of discrete pulmonary arteriovenous communications has been used successfully and may have a place in improving right to left shunt in rare patients with large fistulae amenable to radiographic intervention .…”

Section: Prognosis and Treatmentmentioning

confidence: 99%

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

Grace

Angus

2013

J of Gastro and Hepatol

101

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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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“…However, there has been continued interest in evaluating the effects of lowering portal pressure with transjugular intrahepatic shunt (TIPS) in patients with HPS [39][40][41][42][43][44][45][46][47]. Two recent cases of TIPS improving oxygenation in HPS have been published [48,49], increasing the number of reported cases to nine. However, similar to the pharmacologic therapies, TIPS has not been consistently beneficial in HPS, and the short duration of the follow-up reported makes it difficult to know whether TIPS is a reliable treatment option.…”

Section: Human Diseasementioning

confidence: 99%