A Case Report of Portopulmonary Hypertension Precipitated by Transjugular Intrahepatic Portosystemic Shunt

Tatah, Jasmine; Weir, Ε. Kenneth; Prins, Kurt W.; Thenappan, Thenappan

doi:10.1016/j.chest.2020.11.014

Cited by 2 publications

(1 citation statement)

References 16 publications

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“…This procedure will increase flow through the pulmonary circulation, increase right-sided pressures, and may precipitate worsening right ventricular failure. 25 Screening echocardiograms are often done before elective transjugular intrahepatic portosystemic shunt procedures to rule out subclinical PAH.…”

Section: Managementmentioning

confidence: 99%

Portopulmonary Hypertension: A Review

Ruggiero

Bartolome

2022

Advances in Pulmonary Hypertension

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Portopulmonary hypertension (POPH) is a rare complication of liver disease occurring when pulmonary arterial hypertension develops in the setting of portal hypertension. It increases the morbidity and mortality compared to patients with cirrhosis alone. POPH is classified in Group 1 pulmonary arterial hypertension, which has important implications on treatment. After aggressive treatment and in carefully selected patients, liver transplantation can be performed; this can be curative of not only their liver disease but also of their POPH. Treatment and patient selection for optimum results continues to evolve. This article provides updates on the definition, clinical course, and treatment of patients with POPH. We will also discuss the evolving data in treatment and liver transplantation in POPH.

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Section: Managementmentioning

confidence: 99%

Portopulmonary Hypertension: A Review

Ruggiero

Bartolome

2022

Advances in Pulmonary Hypertension

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Synapomorphic features of hepatic and pulmonary vasculatures include comparable purinergic signaling responses in host defense and modulation of inflammation

Hanidziar

Robson

2021

American Journal of Physiology-Gastrointestinal and Liver Physi

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Hepato-splanchnic and pulmonary vasculatures constitute synapomorphic, highly comparable networks integrated with the external environment. Given functionality related to obligatory requirements of "feeding and breathing", these organs are subject to constant environmental challenges entailing infectious risk, antigenic and xenobiotic exposures. Host responses to these stimuli need to be both protective and tightly regulated. These functions are facilitated by dualistic, high-low pressure blood supply of the liver and lungs, as well as tolerogenic characteristics of resident immune cells and signaling pathways. Dysregulation in hepato-splanchnic and pulmonary blood flow, immune responses, and microbiome implicate common pathogenic mechanisms across these vascular networks. Hepato-splanchnic diseases, such as cirrhosis and portal hypertension, often impact lungs and perturb pulmonary circulation and oxygenation. The reverse situation is also noted with lung disease resulting in hepatic dysfunction. Others, and we, have described common features of dysregulated cell signaling during liver and lung inflammation involving extracellular purines (e.g., ATP, ADP), either generated exogenously or endogenously. These metabokines serve as danger signals, when released by bacteria or during cellular stress and cause pro-inflammatory and prothrombotic signals in the gut/liver-lung vasculature. Dampening of these danger signals and organ protection largely depends upon activities of vascular and immune cell-expressed ectonucleotidases (CD39 and CD73), which convert ATP and ADP into anti-inflammatory adenosine. However, in many inflammatory disorders involving gut, liver and lungs, these protective mechanisms are compromised, causing perpetuation of tissue injury. We propose that interventions that specifically target aberrant purinergic signaling might prevent and/or ameliorate inflammatory disorders of the gut/liver and lung axis.

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A Case Report of Portopulmonary Hypertension Precipitated by Transjugular Intrahepatic Portosystemic Shunt

Abstract: We report here a case of portopulmonary hypertension following transjugular intrahepatic portosystemic shunt.

Cited by 2 publications

References 16 publications

Portopulmonary Hypertension: A Review

Portopulmonary Hypertension: A Review

Synapomorphic features of hepatic and pulmonary vasculatures include comparable purinergic signaling responses in host defense and modulation of inflammation

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