Combined lung and liver transplantation for noncirrhotic portal hypertension with severe hepatopulmonary syndrome in a patient with dyskeratosis congenital

2020 ◽  
Author(s):  
Sohyun Shin ◽  
Dong In Suh ◽  
Jung Min Ko ◽  
June Dong Park ◽  
Jeong‐Moo Lee ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Hepatopulmonary Syndrome ◽  
Noncirrhotic Portal Hypertension ◽  
Dyskeratosis Congenital

Liver Transplantation for Hepatopulmonary Syndrome Due to Noncirrhotic Portal Hypertension

2011 ◽  
Vol 43 (7) ◽  
pp. 2814-2816 ◽  
Author(s):  
K. Maganty ◽  
R. Ghanta ◽  
P. Bejarano ◽  
D. Weppler ◽  
A. Tekin ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Hepatopulmonary Syndrome ◽  
Noncirrhotic Portal Hypertension

scholarly journals Hepatopulmonary Syndrome. Review

2021 ◽  
Vol 6 (3) ◽  
pp. 45-52
Author(s):  
V. V. Potii ◽  
◽  
V. T. Kiriienko ◽  
E. I. Glukhova ◽  
O. S. Kunickaya ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Hepatopulmonary Syndrome ◽  
Gas Analysis ◽  
Arterial Oxygen ◽  
Common Symptom ◽  
Portopulmonary Hypertension ◽  
Oxygen Gradient ◽  
Arterial Blood ◽  
Intrapulmonary Shunting

Liver cirrhosis is often accompanied by complications from the pulmonary system. These include hydrothorax, portopulmonary hypertension and hepatopulmonary syndrome. Hepatic hydrothorax affects about 6-10% of patients with end-stage disease, which results in the passage of ascetic fluid into the pleural space through diaphragm defects. The common cause of the hepatopulmonary syndrome and portopulmonary hypertension is portal hypertension and portosystemic shunting, indicating that vasoactive and angiogenetic factors originating from the liver normally control the pulmonary circulation. Portopulmonary hypertension is like pulmonary arterial hypertension, which develops against the background of portal hypertension as a result of chronic liver disease or without other causes of increased pressure in the pulmonary vessels. The prevalence of portopulmonary hypertension ranges from 2% to 8.5% among patients with portal hypertension and is associated with a poor prognosis. Hepatopulmonary syndrome is characterized by intrapulmonary dilatation of microvessels, which causes intrapulmonary shunting and leads to impaired gas exchange in liver diseases, and is associated with a decrease in the quality and duration of life in patients with cirrhosis. Nitric oxide overproduction and angiogenesis seem to be the hallmarks of a complicated pathogenetic mechanism, leading to intrapulmonary shunting and ventilation-perfusion mismatch. A classification of hepatopulmonary syndrome according to the severity of hypoxemia has been suggested. Hepatopulmonary syndrome includes a triad: hepatic dysfunction and / or portal hypertension, dilatation of intrapulmonary vessels, and increased alveolar-arterial oxygen gradient. The prevalence of hepatopulmonary syndrome varies depending on the study groups from 5% to 30%. The most common symptom of the complication is shortness of breath, but in most cases, hepatopulmonary syndrome is asymptomatic. A decrease in oxygen saturation less than 96% corresponds to a decrease in PaO2<70 mm Hg and testifies to the possible development of hepatopulmonary syndrome. In the case of a positive screening, the patient should undergo arterial blood gas analysis, which helps to determine PaO2 and alveolar to arterial oxygen gradient. Conclusion. Contrast-enhanced echocardiography with agitated saline is the gold standard in the diagnosis of intrapulmonary dilatation. The only effective treatment for hepatopulmonary syndrome is liver transplantation. Complete recovery of hepatopulmonary syndrome after liver transplantation is observed within a year in most patients with cirrhosis and hepatopulmonary syndrome

Impact of Hepatopulmonary Syndrome in Liver Transplantation Candidates and the Role of Angiogenesis

2021 ◽  
pp. 2102304
Author(s):  
Steven M. Kawut ◽  
Michael J. Krowka ◽  
Kimberly A. Forde ◽  
Nadine Al-Naamani ◽  
Karen L. Krok ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Liver Disease ◽  
Vascular Complications ◽  
Hepatopulmonary Syndrome ◽  
Functional Class ◽  
Hazard Ratio ◽  
Arterial Oxygen ◽  
Risk Of Death ◽  
Increased Risk

Hepatopulmonary syndrome affects 10–30% of patients with cirrhosis and portal hypertension. We evaluated the serum angiogenic profile of hepatopulmonary syndrome and assessed the clinical impact of hepatopulmonary syndrome in patients evaluated for liver transplantation.The Pulmonary Vascular Complications of Liver Disease 2 study was a multicentre, prospective cohort study of adults undergoing their first liver transplantation evaluation. Hepatopulmonary syndrome was defined as an alveolar-arterial oxygen gradient ≥15 mmHg (≥20 mmHg if age >64 years), positive contrast-enhanced transthoracic echocardiography, and absence of lung disease.We included 85 patients with hepatopulmonary syndrome and 146 patients without hepatopulmonary syndrome. Patients with hepatopulmonary syndrome had more complications of portal hypertension and slightly higher Model for End-stage Liver Disease-Na score compared to those without hepatopulmonary syndrome (median [interquartile range] 15 [12, 19] versus 14 [10, 17], p=0.006). Hepatopulmonary syndrome patients had significantly lower six minute walk distance and worse functional class. Hepatopulmonary syndrome patients had higher circulating angiopoietin-2, Tie2, tenascin-C, c-kit, VCAM-1, and von Willebrand factor levels, and lower E-selectin levels. Patients with hepatopulmonary syndrome had an increased risk of death (hazard ratio 1.80 [1.03–3.16], p=0.04) which persisted despite adjustment for covariates (hazard ratio 1.79 [1.02–3.15], p=0.04). This association did not vary based on levels of oxygenation reflecting the severity of hepatopulmonary syndrome.Hepatopulmonary syndrome was associated with a profile of abnormal systemic angiogenesis, worse exercise and functional capacity, and an overall increased risk of death.

scholarly journals The hepatopulmonary syndrome

2014 ◽  
Vol 27 (2) ◽  
pp. 145-147 ◽  
Author(s):  
Lucas Souto NACIF ◽  
Wellington ANDRAUS ◽  
Rafael Soares PINHEIRO ◽  
Liliana DUCATTI ◽  
Luciana BP HADDAD ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Clinical Manifestation ◽  
Therapeutic Option ◽  
Hepatopulmonary Syndrome ◽  
Supplemental Oxygen ◽  
Vascular Complication ◽  
Low Flow ◽  
Arterial Oxygen ◽  
Effective Therapeutic Option

INTRODUCTION: The hepatopulmonary syndrome has been acknowledged as an important vascular complication in lungs developing systemic hypoxemia in patients with cirrhosis and portal hypertension. Is formed by arterial oxygenation abnormalities induced from intrapulmonary vascular dilatations with liver disease. It is present in 4-32% of patients with cirrhosis. It increases mortality in the setting of cirrhosis and may influence the frequency and severity. Initially the hypoxemia responds to low-flow supplemental oxygen, but over time, the need for oxygen supplementation is necessary. The liver transplantation is the only effective therapeutic option for its resolution. AIM: To update clinical manifestation, diagnosis and treatment of this entity. METHOD: A literature review was performed on management of hepatopulmonary syndrome. The electronic search was held of the Medline-PubMed, in English crossing the headings "hepatopulmonary syndrome", "liver transplantation" and "surgery". The search was completed in September 2013. RESULTS: Hepatopulmonary syndrome is classically defined by a widened alveolar-arterial oxygen gradient (AaPO2) on room air (>15 mmHg, or >20 mmHg in patients >64 years of age) with or without hypoxemia resulting from intrapulmonary vasodilatation in the presence of hepatic dysfunction or portal hypertension. Clinical manifestation, diagnosis, classification, treatments and outcomes are varied. CONCLUSION: The severity of hepatopulmonary syndrome is an important survival predictor and determine the improvement, the time and risks for liver transplantation. The liver transplantation still remains the only effective therapeutic.

Portopulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2534-2537
Author(s):  
Olivier Sitbon ◽  
Laurent Savale
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Hepatopulmonary Syndrome ◽  
Right Heart Failure ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Channel Blockers ◽  
Portopulmonary Hypertension ◽  
Right Heart ◽  
Pulmonary Haemodynamics

Portal hypertension, with or without liver disease, may have major consequences on pulmonary circulation due to complex pathophysiological interactions between the liver and the lungs. There are two distinct pulmonary vascular disorders associated with portal hypertension: hepatopulmonary syndrome, characterized by gas exchange impairment due to intrapulmonary shunts, and portopulmonary hypertension (PoPH), which is a particular form of pulmonary arterial hypertension (PAH). PoPH is a severe complication of portal hypertension, affecting functional status, exercise capacity, and survival of affected patients. Detection of PoPH by transthoracic echocardiography must be performed in symptomatic patients and in all candidates for liver transplantation. Right heart catheterization is mandatory to confirm the diagnosis of PoPH and exclude other causes of pulmonary artery pressure elevation, including fluid overload and high cardiac output, that are common features in portal hypertension. The management of PoPH is similar to that of other forms of PAH although very few trials have investigated the effects of PAH-targeted therapies in this particular indication. Anticoagulants and calcium channel blockers are usually not indicated in this setting. PAH-targeted medications can be used to improve pulmonary haemodynamics and bridge patients with severe PoPH to liver transplantation. However, the impact of liver transplantation on outcomes of patients with PoPH remains unpredictable. While uncontrolled PoPH is associated with a higher risk of perioperative right heart failure and death, stabilization, improvement, or normalization of pulmonary haemodynamics after liver transplantation seem to be achievable goals in selected patients with PoPH.

Pathology of Noncirrhotic Portal Hypertension: Clinicopathologic Study in Pediatric Patients

2003 ◽  
Vol 6 (5) ◽  
pp. 421-426 ◽  
Author(s):  
Carlos Abramowsky ◽  
Rene Romero ◽  
Thomas Heffron
Keyword(s):  
Portal Hypertension ◽  
Portal Vein ◽  
Hepatopulmonary Syndrome ◽  
Intestinal Bleeding ◽  
Liver Transplants ◽  
Venous Flow ◽  
Noncirrhotic Portal Hypertension ◽  
Clinicopathologic Study ◽  
Esophageal Bleeding ◽  
Portal Vein Obstruction

From 1995–2002, 14 patients with predominantly prehepatic, noncirrhotic portal hypertension were evaluated. At presentation, the eight females and six males had a mean age of 9 years (range 2–18). Seven were admitted with gastrointestinal, mostly esophageal bleeding, three with splenomegaly, three with hepato-pulmonary syndrome, and one with hyperammonemia. Imaging studies showed portal vein obstruction in six patients and non-obstructed but frequently anomalous vascular patterns, including hypoplasia of the portal vein, in the remaining eight patients. At the onset, liver function was marginally abnormal in all patients, but thrombocytopenia of approximately 100 × 109/L was consistently observed, probably reflecting chronic mild consumption coagulopathy and hypersplenism. The most striking and frequent histopathologic finding in 25 liver samples, was the presence of hypoplastic portal triads with collapsed portal vein radicles. In contrast, other triads showed markedly distended and misshapen portal vein radicles and likely lymphatics. These two patterns of collapse and distention presumably reflect areas of impaired versus overloaded intrahepatic portal venous flow. Some of the biopsies showed variable portal/sinusoidal fibrosis. Four patients (two with intestinal bleeding, two with hepatopulmonary syndrome) required liver transplants and are doing well. Eight patients are doing well clinically after surgical or spontaneous vascular shunting. Two patients with intestinal bleeding and hepato-pulmonary syndrome, respectively) who had congenital dyskeratosis, underwent bone marrow transplantation and died of nonhepatic-related complications. It is possible to suggest prehepatic causes of portal hypertension even in needle biopsies when collapsed portal vein radicles are present in portal triads, but more than one biopsy sample with larger bore bioptomes may be required to see the changes. Conversely, identifying these changes may suggest to the clinicians the need to work-up a patient for portal hypertension.

Liver transplantation for severe intrahepatic noncirrhotic portal hypertension

2005 ◽  
Vol 11 (6) ◽  
pp. 627-634 ◽  
Author(s):  
Alyssa M. Krasinskas ◽  
Bijan Eghtesad ◽  
Patrick S. Kamath ◽  
Anthony J. Demetris ◽  
Susan C. Abraham
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Noncirrhotic Portal Hypertension
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