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Published on: May 11, 2015
Portopulmonary hypertension: short review
Rudolf E Stauber1, Horst Olschewski
1Divisions of Gastroenterology and Hepatology, Department of Internal Medicine, Medical University of Graz, Austria. rudolf.stauber@medunigraz.at
Portopulmonary hypertension (PoPH) affects 5-6% of patients with advanced liver disease, potentially impacting liver transplant outcomes. While treatments for pulmonary hypertension show promise, randomized trials confirming their benefit in PoPH are needed.
Area of Science:
- Cardiology
- Hepatology
- Pulmonology
Background:
- Portopulmonary hypertension (PoPH) is a serious complication of portal hypertension, affecting patients with cirrhosis and noncirrhotic portal hypertension.
- It is diagnosed in 5-6% of patients with decompensated liver disease and can negatively influence outcomes after liver transplantation.
- PoPH prevalence is not linked to liver disease severity but is more common in females and those with autoimmune liver disease.
Purpose of the Study:
- To review the diagnosis and treatment of portopulmonary hypertension.
- To highlight the current understanding of PoPH prevalence and associated factors.
- To discuss the potential application of idiopathic pulmonary hypertension treatments in PoPH.
Main Methods:
- Literature review of studies on portopulmonary hypertension.
- Analysis of diagnostic criteria including Doppler echocardiography and right-heart catheterization.
- Evaluation of treatment strategies used for idiopathic pulmonary hypertension.
Main Results:
- PoPH is an underrecognized complication in 5-6% of decompensated liver disease patients.
- Female sex and autoimmune liver disease are associated with PoPH.
- Small studies suggest safety of targeted treatments (endothelin receptor antagonists, prostanoids, sildenafil) in PoPH, but randomized trials are lacking.
Conclusions:
- Portopulmonary hypertension is an important consideration in patients with portal hypertension and liver disease.
- Current diagnostic methods involve echocardiography and right-heart catheterization.
- Further randomized controlled trials are necessary to establish the efficacy of targeted pulmonary hypertension therapies in PoPH.
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