Abstract:Patients with advanced chronic liver diseases, particularly with decompensated liver cirrhosis, can develop specific pulmonary complications independently of any pre-existing lung disease. Especially when dyspnea occurs in combination with liver cirrhosis, patients should be evaluated for hepato-pulmonary syndrome (HPS), porto-pulmonary hypertension (PPHT), hepatic hydrothorax and spontaneous bacterial empyema, which represent the clinically most relevant pulmonary complications of liver cirrhosis. Importantly… Show more
“…The definition of portopulmonary hypertension (PoPH) is pulmonary artery hypertension (PAH) that is associated, or caused by, an increase in portal pressure, which may occur with or without significant liver disease 1. PoPH is part of a group of syndromes resulting in dyspnoea as a consequence of liver disease.…”
Section: Introductionmentioning
confidence: 99%
“…PoPH is part of the group 1 classification of pulmonary hypertension 4. The increase in pulmonary pressure causes remodelling of the pulmonary vasculature which then leads to higher vasculature resistance 1. In patients with hyperdynamic circulations the increased cardiac output leads to an increase in the sheer stress placed on the pulmonary circulation 5.…”
Portopulmonary hypertension (PoPH) is a poorly understood complication of liver disease which affects about 10% of patients with pulmonary hypertension. This case report outlines the difficulties in diagnosing and managing a patient with advanced disease, and the impact of these delays on the patient.PoPH has a significant risk of mortality with a 2-year survival rate of 67%. There are also few treatment options available and those which do exist are associated with multiple contraindications and risks. Patients with PoPH commonly present with dyspnoea, pulmonary hypertension and portal hypertension. The presence of coexisting chronic liver disease is also sometimes present. Traditional management for heart failure can temporarily alleviate symptoms but there is no proven long-term benefit. As a result, an understanding of the pathophysiology, diagnostics and management is crucial to ensure the best possible patient outcomes.
“…The definition of portopulmonary hypertension (PoPH) is pulmonary artery hypertension (PAH) that is associated, or caused by, an increase in portal pressure, which may occur with or without significant liver disease 1. PoPH is part of a group of syndromes resulting in dyspnoea as a consequence of liver disease.…”
Section: Introductionmentioning
confidence: 99%
“…PoPH is part of the group 1 classification of pulmonary hypertension 4. The increase in pulmonary pressure causes remodelling of the pulmonary vasculature which then leads to higher vasculature resistance 1. In patients with hyperdynamic circulations the increased cardiac output leads to an increase in the sheer stress placed on the pulmonary circulation 5.…”
Portopulmonary hypertension (PoPH) is a poorly understood complication of liver disease which affects about 10% of patients with pulmonary hypertension. This case report outlines the difficulties in diagnosing and managing a patient with advanced disease, and the impact of these delays on the patient.PoPH has a significant risk of mortality with a 2-year survival rate of 67%. There are also few treatment options available and those which do exist are associated with multiple contraindications and risks. Patients with PoPH commonly present with dyspnoea, pulmonary hypertension and portal hypertension. The presence of coexisting chronic liver disease is also sometimes present. Traditional management for heart failure can temporarily alleviate symptoms but there is no proven long-term benefit. As a result, an understanding of the pathophysiology, diagnostics and management is crucial to ensure the best possible patient outcomes.
“…Pulmonary hypertension is defined as mean pulmonary artery pressure of more than 25 mmHg at rest as measured on right heart catheterization. 1 Porto pulmonary hypertension (PPHTN) is a complication of cirrhosis of any cause. It affects 2-5% of patients with portal hypertension following portoenterostomy even with normalization of liver markers.…”
Section: Introductionmentioning
confidence: 99%
“…The median age of presentation is 11.5 years. 1 However, it can occur even in early stages of the liver disease. 3 Development of PPHTN is postulated to have many mechanisms, some of which include hyperdynamic state, increased pulmonary venous congestion and vascular constriction with obstruction of the pulmonary arterial bed and genetic predisposition in a minority.…”
Porto pulmonary hypertension is the presence of pulmonary arterial hypertension in patients with advanced liver disease and portal hypertension. It affects minority of advanced liver disease patients without a significant correlation between hepatic impairment and severe porto pulmonary hypertension. It is a rare complication of portal hypertension following successful portoenterostomy. We present a case of a seventeen-year-old male with a past history of Kasai procedure for congenital biliary atresia, presenting with severe pulmonary hypertension as a complication of cirrhosis. He was defaulted during childhood follow up and was asymptomatic during last 17 years which has led to silent progression of secondary biliary cirrhosis. Hence this highlights the importance of long-term follow up after Kasai procedure.
“…нарушением баланса между сосудосуживающими и сосудорасширяющими веществами. В одном из исследований, одышка, показала отрицательную прогностическую ценность 75% при диагностике ГПС, при этом не было обнаружено корреляции между ГПС и спленомегалией, асцитом, отеками, желтухой, олигурией, также варикозно расширенными венами пищевода [15]. В другом исследовании показано корреляцию десатурации кислорода во время сна с наличием ГПС [16].…”
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
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