portopulmonale Hypertension
Hepatopulmonary
syndrome and portopulmonary hypertension: a case report. Axial CT image, mediastinal window: the main pulmonary artery is enlarged, at this level it presents a caliber of 38mm. No further mediastinal anomalies are evident.
Hepatopulmonary
syndrome and portopulmonary hypertension: a case report. Axial CT image, mediastinal window: the main pulmonary artery is enlarged, at this level it presents a caliber of 32mm. No further mediastinal anomalies are evident.
Hepatopulmonary
syndrome and portopulmonary hypertension: a case report. Axial CT image at upper zone, lung window: the lungs show a marked and diffuse dilatation of pulmonary veins and arteries at upper, middle and lower zones.
Hepatopulmonary
syndrome and portopulmonary hypertension: a case report. Axial CT image at middle zone, lung window.
Hepatopulmonary
syndrome and portopulmonary hypertension: a case report. Axial CT image at lower zone, lung window: the vessel dilatation is more evident in the lower zones, small and hazy ground glass areas are also evident.
Hepatopulmonary
syndrome and portopulmonary hypertension: a case report. Abdominal contrast-enhanced CT shows liver signs of cirrhosis: tortuous hepatic arteries, enlarged lobe caudate, volume reduction of right lobe, irregular liver edges. A perihepatic and perisplenic amount of ascites is also evident.
Portopulmonary hypertension (POPH/PPHTN) refers to pulmonary artery hypertension that develops in the setting of portal hypertension (with or without underlying liver disease). It falls under group 1.4 of the Dana point 2008 pulmonary hypertension classification system.
Epidemiology
The prevalence of portopulmonary hypertension ranges from 2-5% in patients with liver cirrhosis.
Pathology
Three presumed mechanisms have been proposed:
- vasoactive substances (e.g. serotonin, interleukin-1, endothelin-1, and thromboxane) cause vasoconstriction and mitogenesis in pulmonary arteries, bypass the liver through portosystemic shunts or not effectively cleared by the diseased cirrhotic liver
- venous thromboembolism: blood clots from the portal vein or other systemic sources passing through portosystemic shunts to reach the pulmonary circulation, causing pulmonary hypertension
- high cardiac output associated with cirrhosis exposing the pulmonary vascular bed to increased shear stress and in turn causing vasoconstriction, hypertrophy, and the proliferation of pulmonary arterial endothelial cells
Suggested diagnostic criteria
- mean pulmonary artery pressure greater than 25 mmHg at rest
- increased pulmonary vascular resistance, pulmonary capillary wedge pressure less than 15 mmHg
- evidence of portal hypertension
See also
- pulmonary complications of cirrhosis and portal hypertension
- hepatopulmonary syndrome (HPS)
- portopulmonary hypertension (POPH)
- hepatic hydrothorax (HH)
Siehe auch:
Assoziationen und Differentialdiagnosen zu portopulmonale Hypertension:
