Clinical findings and results of hemodynamic, angiographic, and imaging studies in 14 female and 5 male patients with the disease were examined by Guadeloupe Garcia-Tao and colleagues from New Haven, USA.
The report appears in this week's New England Journal of Medicine. In eight patients, the clinical findings were consistent with the presence of high-output heart failure. After a median period of 24 months, three had improved, four were stable with medical therapy, and one had died.
Six patients had manifestations of portal hypertension, such as ascites or variceal bleeding. In four of these the hepatic sinusoidal pressure was elevated. After a median period of 19 months, the condition of two of the six patients had improved, and the other four had died.
High-output heart failure, portal hypertension and biliary disease.
Five patients had manifestations of biliary disease - an elevated alkaline phosphatase and abnormalities on bile duct imaging. After a median period of 30 months, two of the five had improved, one was unchanged, heart failure had developed in another, and one had died.
Concluding, Dr. Garcia-Tsao said, "In patients with hereditary hemorrhagic telangiectasia and symptomatic liver involvement, the typical clinical presentations include high-output heart failure, portal hypertension, and biliary disease."
Report Copyright: Englemed Health News at http://www.internationalmedicalnews.com