Macro- and microcirculation patterns of intrahepatic blood flow changes in patients with hereditary hemorrhagic telangiectasia

Abstract

AIMTo evaluated vascular dynamic processes in the liver of hereditary hemorrhagic telangiectasia (HHT) patients by ultrasound (US) considering quantitative analytic methods.METHODSThe imaging features on US and contrast-enhanced ultrasound (CEUS) in 18 patients diagnosed with HHT were retrospectively analyzed. Regarding CEUS, real-time contrast harmonic imaging and sulfur hexafluoride-filled microbubbles were used.RESULTSHVaMs were identified in all 18 patients. By US, the two major Caselitz criteria could be detected in 55.6% patients. "Color spots" were detected in 72.2% of the cases. Respecting sonographic grading criteria by Buscarini, grade 3 could be demonstrated most frequent (40%). By CEUS, all the patients showed quick and early hyperenhancement during the arterial phase. Significant lowest time to peak (TTP) and highest area under the curve (AUC) values were identified in the hepatic artery (TTP: 69.8%; AUC: 100%) and highest TTP and lowest AUC in the hepatic parenchyma and the portal vein.CONCLUSIONFor the first time we analyzed CEUS findings of a group of HHT patients regarding macro- and microcirculation. Our data demonstrate significant differences in TTP and AUC values in the four selected regions: hepatic artery, shunt region, portal vein and hepatic parenchyma.

Identification of two distinct hereditary hemorrhagic telangiectasia patient subsets with different hepatic perfusion properties by combination of contrast-enhanced ultrasound (CEUS) with perfusion imaging quantification

“…Recently, we reported on the first CEUS analysis of 18 HHT cases concerning hepatic macro- and microcirculation, complementing qualitative results by quantitative perfusion time intensity curve (TIC) evaluation. Our findings showed significant distinctions in time to peak (TTP) and area under the curve (AUC) values in the four selected regions: hepatic artery, shunt region, portal vein and hepatic parenchyma [13].…”

Orthotopic Liver Transplantation for Hereditary Hemorrhagic Telangiectasia and MEN Type I Syndrome - Case Report and Review of Literature

“…The most common cause of diffuse vascular malformations in the liver is HHT (28). Although a majority of HHT patients display liver AVMs (29), only 8% of these ever become clinically manifest (5). Hepatic arteriovenous malformations (HAVMs) are predominantly seen in HHT type 2 and classically manifest in either of the following ways: HOCF (most frequently) (30), biliary sepsis or portal hypertension.…”

“…HHT is considered a rare disease (1:5000) (4), with no significant difference regarding gender or race (5). The highest prevalence has been observed in Europe, on the Danish island of Fyn and in the French departments Ain, Deux-Sevres and Jura (6).…”

The Role of Liver Imaging in Hereditary Hemorrhagic Telangiectasia

“…US imaging with grey scale and Doppler evaluation has been a standard screening test for liver AVMs and in the initial work up of patients suspected of having liver involvement due to HHT [ 10 ]. US can detect the location of large VMs in the liver, and Doppler imaging permits the evaluation of the direction and magnitude of blood flow [ 22 ]. US can also detect focal liver lesions, evaluate the biliary tree, and look for manifestations of portal hypertension such as splenomegaly, ascites, and portal varices.…”