Abstract
BackgroundHereditary hemorrhagic telangiectasia (HHT) is marked by arteriovenous fusion comprising hepatic vascular malformations (HVaMs) with the chance of bleeding.AimsWe investigated HVaMs in HHT patients by combination of contrast-enhanced ultrasound (CEUS) with perfusion imaging quantification to be able to sub-classify a high risk cohort of asymptomatic HHT patients.MethodsThe imaging characteristics on CEUS in 34 patients (aged 21–84 years; mean 58.9) with HHT were retrospectively evaluated. Real-time contrast harmonic imaging, sulfur hexafluoride-filled microbubbles and motion adjustment were utilized. Cine loops of the liver were digital stored, perfusion was quantified using a software reading DICOM data`s.ResultsHVaMs were diagnosed in 31 out of 34 patients. Significant uppermost peak enhancement (PE), wash-in area under the curve (WiAUC) and wash-in perfusion index (WiPI) were identified in the shunt region (100%), next in the hilar region (PE 32.6%; WiAUC 33.9%; WiPI 34.1%), and the lowest in the hepatic parenchyma (PE 10.2%; WiAUC 12.0%; WiPI 9.5%). The perfusion parameters in the shunt region compared to the other regions were significantly increased in one subgroup of patients. Consistent with this, the intrahepatic portal vein diameter and Buscarini grading was significantly higher, while portal vein peak velocity was significantly lower in this patient subset. By statistical analysis, we could correlate PE and WiPI to these clinical parameters, while WiAUC showed no clinical association.ConclusionsFor the first time we combined CEUS findings with motion adjustment software to quantitative determine perfusion parameters of a cohort of HHT patients. Hereby, we could identify a subset of HHT patients with two markedly increased parameter values in the shunt region compared to the hilus/hepatic parenchyma. This could contribute to sub-classify a high-risk group of HHT patients with therapeutic indication.
Highlights
Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominantly transmitted disorder affecting small mucocutaneous blood vessels and/or the vasculature of various viscera [1, 2].Hepatic vascular malformations (HVaMs) represent the most frequent type of visceral involvement, demonstrated in approximately 78% of HHT patients, only 8% being symptomatic [3, 4]
Two HHT patient subsets with different hepatic perfusion properties significantly increased in one subgroup of patients
Consistent with this, the intrahepatic portal vein diameter and Buscarini grading was significantly higher, while portal vein peak velocity was significantly lower in this patient subset
Summary
Hepatic vascular malformations (HVaMs) represent the most frequent type of visceral involvement, demonstrated in approximately 78% of HHT patients, only 8% being symptomatic [3, 4]. HVaMs are currently diagnosed most frequently by vascular ultrasound (vUS) comprising Color Coded Doppler sonography (CCDS) and Power Doppler (PD) but as well by magnetic resonance imaging tomography (MRI) or by computerized tomography (CT). 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]. Hereditary hemorrhagic telangiectasia (HHT) is marked by arteriovenous fusion comprising hepatic vascular malformations (HVaMs) with the chance of bleeding
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