Case 1: A 59-year-old male was admitted to the hospital with “shortness of breath after activity and epigastric discomfort for 3 months”, with no family history of hereditary disease. Physical examination: general condition was good, liver and spleen were not palpable under the ribs, continuous blood murmur could be heard in the liver region, and mobile turbidity was negative. Blood biochemistry: ALT, AST, alkaline phosphatase 138U/l, total bilirubin 15μmol/l. Hepatitis series was negative. Abdominal ultrasound showed: abnormal honeycomb blood flow signal in the hepatic portal region, multiple cysts in the liver, and increased hepatic artery blood flow. Abdominal CT and CTA showed: hepatic innominate arteries and left and right hepatic arteries were obviously tortuous and thickened; multiple cysts in the liver; and the portal vein of the right lobe of the liver in the arterial phase and part of the hepatic vein were abnormally early manifested. Since the diagnosis of HHT in 2006, the patient was treated with self-administered Chinese herbal medicine, which was ineffective, and the symptoms of shortness of breath and epigastric discomfort did not improve fundamentally. After that, ascites gradually appeared and liver function was abnormal. After taking oral diuretics, the ascites gradually decreased. At present, the patient still has a small amount of ascites, with normal range of liver function indexes and normal bilirubin, but she has shortness of breath after minor activities and cannot engage in heavy physical labor. Case 2: A 35-year-old woman was admitted to the hospital with right upper abdominal discomfort for 3 years and right shoulder and back pain for 2 months. She had a history of nosebleeds, and her mother had died of unexplained upper gastrointestinal hemorrhage. Admission examination: general condition was good, the liver was about 2 cm below the costal margin, tough, and a blood flow murmur could be heard on auscultation. Serologic examination: ALT, AST normal range, alkaline phosphatase 193U/l, total bilirubin 19μmol/l, hepatitis B five and hepatitis C antibody are negative. Abdominal ultrasound showed: the liver was obviously enlarged, the parenchymal echogenicity was enhanced, the hepatic artery was obviously thickened, and there was a hepatic artery-portal vein fistula. Intensified CT showed: the liver was enlarged with moderate cirrhosis, the hepatic artery in the portal region was obviously thickened and disorganized; some branches of the portal vein could be seen in the arterial phase; the lower part of the esophagus and the gastric fundus had mild varicose veins.CT angiography (CTA) showed: the right, middle, and left hepatic arteries originated from the celiac trunk; the right and middle hepatic arteries were obviously dilated; and the hepatic arteries had tortuous dilatation in various branches. Digital subtraction angiography (DSA) showed that the three tortuous and dilated hepatic arteries originated from the celiac trunk, and their branches were obviously thickened and tortuous, with a large number of fuzzy mass-like shadows, and the blood flow rate was obviously accelerated. The patient underwent hepatic artery ligation in 2005. During the operation, the liver was enlarged with nodular sclerosis-like changes; the right hepatic artery, middle hepatic artery, and left hepatic artery originated from the celiac trunk; the right and middle hepatic arteries were significantly thickened and dilated, with diameters of up to 2 cm, while the left hepatic artery was mildly dilated with a diameter of about 1 cm. the right hepatic artery and middle hepatic artery were doubly ligated at their origins and at the point of entry into the liver, while the left hepatic artery was ligated with a bundle. The left hepatic artery was ligated. Pathological examination of some liver tissues and the dilated left hepatic artery showed diffuse capillary dilatation, small vessel dilatation, and alternating hepatic regeneration and atrophy in the liver parenchyma, and fibrous tissue nodular hyperplasia around the dilated blood vessels in some areas. Postoperatively, the patient recovered well, with relief of abdominal and right shoulder and back pain. At present, the patient’s general condition is good, with no obvious self-conscious discomfort, and can engage in normal physical labor. CT and CTA showed that the liver and spleen decreased in size, and laboratory tests showed that liver function and bilirubin were in the normal range. Case 3: A 36-year-old woman was admitted to the hospital with shortness of breath, fatigue, and a feeling of fullness in the right upper abdomen for 2 months. She had a history of recurrent nosebleeds with no clear family history. Physical examination: general condition, regular heart rhythm, audible high-pitched systolic murmur, natural abdominal distension, bruise-like changes in the abdominal skin, discomfort to the touch in the right epigastric region, the liver was about 3 cm below the costal margins, the spleen was palpable below the costal margins, and a distinctive continuous vascular murmur was audible on auscultation of the hepatic region, with a negative mobility turbidity tone. Serology: ALT, AST normal range, alkaline phosphatase 237 U/l, total bilirubin 41.3 μmol/l. Cardiac ultrasound: left atrium, left ventricle enlarged, mitral valve, tricuspid valve insufficiency. Abdominal color ultrasound showed: enlarged liver, obvious thickening and tortuosity of hepatic artery, and abnormal vascular mass in the liver. Abdominal CT showed that the liver was enlarged, the hepatic artery was obviously tortuous, thickened and aneurysmal, the hepatic vein was dilated, and there were multiple spots and patchy enhancement shadows in the liver. The patient underwent hepatic artery interventional embolization in 2006, which was ineffective and did not alleviate the clinical symptoms. Afterwards, the abnormal hepatic arteriovenous shunt flow increased gradually, which led to an increase in the cardiac load and a worsening of the hyperdynamic blood circulation condition, and meanwhile, severe arteriovenous shunt flow led to the progressive aggravation of hepatic ischemia, which triggered an increase in hepatic impairment and a gradual deterioration of his health, and he eventually developed hepatic and cardiac failure, and he passed away at the end of 2008. Discussion HHT is triggered by mutations in the genes encoding the TGF-β superfamily [1-3], which cause abnormal TGF-β signaling in vascular endothelial cells, leading to impaired angiogenic development. Pathologic changes in the liver include abnormal capillary dilatation, arteriovenous fistulae, and aneurysm formation, usually without hepatocellular necrosis and inflammatory cell infiltration. The formation of arteriovenous fistulae, however, plays a decisive role in the pathophysiologic changes and disease progression and regression, and is subdivided into three types, i.e., hepatic artery-hepatic vein fistulae, hepatic artery-portal vein fistulae, and portal vein-hepatic vein fistulae [4][5]. Arteriovenous fistulas lead to abnormal arteriovenous shunts, which on the one hand affect intrahepatic blood flow distribution and lead to localized ischemia in the liver and biliary tract, which can result in cyst-like dilatation of the biliary tract, stenosis, focal ischemic necrosis, nodular regenerative hyperplasia (NRH), focal nodular hyperplasia (FNH), and focal hyperplasia (FNH); on the other hand, increased portal and/or hepatic venous blood volume. Hepatic artery-portal vein fistula as well as abnormal proliferation of liver tissue caused by HHT can cause portal hypertension. And the abnormal blood shunt of hepatic artery-hepatic vein fistula and portal vein-hepatic vein fistula can not only trigger portal shunt encephalopathy, but also increase cardiac preload, raise cardiac output, and produce a hyperdynamic circulatory state, which can be secondary to severe congestive heart failure. The clinical manifestations of patients with hepatic HHT are not specific, but there are mostly shortness of breath, fatigue, and discomfort in the right upper abdomen, etc. Aneurysms and some patients with arteriovenous shunts may have pulsatile masses, vascular murmurs, tremors, and in severe cases, the corresponding symptoms of congestive heart failure and cirrhosis may appear. Serologic examination is also not specific, and some patients with biliary tract lesions may have abnormal biliary enzyme profiles. Therefore, to capture the pathological features of abnormal dilatation of liver capillaries, arteriovenous fistulae and aneurysm formation, imaging examination is more diagnostic value, and ultrasound, CT, MRI, digital subtraction angiography (DSA) are all helpful for diagnosis. Ultrasonography is simple and convenient, and it can sensitively and reliably reflect liver lesions and hemodynamic changes in the hepatic artery, which is an effective means of screening. Its indicators mainly include increased diameter of hepatic artery, tortuous deformation, high speed and low obstruction of arterial blood flow, etc. [6].CT shows tortuous and dilated arterial vessels around the abdominal aorta, the hepatic portal region and intrahepatic, even hepatic aneurysm, and diffusely dilated capillary network or vascular mass shadow can be seen in the liver, and the formation of biliary cysts, biliary dilatation or stenosis can be seen as well. Due to the presence of hepatic arteriovenous fistulae, early visualization of portal and/or hepatic venous anomalies and Transient Hepatic Attenuation Differences (THADs) in the hepatic parenchyma can be seen in the arterial phase [7].The diagnostic value of MRI is approximately the same as that of CT. In general, DSA images are clear, with good contrast and no motion artifacts, and can clearly determine the origin, course, branching and distribution of arteries, which is the “gold standard” for diagnosing vascular lesions. For hepatic HHT, DSA can visualize the thickness of hepatic artery diameter and intrahepatic arteriovenous fistula, which has high diagnostic value. However, the effect of DSA imaging depends on the amount of contrast and its injection flow rate. We believe that for complex HHT lesions with large hepatic artery aneurysms, large hepatic arteriovenous shunts, and fast blood flow rates, the contrast agent diffuses poorly and dissipates quickly in the lesion area, so that DSA cannot achieve the desired effect. However, multilayer spiral CT angiography (CTA) can visualize the distortion, dilatation and malformation of hepatic blood vessels by combining volumetric reconstruction and maximum density projection, and multi-phase scanning can show arterial and venous malformations, as well as observe the positional relationship between various vessels from multiple angles. Its combination with CT can clarify the liver parenchyma and bile ducts, and it is a non-invasive operation, avoiding complications such as puncture infection. Therefore, CTA is superior to DSA for this part of complex hepatic HHT lesions. In the above three cases, none of the clinical manifestations were specific, but ultrasound, CT, DSA and other imaging examinations, especially CTA, all confirmed obvious tortuous dilatation of the hepatic artery, intrahepatic arteriovenous fistulae, and dilatation of the capillaries, and the diagnosis of hepatic hereditary hemorrhagic capillaritis was confirmed. Case 3 was the most severe, with aneurysmal changes in the hepatic artery and the presence of numerous arteriovenous fistulas and diffuse abnormal vascular clusters in the liver. Case 1 was the mildest, with a unibranched hepatic artery, and angiography showed less distortion of the left and right hepatic arteries, and fewer abnormal vascular clusters in the liver. The treatment for HHT or HHT liver lesions is still controversial [8-11], but we believe that individualized treatment is needed. For asymptomatic and mild patients, they should be followed up with observation or pharmacologic symptomatic treatment, whereas for more severe patients with complex hepatic HHT, aggressive surgical interventions are beneficial, mainly including hepatic artery ligation, fasciculation, hepatic artery interventional embolization, and liver transplantation. Liver transplantation is the only curative treatment and has been reported in Europe and the United States with an overall favorable prognosis [9]. However, the scarcity of donor livers, stringent requirements for surgical conditions and techniques, and the high cost constrain liver transplantation, and it can only be used as a waitlist treatment for patients who have failed other treatments [9, 12], and especially transplantation does not make sense for asymptomatic patients. The therapeutic principles of hepatic artery ligation, fasciculation and embolization are similar, i.e., by reducing the hepatic venous fistulae blood supply, mitigating the abnormal shunt, reducing the complications and improving the organism’s condition. Hepatic artery ligation can effectively close the arteriovenous fistula and reduce hepatic steal and cardiac load, but there are certain requirements for the applicable conditions, i.e., the absence of severe biliary ischemic complications and the presence of no obvious portal-hepatic venous fistula. We believe that minimizing intraoperative freeing of the common bile duct, preserving the periportal ligament and arterial collateral circulation can prevent intra- and extrahepatic biliary ischemia caused by hepatic artery ligation to a certain extent. In case 2, ligation of the dilated hepatic artery was used, which effectively improved the abnormal intrahepatic shunt, reduced various complications, and protected the common bile duct and the perihepatic ligament intraoperatively, and since the follow-up for 4 years, there were no hepatic and biliary ischemic complications, the liver function was normal, and the cardiac function had been improved, and the efficacy of the treatment was satisfactory. Interventional embolization was used in case 3, but the efficacy was poor. It should be noted that vascular interventional embolization is effective for some patients [8], but for complex patients with severe hemangiomas, arteriovenous malformations, and hyperdynamic circulatory states, interventional embolization is difficult to achieve therapeutic effects and is prone to recurrence after the procedure. Ligation or bundling of the hepatic artery should be performed in such patients. However, vascular embolization should be considered in patients who are physically unable to tolerate surgery. The above three cases all belong to complex HHT with conscious symptoms. Compared with case 2, the thickened hepatic artery in case 1 was slightly thinner in diameter, single-branched, and the intrahepatic lesions were lighter, so the surgical effect should be better than that of case 2. However, this patient was only treated with medication, and the therapeutic effect was unsatisfactory in the three years of follow-up, and even serious ascites and liver function abnormalities appeared. In case 3, the patient with the most obvious vascular malformation, there is a large amount of abnormally shunted blood in the liver, the effect of vascular interventional embolization is poor, and it cannot effectively control the arteriovenous shunt, and he died of gradually aggravated liver failure and heart failure, so it is more appropriate to perform surgical operation. For HHT patients with severe congestive heart failure, pulmonary hypertension, hepatic failure, portal hypertension, biliary complications, even liver transplantation can not change the pathophysiological status, but can improve the quality of life and prolong the survival time through symptomatic treatment with drugs.