title: Clinical Outcomes of Patients with Severe Hepatic Hereditary Hemorrhagic Telangiectasia After Banding of the Hepatic Artery and Banding/Ligation of Branches of the Hepatic Artery.
abstract: OBJECTIVE
  To evaluate the effectiveness of double banding/ligation of hepatic arteries in treating patients with hepatic hereditary hemorrhagic telangiectasia (HHHT).
  
  
  METHODS
  From January 2004 to December 2013, 35 patients were diagnosed with HHHT, among whom 11 woman and two men with a mean ± SD age of 44 ± 9 years were treated by double hepatic artery banding/ligation for cardiac insufficiency and/or portal hypertension. The outcomes were evaluated prospectively by measuring clinical manifestations, imaging features, liver and cardiac function, pulmonary arterial systolic pressure, and post-operative complications. Quality of life was evaluated with the Short Form Health Survey questionnaire.
  
  
  RESULTS
  For each patient, the common hepatic artery and one branch of the left and/or right hepatic artery were banded, and other significantly dilated hepatic artery branches were ligated. No patient died after surgery. Clinical symptoms were improved in all patients, although ischemic cholangitis was observed in two patients and treated conservatively. Cardiac function, classified per the New York Heart Association (NYHA) cardiac functional grading, improved (NYHA III-IV vs. NYHA I-II); pulmonary arterial systolic pressure significantly decreased in all patients (48 ± 8 mmHg vs. 24 ± 4 mmHg; P < .001) and remained in the normal range (26 ± 3 mmHg) at the end of follow up. The levels of γ-glutamyl transpeptidase and alkaline phosphatase decreased in 11 patients (144 ± 94 U/L vs. 71 ± 34 U/L; P = .003) and 10 patients (207 ± 71 U/L vs. 105 ± 32 U/L; P = .001), respectively. Patients were followed up for 50 ± 28 months (range 6-113 months); one death resulted from causes unrelated to surgery and all dimensions of quality of life improved in all surviving patients.
  
  
  CONCLUSIONS
  This study helps to establish double hepatic artery banding/ligation as an effective therapy for selected patients with HHHT.
authors.affiliation: Department of Hepatobiliary Surgery, Qilu Hospital of Shandong University, Jinan 250012, Shandong Province, China.
authors.forename: Z-C
authors.initials: ZC
authors.lastname: Liu
authors.affiliation: Department of Hepatobiliary Surgery, Qilu Hospital of Shandong University, Jinan 250012, Shandong Province, China; Department of General Surgery, Jinan Central Hospital, Jinan 250013, Shandong Province, China.
authors.forename: X-F
authors.initials: XF
authors.lastname: Lu
authors.affiliation: Department of Hepatobiliary Surgery, Qilu Hospital of Shandong University, Jinan 250012, Shandong Province, China.
authors.forename: H
authors.initials: H
authors.lastname: Yang
authors.affiliation: Department of Hepatobiliary Surgery, Qilu Hospital of Shandong University, Jinan 250012, Shandong Province, China.
authors.forename: H-D
authors.initials: HD
authors.lastname: Liu
authors.affiliation: Department of General Surgery, Shandong Provincial Hospital, Jinan 250021, Shandong Province, China.
authors.forename: X
authors.initials: X
authors.lastname: Song
authors.affiliation: Department of Hepatobiliary Surgery, Qilu Hospital of Shandong University, Jinan 250012, Shandong Province, China.
authors.forename: S-L
authors.initials: SL
authors.lastname: Ning
authors.affiliation: Department of Hepatobiliary Surgery, Qilu Hospital of Shandong University, Jinan 250012, Shandong Province, China.
authors.forename: Y-F
authors.initials: YF
authors.lastname: Xu
authors.affiliation: Department of Hepatobiliary Surgery, Qilu Hospital of Shandong University, Jinan 250012, Shandong Province, China. Electronic address: surgeonchenyuxin@163.com.
authors.forename: Y-X
authors.initials: YX
authors.lastname: Chen
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006331:Heart Diseases / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D006499:Hepatic Artery / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D008026:Ligation; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000209:etiology; D011788:Quality of Life; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000009:adverse effects
keywords: AVM; Arteriovenous malformations; Cardiac insufficiency; Hepatic artery; Liver; Portal hypertension