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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

    作者

    Liu, ZC;Lu, XF;Yang, H;Liu, HD;Song, X;Ning, SL;Xu, YF;Chen, YX

    作者单位

    [Liu, Z-C.; Lu, X-F.; Yang, H.; Liu, H-D.; Ning, S-L.; Xu, Y-F.; Chen, Y-X.] Shandong Univ, Qilu Hosp, Dept Hepatobiliary Surg, Jinan 250012, Shandong, Peoples R China.;-;[Lu, X-F.] Jinan Cent Hosp, Dept Gen Surg, Jinan 250013, Shandong, Peoples R China.;-;[Song, X.] Shandong Prov Hosp, Dept Gen Surg, Jinan 250021, Shandong, Peoples R China.

    摘要

    Objective/background: 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 gamma-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. (C) 2015 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.

    关键词

    WEBER-RENDU DISEASE; QUALITY-OF-LIFE; ROW HELICAL CT; VASCULAR MALFORMATIONS; LIVER-TRANSPLANTATION; ARTERIOVENOUS-FISTULAS; VARICEAL HEMORRHAGE; OSLER-DISEASE; INVOLVEMENT; EMBOLIZATION
基本信息

  • 所属机构:

    归属医师: 宋勰

    PMID:26837480

    UT:000374618400028

    刊名:EUROPEAN JOURNAL OF VASCULAR AND ENDOVASCULAR SURGERY

    年,卷(期):2016年51卷4期

    页码:594-601

    DOI:10.1016/j.ejvs.2015.12.020

    附件:

    收录:   SCIE