Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2022 Sep 24;11(19):5624.
doi: 10.3390/jcm11195624.

Perioperative Complications and Long-Term Follow-Up of Liver Transplantation in Hemorrhagic Hereditary Telangiectasia: Report of Three Cases and Systematic Review

Antoni Riera-Mestre  1   2   3   4 , Pau Cerdà  1   2   3 , Yoelimar Carolina Guzmán  5 , Adriana Iriarte  1   2   3 , Alba Torroella  5 , José María Mora-Luján  1   2   3 , Jose Castellote  1   3   6 , Amelia Hessheimer  5   7 , Constantino Fondevila  5   7 , Laura Lladó  3   4   8
Affiliations
Review

Perioperative Complications and Long-Term Follow-Up of Liver Transplantation in Hemorrhagic Hereditary Telangiectasia: Report of Three Cases and Systematic Review

Antoni Riera-Mestre et al. J Clin Med. .

Abstract

The aim was to describe three patients with hemorrhagic hereditary telangiectasia (HHT) requiring liver transplantation (LT) and to perform a systematic review focusing on surgical complications and long-term follow-up. Unrestricted searches of the Medline and Embase databases were performed through February 2022. Forty-five studies were selected including 80 patients plus the three new reported patients, 68 (81.9%) were female and mean age was 50 (27-72) years. Main indications for LT were high-output cardiac failure (n = 40; 48.2%), ischemic cholangitis (n = 19; 22.9%), and a combination of both conditions (n = 13;15.6%). Mean cold ischemic time and red blood cell units transfused during LT were 554 (300-941) minutes and 11.4 (0-88) units, respectively. Complications within 30 days were described in 28 (33.7%) patients, mainly bleeding complications in 13 patients, hepatic artery (HA) thrombosis in four and hepatic vein thrombosis in one. Mean follow-up was 76.4 (1-288) months, and during it, four new patients developed thrombotic complications in HA, HA aneurysm, celiac artery, and the portal-splenic-mesenteric vein. HHT relapse in the transplant allograft was detected in 13 (17.1%) patients after 1-19 years (including two fatal recurrences). Overall mortality was 12%. In conclusion, previous assessment of HA anatomy and hyperdynamic circulatory state could reduce LT complications. The risk of relapse in the hepatic graft supports a multidisciplinary follow-up for HHT patients with LT.

Keywords: angiogenesis; hemorrhagic hereditary telangiectasia; liver transplantation; vascular malformation.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript; or in the decision to publish the results.

Figures

Figure 1
Figure 1
PRISMA flow diagram.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Shovlin C.L., Guttmacher A.E., Buscarini E., Faughnan M.E., Hyland R.H., Westermann C.J., Kjeldsen A.D., Plauchu H. Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) Am. J. Med. Genet. 2000;91:66–67. doi: 10.1002/(SICI)1096-8628(20000306)91:1<66::AID-AJMG12>3.0.CO;2-P. - DOI - PubMed
    1. McDonald J., Wooderchak-Donahue W., VanSant Webb C., Whitehead K., Stevenson D.A., Bayrak-Toydemir P. Hereditary hemorrhagic telangiectasia: Genetics and molecular diagnostics in a new era. Front. Genet. 2015;6:1. doi: 10.3389/fgene.2015.00001. - DOI - PMC - PubMed
    1. Sánchez-Martínez R., Iriarte A., Mora-Luján J.M., Patier J.L., López-Wolf D., Ojeda A., Torralba M.A., Juyol M.C., Gil R., Añón S., et al. RiHHTa Investigators of the Rare Diseases Working Group from the Spanish Society of Internal Medicine. Current HHT genetic overview in Spain and its phenotypic correlation: Data from RiHHTa registry. Orphanet J. Rare Dis. 2020;15:138. doi: 10.1186/s13023-020-01422-8. - DOI - PMC - PubMed
    1. Alsina-Sanchís E., García-Ibáñez Y., Figueiredo A., Riera-Domingo C., Figueras A., Matias-Guiu X., Casanovas O., Botella L.M., Pujana M.A., Riera-Mestre A., et al. ALK1 Loss Results in Vascular Hyperplasia in Mice and Humans through PI3K Activation. Arter. Thromb. Vasc. Biol. 2018;38:1216–1229. doi: 10.1161/ATVBAHA.118.310760. - DOI - PubMed
    1. Iriarte A., Figueras A., Cerdà P., Mora J.M., Jucglà A., Penín R., Viñals F., Riera-Mestre A. PI3K (Phosphatidylinositol 3-Kinase) Activation and Endothelial Cell Proliferation in Patients with Hemorrhagic Hereditary Telangiectasia Type 1. Cells. 2019;8:971. doi: 10.3390/cells8090971. - DOI - PMC - PubMed