Recurrence of Hereditary Hemorrhagic Telangiectasia (HHT) After Liver Transplantation (HHT)

Recurrence of Hereditary Hemorrhagic Telangiectasia (HHT) After Liver Transplantation: Clinical Implications and Physiopathological Insights.

Liver transplantation (LT) has been proposed as a curative treatment in hereditary hemorrhagic telangiectasia (HHT) with severe hepatic involvement. The investigating team provides a long-term evaluation of graft status after LT for HHT with a focus on the risk of recurrence. The present study included all patients prospectively followed up after LT for HHT in the Lyon Liver Transplant Unit from 1993 to 2010 with a survival of more than 1 year.

Study Overview

• Status: Completed
• Conditions: Liver Transplant; Hereditary Haemorrhagic Telangiectasia
• Intervention / Treatment: Other: Data collection from standard follow-up after liver transplant

• Study Type: Observational
• Enrollment (Actual): 14

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: ADULT; OLDER_ADULT; CHILD
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Hereditary Hemorrhagic Telangiectasia (HHT) patient who underwent liver transplant

Description

Inclusion Criteria:

• Hereditary Hemorrhagic Telangiectasia (HHT) patient who underwent liver transplant for HHT

Exclusion Criteria:

• Patient who died in the year following transplantation

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort

Intervention / Treatment

Liver transplant in Hereditary Hemorrhagic Telangiectasia; Hereditary Hemorrhagic Telangiectasia (HHT) patients who underwent a liver transplant in Lyon between 1993 and 2010, and who survived more than 1 year after transplantation.

Other: Data collection from standard follow-up after liver transplant; All patients underwent regular follow-up every 6 to 12 months after the first year post-liver transplant (LT). Complete laboratory investigations were performed at each visit. Doppler ultrasonography was performed every 1 to 3 years after LT. Computed tomography (CT) scan and/ or magnetic resonance imaging (MRI) was performed at 1, 5, 10, 15, and 20 years after LT, or when clinically indicated. All available radiological material was reviewed. Cardiac evaluation was performed regularly in patients who received transplant for cardiac failure.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Change in graft status after liver transplant for Hereditary Haemorrhagic Telangiectasia (HHT) (risk of recurrence)	Recurrent clinical examinations (including laboratory, histological and radiological investigations)	Every 6 months after transplantation up to 5 years

Collaborators and Investigators

• Sponsor: Hospices Civils de Lyon
• Investigators: Principal Investigator: Jérôme DUMORTIER, MD, Hospices Civils de Lyon (Hôpital Edouard Herriot )

Study record dates

Study Major Dates

• Study Start (ACTUAL): January 1, 2011
• Primary Completion (ACTUAL): February 1, 2018
• Study Completion (ACTUAL): December 1, 2018

Study Registration Dates

• First Submitted: May 2, 2019
• First Submitted That Met QC Criteria: May 6, 2019
• First Posted (ACTUAL): May 8, 2019

Study Record Updates

• Last Update Posted (ACTUAL): May 8, 2019
• Last Update Submitted That Met QC Criteria: May 6, 2019
• Last Verified: May 1, 2019

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Pathologic Processes; Cardiovascular Diseases; Vascular Diseases; Disease Attributes; Congenital Abnormalities; Hematologic Diseases; Hemorrhagic Disorders; Hemostatic Disorders; Cardiovascular Abnormalities; Vascular Malformations; Recurrence; Telangiectasis; Telangiectasia, Hereditary Hemorrhagic
• Other Study ID Numbers: HHT

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No