Pre-emptive Transjugular Intrahepatic Portosystemic Shunt (TIPS) in Cystic Fibrosis Related Liver Disease

NCT05037643 | Completed

• 1 other identifier: B67020071504
• Study Type: interventional
• Target: 5
• Locations: 0 countries
• Sites: N/A

Brief Summary

Portal hypertension (PHT) and its sequelae are the most clinically important manifestation in cystic fibrosis related liver disease (CFLD), with end-stage liver failure as a late and rare manifestation. The aim is to evaluate the safety and efficacy of a pre-emptive Transjugular Intrahepatic Portosystemic Shunt (TIPS) for the prophylaxis of variceal bleeding in pediatric CFLD patients with subclinical non-cirrhotic portal hypertension (NCPH)

Conditions

Cystic Fibrosis Liver Disease; Portal Hypertension Due to Cystic Fibrosis (Disorder); Portal Hypertension, Noncirrhotic

Keywords

Transjugular Intrahepatic Portosystemic Shunt; Gastrointestinal bleeding; Hypersplenism

Outcome Measures

Primary Outcomes (1)

• Variceal bleeding

  Variceal bleeding, diagnosed on endoscopy, is regarded as the main complication of portal hypertension from cystic fibrosis liver disease

  Through study completion, an average of 10 year

Secondary Outcomes (1)

• Hypersplenism

  Through study completion, an average of 10 year

Other Outcomes (4)

• Liver transplant/splenectomy-free survival

  Through study completion, an average of 10 year

• Model for end-stage liver disease (MELD) score

  Through study completion, an average of 10 year

• Body Mass Index (BMI) for age Z-score

  Through study completion, an average of 10 year

Study Arms (1)

Pre-emptive TIPS in Cystic Fibrosis Related Liver Disease and non-cirrhotic portal hypertension

EXPERIMENTAL

Patients with CFLD without cirrhosis were eligible for a pre-emptive TIPS, when early (asymptomatic) signs of portal hypertension. All procedures were performed under general anaesthesia by an experienced interventional radiologist. Depending on the patient's age and physiognomy, TIPS was created following a conventional transjugular technique as for adults or by a dedicated combined percutaneous transhepatic-transjugular (PIPS) approach for small children. Routinely, an expanded polytetrafluoroethylene-covered endoprosthesis was used for shunt creation. If the sheath could not be negotiated into the main portal vein, a self-expandable, non-covered stent was placed. We did not pursue a minimum gradient reduction. Percutaneous liver biopsy was performed during TIPS procedure to confirm the diagnosis of fibrosis or cirrhosis.

Procedure: TIPS

Interventions

TIPS PROCEDURE

Transjugular Intrahepatic Portosystemic Shunt is performed to prevent / treat symptomatic portal hypertension

Pre-emptive TIPS in Cystic Fibrosis Related Liver Disease and non-cirrhotic portal hypertension

Eligibility Criteria

• Age: Up to 18 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64)

You may qualify if:

• Cystic fibrosis
• Liver disease detected by an abnormal physical examination (hepatomegaly or splenomegaly, confirmed on US), abnormalities of liver function tests (increase of AST, ALT, GGT levels above the upper normal limits) or ultrasonographic evidence of liver involvement (US liver score ≥ 5).
• Indirect signs of portal hypertension on Doppler US
• Progressive portal hypertension / liver disease on bi-monthly follow-up, evaluated by physical examination, blood analysis and US

You may not qualify if:

• Cirrhosis on biopsy
• Symptomatic portal hypertension (Portosytemic pressure gradient \> 10 mmHg)

Sponsors & Collaborators

• University Hospital, Ghent: lead

MeSH Terms

Conditions

Hypertension, Portal; Gastrointestinal Hemorrhage; Hypersplenism

Interventions

Portasystemic Shunt, Transjugular Intrahepatic

Condition Hierarchy (Ancestors)

Liver Diseases; Digestive System Diseases; Gastrointestinal Diseases; Hemorrhage; Pathologic Processes; Pathological Conditions, Signs and Symptoms; Splenic Diseases; Lymphatic Diseases; Hemic and Lymphatic Diseases

Intervention Hierarchy (Ancestors)

Portasystemic Shunt, Surgical; Anastomosis, Surgical; Surgical Procedures, Operative; Vascular Grafting; Vascular Surgical Procedures; Cardiovascular Surgical Procedures

Study Officials

• Luc Defreyne, M.D., Ph.D

  University Ghent Hospital

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: PREVENTION
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Model Details: Prospective single-arm study

Study Record Dates

• First Submitted: August 16, 2021
• First Posted: September 8, 2021
• Study Start: June 1, 2007
• Primary Completion: December 1, 2020
• Study Completion: December 1, 2020
• Last Updated: September 8, 2021

Record last verified: 2021-08

Data Sharing

• IPD Sharing: Will not share