Fontan-Sprechstunde

NCT05563376 | Recruiting

• 1 other identifier: 21-195_1-B
• Study Type: interventional
• Target: 200
• Locations: 1 country
• Sites: 1

Brief Summary

After successful Fontan surgery, the risk of mortality in childhood is only low. Unfortunately, some of the patients suffer from Fontan-typical long-term complications in the long-term course, whereby protein loss neuropathy must be mentioned in particular, which is described in the literature with an incidence of 3-14% (1, 2) and still has a 5-year risk of death of 6-12% today (2, 3). Protein loss tereopathy leads to loss of protein in the intestine and subsequently to diarrhea and edema. Other problems concern the liver, which can develop cirrhosis due to chronic congestion (4-6). Cardiac can lead to heart failure and arrhythmias. The registry study described in this protocol is intended to identify factors that influence the treatment outcome of patients in the Fontan circulation in the long term through systematic prospective documentation of the data from our standardized and guideline-oriented treatment.

Conditions

Univentricular Heart

Outcome Measures

Primary Outcomes (1)

• Reducing mortality and morbidity and improving quality of life in long-term care for Fontan patients

  1. Fontan Tunnel Obstruction 2. Fontan-typical klin. Symp. defined in points 5.+6. o. Presence of edema, ascites, susceptibility to infections, reduced resilience, cyanosis, lack of size and weight development, diarrhea 3. Heart or heart valve insufficiency defined as end-diastolic volume/BSA (EDP corrected) above the norm, ventricle ejection fraction (EF) \<50%, atrioventricular heart valve insufficiency (AVI) \>grade 2, aortic valve insufficiency \>grade 2 4. Pathological lymphatic vessel imaging defined as lymphatic vasodilation (LAE) type ≥ 3 according to Biko et al. (abnormal supraclavicular lymphatic vasodilation with infiltration of the mediastinum) (7). Presence of mesenteric lymphatic vascular congestion (8) 5. Hypoproteinaemia defined as serum albumin \<3.4 mg/dl (4), total protein (TP) \<50 g/l 6. protein-losing enteropathy (PLE) defined as (3, 4) 7. Failing Fontan defined as Operative Fontan Take-down, Listing for Heart Transplantation and/or Death

  50 Jahre

Study Arms (1)

TCPC completed

OTHER

All patients after TCPC completion

Diagnostic Test: Univentricular Hearts

Interventions

Univentricular Hearts DIAGNOSTIC_TEST

Prevention when factors are recognized

TCPC completed

Eligibility Criteria

• Age: 6 Years - 60 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64)

You may qualify if:

• congenital heart defect of the univentricular type that has led to Fontan circulation

Sponsors & Collaborators

• University of Erlangen-Nürnberg Medical School: lead

Study Sites (1)

Universitätsklinikum

Erlangen, 91054, Germany

RECRUITING

MeSH Terms

Conditions

Univentricular Heart

Condition Hierarchy (Ancestors)

Heart Defects, Congenital; Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Sven Dittrich, Prof.

  Universitätsklinikum Erlangen

  STUDY CHAIR

Central Study Contacts

Isabelle Schöffl, PD

CONTACT

09131 85 33750; isabelle.schoeffl@uk-erlangen.de

Sven Dittrich, Prof.

CONTACT

09131 85 33750; sven.dittrich@uk-erlangen.de

Study Design

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

Study Record Dates

• First Submitted: September 21, 2022
• First Posted: October 3, 2022
• Study Start: September 1, 2020
• Primary Completion (Estimated): September 1, 2031
• Study Completion (Estimated): September 1, 2041
• Last Updated: October 3, 2022

Record last verified: 2022-09

Locations

DE (1)