Fontan-Sprechstunde

Univentricular Heart Clinical Trial

Official title:

Prospektive Erfassung Von standardmäßig Erhobenen Daten im Rahmen Der Erlanger Fontan-Sprechstunde Zur Behandlung Und Überwachung Von PatientInnen Mit univentrikulärem Herzfehler im Sinne Einer Fontan-Zirkulation

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT05563376
• Other study ID #: 21-195_1-B
• Status: Recruiting
• Phase: N/A
• Start date: September 1, 2020
• Est. completion date: September 1, 2041

Study information

• Verified date: September 2022
• Source: University of Erlangen-Nürnberg Medical School
• Contact: Isabelle Schöffl, PD
• Phone: 09131 85 33750
• Email: isabelle.schoeffl[@]uk-erlangen.de
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial 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.

Description:

In particular, the following points will be scientifically analyzed:

1. The development of lymphatic drainage disorders (7-9)

2. The cellular changes of the immunological system and metabolome (10) In addition to the clinical routine, weight-adapted EDTA blood for the isolation of peripheral blood mononuclear cells (PBMCs) for the scientific investigation of immunological changes and serum for the analysis of the metabolome will be examined at three times (Figure 1: Times 2, 4, 7) as part of a routine blood sample (10).

3. The recording and treatment of psychological, social and developmental abnormalities with the help of standardized questionnaires.

4. The evaluation of special sonography examinations of the liver and kidney in the long-term course, as well as their treatment.

5. The assessment of the hemodynamic peculiarities and the classification with regard to Cardiac function in the long-term course of patients with Fontan circulation.

6. The assessment of various laboratory parameters as risk parameters for the development of complications.

7. The recording of cardiopulmonary performance in the long-term course and its ability to be influenced by education regarding sporting activity and the handing over of an individualized training plan.

8. The influence of physical activity on possible complications of Fontan circulation.

9. The influence of early psychological, nutritional, or social intervention in the event of abnormalities on later complications of Fontan circulation.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 200
• Est. completion date: September 1, 2041
• Est. primary completion date: September 1, 2031
• Accepts healthy volunteers: No
• Gender: All
• Age group: 6 Years to 60 Years

Eligibility

Inclusion Criteria:

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

Exclusion Criteria:

• no exclusion criteria

Related Conditions & MeSH terms

• Univentricular Heart

Intervention

Diagnostic Test:
• Univentricular Hearts
Prevention when factors are recognized

Locations

Country	Name	City	State
Germany	Universitätsklinikum	Erlangen

Sponsors (1)

Lead Sponsor	Collaborator
University of Erlangen-Nürnberg Medical School

Country where clinical trial is conducted

Germany, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Reducing mortality and morbidity and improving quality of life in long-term care for Fontan patients	Fontan Tunnel Obstruction; 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; 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; 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); Hypoproteinaemia defined as serum albumin <3.4 mg/dl (4), total protein (TP) <50 g/l; protein-losing enteropathy (PLE) defined as (3, 4); Failing Fontan defined as Operative Fontan Take-down, Listing for Heart Transplantation and/or Death	50 Jahre