Fontan-Sprechstunde

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

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.

Study Overview

• Status: Recruiting
• Conditions: Univentricular Heart
• Intervention / Treatment: Diagnostic test: Univentricular Hearts

Detailed 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.

• Study Type: Interventional
• Enrollment (Anticipated): 200
• Phase: Not Applicable

Contacts and Locations

• Study Contact: Name: Sven Dittrich, Prof.; Phone Number: 09131 85 33750; Email: sven.dittrich@uk-erlangen.de
• Study Contact Backup: Name: Isabelle Schöffl, PD; Phone Number: 09131 85 33750; Email: isabelle.schoeffl@uk-erlangen.de

Study Locations

• Germany
  • Erlangen, Germany, 91054
    • Recruiting
    • Universitätsklinikum
    • Contact: Sven Dittrich, Prof.; Phone Number: 09131 85 33750; Email: sven.dittrich@uk-erlangen.de
    • Contact: Isabelle Schöffl, PD; Phone Number: 09131 85 33750; Email: isabelle.schoeffl@uk-erlangen.de

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 6 years to 60 years (Child, Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

Inclusion Criteria:

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

Exclusion Criteria:

• no exclusion criteria

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Prevention
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Other: TCPC completed; All patients after TCPC completion	Diagnostic test: Univentricular Hearts; Prevention when factors are recognized

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
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

Collaborators and Investigators

• Sponsor: University of Erlangen-Nürnberg Medical School
• Investigators: Study Chair: Sven Dittrich, Prof., Universitatsklinikum Erlangen

Study record dates

Study Major Dates

• Study Start (Actual): September 1, 2020
• Primary Completion (Anticipated): September 1, 2031
• Study Completion (Anticipated): September 1, 2041

Study Registration Dates

• First Submitted: September 21, 2022
• First Submitted That Met QC Criteria: September 28, 2022
• First Posted (Actual): October 3, 2022

Study Record Updates

• Last Update Posted (Actual): October 3, 2022
• Last Update Submitted That Met QC Criteria: September 28, 2022
• Last Verified: September 1, 2022

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Heart Diseases; Cardiovascular Diseases; Congenital Abnormalities; Heart Defects, Congenital; Cardiovascular Abnormalities; Univentricular Heart
• Other Study ID Numbers: 21-195_1-B

Drug and device information, study documents

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