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Clinical Trial Details — Status: Recruiting

Administrative data

NCT number NCT05620030
Other study ID # 528_20 B
Secondary ID
Status Recruiting
Phase N/A
First received
Last updated
Start date May 20, 2021
Est. completion date May 20, 2041

Study information

Verified date September 2022
Source University of Erlangen-Nürnberg Medical School
Contact Sven Dittrich, Prof.
Phone 09131 85 33750
Email sven.dittrich@uk-erlangen.de
Is FDA regulated No
Health authority
Study type Interventional

Clinical Trial Summary

Univentricular heart defects are among the most complex congenital malformations. The treatment of these patients usually includes 3 operations and accompanying diagnostics, some of which are invasive, within the first 3 to 4 years of life until the "fontan circulation" is established. Unfortunately, the group of patients with a univentricular cardiac malformation is also the group with the highest mortality rate until the Fontan circulation is established. The expected mortality varies depending on the complexity of the underlying intracardiac and extracardiac malformations and possible concomitant morbidity; According to data from the National Quality Assurance in Germany, it is currently up to 30% in patients with hypoplastic left heart syndrome. However, the treatment methods are well standardized and there has been a clinical protocol in Erlangen since 2008 that includes diagnostic examinations using cardiac catheterization (HKU) and magnetic resonance imaging (MRT), as well as non-invasive, instrument-based diagnostics and targeted blood tests in a consistent sequence before and after the operations regulates. The Erlangen protocol ends with the cardiac MRT six months after the Fontan operation (syn: total cavopulmonary connection [TCPC]). If MRT imaging is not possible (e.g. if a cardiac pacemaker is present) or if there is a clinical indication (e.g. an obstruction noticeable on MRT or the presence of an "overflow with right-left shunt"), HKU is performed in the first year after TCPC. These examinations are used to depict hemodynamics, cardiac function, vascular development, and lymphatic drainage disorders in order to evaluate the preoperative risk, adapt treatment strategies to the individual, and identify complications at an early stage. This concept is based on clinical experience and current scientific findings and has proven itself in everyday clinical practice since its introduction for Erlangen-Fontan patients and has proven to be very helpful for individualized treatment. After a successful Fontan operation, the mortality risk in childhood is only low. In the long term, however, some of the patients unfortunately suffer from Fontan-typical long-term complications with protein-loss enteropathy having to be mentioned in particular, which is described in the literature with an incidence of 3-14% and today still a 5-year incidence-has a mortality risk of 6-12%.


Description:

Univentricular heart defects are among the most complex congenital malformations. The treatment of these patients usually includes 3 operations and accompanying diagnostics, some of which are invasive, within the first 3 to 4 years of life until the "fontan circulation" is established. Unfortunately, the group of patients with a univentricular cardiac malformation is also the group with the highest mortality rate until the Fontan circulation is established. The expected mortality varies depending on the complexity of the underlying intracardiac and extracardiac malformations and possible concomitant morbidity; According to data from the National Quality Assurance in Germany, it is currently up to 30% in patients with hypoplastic left heart syndrome. However, the treatment methods are well standardized and there has been a clinical protocol in Erlangen since 2008 that includes diagnostic examinations using cardiac catheterization (HKU) and magnetic resonance imaging (MRT), as well as non-invasive, instrument-based diagnostics and targeted blood tests in a consistent sequence before and after the operations regulates. The Erlangen protocol ends with the cardiac MRT six months after the Fontan operation (syn: total cavopulmonary connection [TCPC]). If MRT imaging is not possible (e.g. if a cardiac pacemaker is present) or if there is a clinical indication (e.g. an obstruction noticeable on MRT or the presence of an "overflow with right-left shunt"), HKU is performed in the first year after TCPC. These examinations are used to depict hemodynamics, cardiac function, vascular development, and lymphatic drainage disorders in order to evaluate the preoperative risk, adapt treatment strategies to the individual, and identify complications at an early stage. This concept is based on clinical experience and current scientific findings and has proven itself in everyday clinical practice since its introduction for Erlangen-Fontan patients and has proven to be very helpful for individualized treatment.


Recruitment information / eligibility

Status Recruiting
Enrollment 30
Est. completion date May 20, 2041
Est. primary completion date May 20, 2031
Accepts healthy volunteers No
Gender All
Age group 6 Years to 60 Years
Eligibility Inclusion Criteria: - congenital heart defect of the univentricular type and the clinical necessity of a multi-stage treatment in terms of the Fontan procedure. Exclusion Criteria: - no exclusion criteria

Study Design


Related Conditions & MeSH terms


Intervention

Other:
Univentricular Heart
Prevention of complications when discovered

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 Number of correct findings in patients after Fontan palliation to assess the outcome after TCPC correct perioperative course after TCPC, correct anatomy after surgery without vascular or anastomotic obstructions, correct cardiovascular function, correct lymphatic vessel presentation, correct liver sonography, blood protein values in the normal range, absence of clinical symptoms 50 years
Primary Heart or valvular insufficiency assessed by echocardiography and MRI Measured through ejaction fraction (EF; [EF]=%) and through visual assessment of the valves with e.g. doppler echocardiography.
Additionally, measurement of the same values is performed through MRI
50 years
Primary Pathologic presentation of lymph vessels assessed by MRI Described through various scores (Biko et al., Schröder et al.) 50 years
Primary Hypoalbuminemia, hypoproteinemia measured through blood analysis (in g/L) 50 years
Primary Number of patients with Protein-losing enteropathy (PLE) or plastic bronchitis Assessed by clinical diagnosis 50 years
Primary Number of patients with "Failing Fontan" Examples would be Fontan take-down, heart transplant or passing away 50 years
Primary Prevalence and cause of early post-Fontan morbidity assessed by clinical diagnosis 50 years
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