Prospective Evaluation of Univentricular Hearts
Primary Purpose
Univentricular Heart
Status
Recruiting
Phase
Not Applicable
Locations
Germany
Study Type
Interventional
Intervention
Univentricular Heart
Sponsored by
About this trial
This is an interventional health services research trial for Univentricular Heart
Eligibility Criteria
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
Sites / Locations
- UniversitätsklinikumRecruiting
Arms of the Study
Arm 1
Arm Type
Experimental
Arm Label
Univentricular Heart
Arm Description
All patients with univentricular hearts
Outcomes
Primary Outcome Measures
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
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
Pathologic presentation of lymph vessels assessed by MRI
Described through various scores (Biko et al., Schröder et al.)
Hypoalbuminemia, hypoproteinemia
measured through blood analysis (in g/L)
Number of patients with Protein-losing enteropathy (PLE) or plastic bronchitis
Assessed by clinical diagnosis
Number of patients with "Failing Fontan"
Examples would be Fontan take-down, heart transplant or passing away
Prevalence and cause of early post-Fontan morbidity
assessed by clinical diagnosis
Secondary Outcome Measures
Full Information
NCT ID
NCT05620030
First Posted
September 21, 2022
Last Updated
November 10, 2022
Sponsor
University of Erlangen-Nürnberg Medical School
1. Study Identification
Unique Protocol Identification Number
NCT05620030
Brief Title
Prospective Evaluation of Univentricular Hearts
Official Title
Evaluation of the Standard Data Collected as Part of the Erlangen Protocol for the Treatment and Monitoring of Patients With Univentricular Heart Defects in Terms of the Fontan Procedure
Study Type
Interventional
2. Study Status
Record Verification Date
September 2022
Overall Recruitment Status
Recruiting
Study Start Date
May 20, 2021 (Actual)
Primary Completion Date
May 20, 2031 (Anticipated)
Study Completion Date
May 20, 2041 (Anticipated)
3. Sponsor/Collaborators
Responsible Party, by Official Title
Sponsor
Name of the Sponsor
University of Erlangen-Nürnberg Medical School
4. Oversight
Studies a U.S. FDA-regulated Drug Product
No
Studies a U.S. FDA-regulated Device Product
No
Data Monitoring Committee
No
5. Study Description
Brief 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%.
Detailed 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.
6. Conditions and Keywords
Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
Univentricular Heart
7. Study Design
Primary Purpose
Health Services Research
Study Phase
Not Applicable
Interventional Study Model
Single Group Assignment
Masking
None (Open Label)
Allocation
N/A
Enrollment
30 (Anticipated)
8. Arms, Groups, and Interventions
Arm Title
Univentricular Heart
Arm Type
Experimental
Arm Description
All patients with univentricular hearts
Intervention Type
Other
Intervention Name(s)
Univentricular Heart
Intervention Description
Prevention of complications when discovered
Primary Outcome Measure Information:
Title
Number of correct findings in patients after Fontan palliation to assess the outcome after TCPC
Description
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
Time Frame
50 years
Title
Heart or valvular insufficiency assessed by echocardiography and MRI
Description
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
Time Frame
50 years
Title
Pathologic presentation of lymph vessels assessed by MRI
Description
Described through various scores (Biko et al., Schröder et al.)
Time Frame
50 years
Title
Hypoalbuminemia, hypoproteinemia
Description
measured through blood analysis (in g/L)
Time Frame
50 years
Title
Number of patients with Protein-losing enteropathy (PLE) or plastic bronchitis
Description
Assessed by clinical diagnosis
Time Frame
50 years
Title
Number of patients with "Failing Fontan"
Description
Examples would be Fontan take-down, heart transplant or passing away
Time Frame
50 years
Title
Prevalence and cause of early post-Fontan morbidity
Description
assessed by clinical diagnosis
Time Frame
50 years
10. Eligibility
Sex
All
Minimum Age & Unit of Time
6 Years
Maximum Age & Unit of Time
60 Years
Accepts Healthy Volunteers
No
Eligibility Criteria
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
Central Contact Person:
First Name & Middle Initial & Last Name or Official Title & Degree
Sven Dittrich, Prof.
Phone
09131 85 33750
Email
sven.dittrich@uk-erlangen.de
First Name & Middle Initial & Last Name or Official Title & Degree
Isabelle Schöffl, PD
Phone
09131 85 33750
Email
isabelle.schoeffl@uk-erlangen.de
Overall Study Officials:
First Name & Middle Initial & Last Name & Degree
Sven Dittrich, Prof.
Organizational Affiliation
Universitätsklinikum Erlangen
Official's Role
Study Chair
Facility Information:
Facility Name
Universitätsklinikum
City
Erlangen
ZIP/Postal Code
91054
Country
Germany
Individual Site Status
Recruiting
Facility Contact:
First Name & Middle Initial & Last Name & Degree
Sven Dittrich, Prof.
Phone
09131 85 33750
Email
sven.dittrich@uk-erlangen.de
First Name & Middle Initial & Last Name & Degree
Isabelle Schöffl, PD
Phone
09131 85 33750
Email
isabelle.schoeffl@uk-erlangen.de
12. IPD Sharing Statement
Plan to Share IPD
No
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Prospective Evaluation of Univentricular Hearts
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