Prospective Evaluation of Univentricular Hearts

Univentricular Heart Clinical Trial

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

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT05620030
• Other study ID #: 528_20 B
• Status: Recruiting
• Phase: N/A
• 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
• 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

Related Conditions & MeSH terms

• Univentricular Heart

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