Clinical trial NCT05620030: 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
Overall status: RECRUITING
Phase: NA
Conditions: Univentricular Heart
Lead sponsor: University of Erlangen-Nürnberg Medical School
Sponsor class: OTHER
First posted: 2022-11-17
Enrollment: 30
Sex: ALL
Minimum age (years): 6
Maximum age (years): 60
Standard ages: CHILD
ADULT
Accepts healthy volunteers: no
Allocation: NA
Intervention model: SINGLE_GROUP
Primary purpose: HEALTH_SERVICES_RESEARCH

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.

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

Primary outcomes:
measure: Number of correct findings in patients after Fontan palliation to assess the outcome after TCPC
timeFrame: 50 years
measure: Heart or valvular insufficiency assessed by echocardiography and MRI
timeFrame: 50 years
measure: Pathologic presentation of lymph vessels assessed by MRI
timeFrame: 50 years
measure: Hypoalbuminemia, hypoproteinemia
timeFrame: 50 years
measure: Number of patients with Protein-losing enteropathy (PLE) or plastic bronchitis
timeFrame: 50 years
measure: Number of patients with "Failing Fontan"
timeFrame: 50 years
measure: Prevalence and cause of early post-Fontan morbidity
timeFrame: 50 years
Secondary outcomes:
Other outcomes:
Locations:
city: Erlangen
country: Germany
facility: Universitätsklinikum
Study officials:
affiliation: Universitätsklinikum Erlangen
name: Sven Dittrich, Prof.
role: STUDY_CHAIR
References: