Prospective Evaluation of Univentricular Hearts

NCT05620030 | Recruiting

• 1 other identifier: 528_20 B
• Study Type: interventional
• Target: 30
• Locations: 1 country
• Sites: 1

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%.

Conditions

Univentricular Heart

Outcome Measures

Primary Outcomes (7)

• 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

• 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

• Pathologic presentation of lymph vessels assessed by MRI

  Described through various scores (Biko et al., Schröder et al.)

  50 years

• Hypoalbuminemia, hypoproteinemia

  measured through blood analysis (in g/L)

  50 years

• Number of patients with Protein-losing enteropathy (PLE) or plastic bronchitis

  Assessed by clinical diagnosis

  50 years

• Number of patients with "Failing Fontan"

  Examples would be Fontan take-down, heart transplant or passing away

  50 years

• Prevalence and cause of early post-Fontan morbidity

  assessed by clinical diagnosis

  50 years

Study Arms (1)

Univentricular Heart

EXPERIMENTAL

All patients with univentricular hearts

Other: Univentricular Heart

Interventions

Univentricular Heart OTHER

Prevention of complications when discovered

Univentricular Heart

Eligibility Criteria

• Age: 6 Years - 60 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64)

You may qualify if:

• congenital heart defect of the univentricular type and the clinical necessity of a multi-stage treatment in terms of the Fontan procedure.

Sponsors & Collaborators

• University of Erlangen-Nürnberg Medical School: lead

Study Sites (1)

Universitätsklinikum

Erlangen, 91054, Germany

RECRUITING

MeSH Terms

Conditions

Univentricular Heart

Condition Hierarchy (Ancestors)

Heart Defects, Congenital; Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Sven Dittrich, Prof.

  Universitätsklinikum Erlangen

  STUDY CHAIR

Central Study Contacts

Sven Dittrich, Prof.

CONTACT

09131 85 33750; sven.dittrich@uk-erlangen.de

Isabelle Schöffl, PD

CONTACT

09131 85 33750; isabelle.schoeffl@uk-erlangen.de

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: HEALTH SERVICES RESEARCH
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: September 21, 2022
• First Posted: November 17, 2022
• Study Start: May 20, 2021
• Primary Completion (Estimated): May 20, 2031
• Study Completion (Estimated): May 20, 2041
• Last Updated: November 17, 2022

Record last verified: 2022-09

Data Sharing

• IPD Sharing: Will not share

Locations

DE (1)