Evaluation of Data Collected in the Erlangen Protocol for Treating and Monitoring Patients With Univentricular Heart Defects During the Fontan Procedure

What this Trial Is About

Researchers are studying patients with univentricular heart defects, which are complex congenital heart malformations that typically require three surgeries and various diagnostic tests within the first three to four years of life to establish the Fontan circulation. This condition carries a high risk of mortality until the Fontan circulation is established, with risks varying based on the heart defect's complexity and other health issues. The study employs a well-established clinical protocol from Erlangen that uses invasive and non-invasive diagnostic methods to monitor patients before and after surgery. The study follows the Erlangen protocol, which includes cardiac catheterization, magnetic resonance imaging (MRI), non-invasive diagnostics, and blood tests in a defined sequence before and after each operation. After the Fontan operation, a cardiac MRI is performed six months later, with additional catheterization if MRI is not possible or if clinical signs indicate complications. These procedures help evaluate heart function, blood flow, vascular development, and lymphatic drainage to guide personalized treatment and detect complications early. Participants will undergo scheduled diagnostic tests including imaging, blood tests, and heart function evaluations throughout their care. Researchers will monitor outcomes such as heart valve function, lymph vessel abnormalities, protein levels, and complications like protein-losing enteropathy over a long period, up to 50 years. The study aims to improve understanding of long-term outcomes and complications after the Fontan procedure while supporting individualized patient care.

Prospective Evaluation of Univentricular Hearts