Metabolomics and Proteomics in the adult Fontan patient

Patients with complex congenital heart disease and established Fontan circulation exhibit alterations of all organ systems. Particularly the alteration of liver structure and function plays a central role. The hypothesis is that these alterations significantly contribute to the development of a failing Fontan circulation, and that the underlying driving mechanisms are altered regulatory processes in terms of inflammation and metabolism. Within the frame of our project, we will focus on these regulatory processes at the level of small molecules, the so-called metabolome and proteome. In collaboration with the Heart- and Diabetes Center Bad Oeynhausen, Germany, profiles of metabolism-derived analytes, of cell surface markers, cytokines and chemokines will be compared between Fontan patients with vs. without liver disease. Additionally, using non-invasive and invasive imaging and hemodynamic data, we will quantify each individuals status of Fontan circulation. By doing so, we aim to gain insight into the interaction of hepatic derangement, inflammatory and metabolic processes, and the incipient failure of Fontan circulation, optimally pointing out new therapeutic approaches.