Liver Disease, Myocardial Fibrosis and Collaterals in the Adult Fontan Patient a Metabolomics and Proteomics Approach

Inclusion Criteria: * written informed consent of participants * age at testing of ≥18 years * 8 h fasting before blood sampling * Fontan circulation (patients) * biventricular heart without structural or functional abnormality (controls) Exclusion Criteria: * Protein losing enteropathy (PLE) (patients are defined as "PLE positive" if there is/are serum protein \<5g/dL and serum albumin \<3g/dL (duration for more than 3 months and exclusion of other causes for hypoproteinemia) and documented enteric protein loss: faecal alpha-1-antitrypsin ≥400 µg/g * medication directly affecting metabolic state, such as cholesterol-lowering agents, or haemodynamic state, such as beta-blockers or sildenafil, with the exception of angiotensin converting enzyme inhibitors, diuretics, and anticoagulants * atrial or ventricular arrhythmia * coronary artery disease (history of myocardial infarction, myocardial revascularisation, percutaneous coronary intervention, or coronary artery bypass surgery) * any metabolic disease, such as diabetes mellitus * malignancy * obesity (body mass index (BMI) \>25 kg/m2) * underweight (BMI \< 18 kg/m2) * renal disease * inflammatory disease such as acute or chronic infection * myeloproliferative disorder * pregnancy or lactation * malnourishment * mental handicap not allowing valid consent to participation in the study or CMR * need of sedation or general anesthesia for CMR * typical contraindications for performing CMR such as metal-containing mechanical or …