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

Summary

Out objective is to identify the mechanisms that promote hepatic and myocardial fibrosis, and collateral vessel formation in patients with complex congenital heart disease and Fontan circulation.

Eligibility

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 electronic implants
* claustrophobia

Conditions7

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