Investigating liver and heart issues in Fontan patients

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

This study looks at how liver and heart problems develop in adults with Fontan circulation to see if certain body processes can help us find better ways to diagnose and treat these issues.

Quick facts

• Study type: Observational
• Enrollment: 300 (estimated)
• Ages: 18 Years and up
• Sex: All
• Sponsor: Medical University Innsbruck (other)
• Locations: 1 site (Innsbruck)
• Trial ID: NCT04764305 on ClinicalTrials.gov

What this trial studies

This observational study aims to explore the mechanisms behind liver and heart fibrosis, as well as collateral vessel formation in adults with Fontan circulation. The researchers will focus on the metabolic processes involving phospholipids, amino acids, and bile acids, alongside inflammatory markers to understand the underlying pro-inflammatory conditions. By linking these factors, the study seeks to provide insights into the pathophysiology of Fontan patients and identify potential diagnostic or therapeutic targets for those at risk of developing these complications.

Who should consider this trial

Good fit: Ideal candidates include adults aged 18 and older with Fontan circulation and those with a biventricular heart without structural or functional abnormalities.

Not a fit: Patients with protein losing enteropathy or those on medications that significantly affect metabolic or hemodynamic states may not benefit from this study.

Why it matters

Potential benefit: If successful, this study could lead to improved diagnostic and therapeutic strategies for patients with Fontan circulation suffering from liver and heart complications.

How similar studies have performed: While this study addresses a specific patient population, similar approaches in investigating metabolic and inflammatory mechanisms in congenital heart disease have shown promise, suggesting potential for success.

Eligibility criteria

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

Where this trial is running

Innsbruck

• Medical University of Innsbruck — Innsbruck, Austria (RECRUITING)

Study contacts

• Principal investigator: Miriam Michel, MD — Medical University of Innsbruck
• Study coordinator: Maria-Miriam Michel, MD

Conditions: Patient Participation, Heart Defects, Congenital, Liver Diseases, Collateral Circulation, Any Site, Myocardial Fibrosis, Fontan, congenital heart disease, inflammation