Metabolic Remodeling in Fontan Patients | Clinical Trial

Key information

Conditions

Congenital Heart Disease Cardiovascular Abnormalities Cardiovascular Diseases Congenital Abnormalities Congenital, Hereditary, and Neonatal Diseases and Abnormalities Heart Defects, Congenital Heart Diseases Metabolic Diseases Metabolism Disorder Nutritional and Metabolic Diseases

Sex eligibility

All sexes

Study type

Observational

About this study

Patients who are born with just one single heart chamber need to undergo surgical therapy allowing the single heart chamber to pump the blood into the systemic circulation and allowing the blood to flow passively to the lungs (Fontan circulation). Regular ultrasound, cardiopulmonary exercise testing, and invasive diagnostic tools (catheterization, general anaesthesia needed) are necessary to early find out cardiac, vascular, or circulatory impairment. It is still very difficult to diagnose and therapy failure of this Fontan system early enough.

It is reported that in patients with a failing two-chambered heart, the energy source for the heart and the body in general switches from the use of lipids to the use of sugar and ketone bodies. First studies show decreased concentrations of membrane lipids in Fontan patients with a left dominant ventricle, and the energy metabolism has not been focused yet in those patients.

The investigators hypothesize that there are differences in the pattern of the structural metabolism in adult Fontan patients with a left-dominant vs. a right-dominant ventricle. Furthermore the investigators hypothesize that there are alterations in the energy metabolism in adult Fontan patients in comparison to healthy two-chambered controls, and that those alterations correlate with the grade of impairment of cardiopulmonary function.

With the help of a special biochemical examination (mass spectrometry-based Metabolomics study) blood of Fontan patients will be analyzed, and the results will be correlated with the results of ultrasound and cardiopulmonary exercise testing. The aim of this study is to establish sensitive blood markers indicating cardiac, vascular, circulatory or further organ dysfunction in Fontan patients. This should allow optimal Fontan system monitoring with an optimal timing of an additional invasive diagnostic catheterization and of nutritional, medical or interventional therapy.

Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

Fontan circulation (right systemic ventricle), biventricular heart with heart failure resp.
≥ 18 years
Written informed consent of patients
8 hours fasting period before blood sample

Exclusion criteria

Intake of medication directly affecting metabolic (e.g. metabolism of lipids (statine)) or hemodynamic state (e.g. beta-blockers, sildenafil) (other than angiotensin converting enzyme (ACE)-inhibitors and anticoagulation therapy)

Cachectic disease
Non-congestive hepatic or renal dysfunction
(Inherited) metabolic disorders

Treatment and study plan

Metabolomics

Diagnostic Test

Primary outcomes

Metabolism of lipids

Time frame: blood sampling at one single point of time (baseline/day 1) in the context of a planned patient presentation at the pediatric cardiology outpatient clinic (without assessment of changes)

Measurement of the serum concentration of the extended metabolism of lipids using the technique of Metabolomics (AbsoluteIDQ p180 kit, Biocrates Life Sciences AG, Innsbruck, Austria)
Metabolism of amino acids and related compounds

Time frame: blood sampling at one single point of time (baseline/day 1) in the context of a planned patient presentation at the pediatric cardiology outpatient clinic (without assessment of changes)

Measurement of the serum concentration of the extended metabolism of lamino acids using the technique of Metabolomics (AbsoluteIDQ p180 kit, Biocrates Life Sciences AG, Innsbruck, Austria)
Metabolism of carbohydrates

Time frame: blood sampling at one single point of time (baseline/day 1) in the context of a planned patient presentation at the pediatric cardiology outpatient clinic (without assessment of changes)

Measurement of the serum concentration of the extended metabolism of carbohydrates using the technique of Metabolomics (AbsoluteIDQ p180 kit, Biocrates Life Sciences AG, Innsbruck, Austria)
Metabolism of ketone bodies

Time frame: blood sampling at one single point of time (baseline/day 1) in the context of a planned patient presentation at the pediatric cardiology outpatient clinic (without assessment of changes)

Measurement of the serum concentration of ketone bodies using the technique of Metabolomics (AbsoluteIDQ p180 kit, Biocrates Life Sciences AG, Innsbruck, Austria)

Sponsors and collaborators

Lead sponsor

Medical University Innsbruck

Other

Collaborators

Biocrates Life Sciences AG, Innsbruck, Austria
Tiroler Wissenschaftsförderung

Registry information

Official study title

Metabolic Remodeling in Fontan Patients: a Metabolomics Study

Important dates

Study start: 2017
Primary completion: 2018
Study completion: 2019
First posted: Mar 22, 2019
Registry last updated: Feb 24, 2021

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

Published trials that share one or more normalized conditions with this study.

Completed