Metabolic Remodeling in Fontan Patients

NCT ID: NCT03886935

Last Updated: 2021-02-24

Basic Information

• Recruitment Status: COMPLETED
• Total Enrollment: 100 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2017-01-01
• Study Completion Date: 2019-01-01

Brief Summary

Assessment of metabolic alterations in adult Fontan patients with a dominant left ventricle with the help of serum examinations (Metabolomics). The aim is to find a tool for the completion of the (semi-)invasive monitoring of Fontan hemodynamics.

Detailed Description

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.

Conditions

Congenital Heart Disease; Metabolism Disorder

Study Design

• Observational Model Type: CASE_CONTROL
• Study Time Perspective: PROSPECTIVE

Study Groups

Fontan patients

The serum of Fontan patients is compared to the serum of healthy biventricular controls (Metabolomics)

Metabolomics

Intervention Type: DIAGNOSTIC_TEST

Healthy biventricular controls

The serum of Fontan patients is compared to the serum of healthy biventricular controls (Metabolomics)

Metabolomics

Intervention Type: DIAGNOSTIC_TEST

Interventions

Metabolomics

Intervention Type: DIAGNOSTIC_TEST

Eligibility Criteria

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

• Eligible Sex: ALL
• Accepts Healthy Volunteers: Yes

Sponsors

Biocrates Life Sciences AG, Innsbruck, Austria

UNKNOWN

Sponsor Role: collaborator

Tiroler Wissenschaftsförderung

UNKNOWN

Sponsor Role: collaborator

Medical University Innsbruck

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Daniela Karall, Prof.

Role: PRINCIPAL_INVESTIGATOR

Medical University of Innsbruck

References

Michel M, Salvador C, Wiedemair V, Adam MG, Laser KT, Dubowy KO, Entenmann A, Karall D, Geiger R, Zlamy M, Scholl-Burgi S. Method comparison of HPLC-ninhydrin-photometry and UHPLC-PITC-tandem mass spectrometry for serum amino acid analyses in patients with complex congenital heart disease and controls. Metabolomics. 2020 Dec 15;16(12):128. doi: 10.1007/s11306-020-01741-8.

Reference Type: DERIVED

PMID: 33319318 (View on PubMed)

Michel M, Dubowy KO, Zlamy M, Karall D, Adam MG, Entenmann A, Keller MA, Koch J, Odri Komazec I, Geiger R, Salvador C, Niederwanger C, Muller U, Scholl-Burgi S, Laser KT. Targeted metabolomic analysis of serum phospholipid and acylcarnitine in the adult Fontan patient with a dominant left ventricle. Ther Adv Chronic Dis. 2020 Apr 27;11:2040622320916031. doi: 10.1177/2040622320916031. eCollection 2020.

Reference Type: DERIVED

PMID: 32426103 (View on PubMed)

Other Identifiers

201810011837

Identifier Type: -

Identifier Source: org_study_id