To visualize and quantify areas of dysfunctional flow in the TCPC and within the single ventricle in patients after a Fontan procedure and try to determine a relationship between these quantitative energetic parameters and adverse outcome.
ID
Source
Brief title
Condition
- Congenital cardiac disorders
- Cardiac and vascular disorders congenital
- Cardiac therapeutic procedures
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
Echocardiographic parameters:
Cardiac index
MRI parameters:
Cardiac and Fontan tunnel:
Flow patterns and Energetics (energy loss)
Liver: Liver Multi-scan (fat, iron, fibrosis level)
Exercise test parameters:
peak oxygen uptake (VO2 max)
Stool parameter:
alpha-1 antitrypsin
Clinical parameters:
NYHA class
Episodes of PLE in history
Holter:
Arrythmias
Secondary outcome
Echocardiographic parameters:
ejection fraction, grading of AVV regurgitation, E/A ratio, propagation velocity
MRI parameters:
Cardiac:
End diastolic volume (EDV), End systolic volume (ESV), single ventricle
ejection fraction (EF), quantification of AVV regurgitation
Cardiac and Fontan tunnel:
Energetics (kinetic energy, turbulent kinetic energy, vorticity, wall shear
stress)
Exercise test parameters:
Peak heart rate, percentage of expected Watts.
Blood parameters:
complete blood count
liver function
kidney function
albumin and total protein
NT-proBNP, hs-TnT, GDF-15
Urine parameter:
Protein
Clinical parameters:
Time from Fontan operation
Background summary
Patients born with a functional univentricular heart are palliated via a staged
approach leading to a Fontan circulation. The Fontan circulation provides a
palliative solution by connecting the vena cavae directly to the pulmonary
circulation without interposition of the heart, the so called total
cavopulmonary connection. It is thought that an efficient flow of blood results
in better outcome, as there is no heart which actively pumps blood into the
pulmonary circulation.
Although early mortality has improved, the risk of complications such as
protein losing enteropathy, plastic bronchitis, liver fibrosis and cirrhosis
and reduced exercise capacity remains high.
Reduced efficiency of blood flow in Fontan patients have been an area of great
interest as it is assumed to be important as there is no heart actively pumping
the blood into the pulmonary circulation.
Study objective
To visualize and quantify areas of dysfunctional flow in the TCPC and within
the single ventricle in patients after a Fontan procedure and try to determine
a relationship between these quantitative energetic parameters and adverse
outcome.
Study design
prospective cohort study
Study burden and risks
The possible complications after the Fontan procedure, which is nowadays
performed on an age of 3-5 years, can occur early till late after the
operation. It is therefore essential that patients with different timeframes
after correction are included in this study. If only adults were included in
this study, the younger patients will probably not benefit from the findings of
this study. Additionally, the Fontan operation has changed over time, with the
modern ECC procedure being the preferred approach nowadays and has been
implemented since 2000 in our hospital. This means that most of these patients
are <20 years old and makes selection of only adults unfeasible.
There is no additional risk for patients in this study. All examinations are
part of standard routine clinical practice in the follow-up of patients with a
Fontan circulation.
In the future patients can benefit from entry into this study, as results from
this study can possibly identify which patients are at increased risk of
complications and lead to potential better treatment possibilities in the
future.
Albinusdreef 2
Leiden 2333ZA
NL
Albinusdreef 2
Leiden 2333ZA
NL
Listed location countries
Age
Inclusion criteria
Patients with an intact Fontan-TCPC circulation performed at the LUMC
>8years of age
No contra-indications for exercise test
No contra-indications for MRI examination
Exclusion criteria
Claustrophobia
Contraindication for MRI
<8 years old
Pacemaker in situ
Design
Recruitment
metc-ldd@lumc.nl
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL63854.058.17 |