Clinical trial NCT04163653: Lymphatic Morphology of Fontan Patients
Official title: Lymphatic Morphology and Classification in Patients With a Fontan Circulation - A Multicenter Study
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Lymphatic Abnormality
Plastic Bronchitis
Protein-Losing Enteropathies
Univentricular Heart
Congenital Heart Disease
Lead sponsor: University of Aarhus
Sponsor class: OTHER
Collaborators: name: InCor Heart Institute
name: Hospital das Clínicas de Ribeirão Preto
First posted: 2019-11-15
Enrollment: 41
Sex: ALL
Minimum age (years): 18
Maximum age (years): 100
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: yes
Observational model: CASE_CONTROL
Time perspective: CROSS_SECTIONAL
Sampling method: PROBABILITY_SAMPLE
Study population: Patients with a Fontan circulation.

Brief summary: The Fontan procedure has revolutionized the treatment of patients born with a congenital univentricular heart defect. However, over time, it is associated with severe lymphatic complications such as plastic bronchitis, protein-losing enteropathy (PLE) and peripheral edema.

The hypothesis is that patients with a univentricular circulation have a changed morphology which may be associated with both the degree of lymphatic complications and their physical capacity.

The morphology will be described using T2-weighted non-contrast MRI.

Detailed description: The Fontan procedure has revolutionized the treatment of patients born with a congenital univentricular heart defect. However, over time, it is associated with severe lymphatic complications such as plastic bronchitis, protein-losing enteropathy (PLE) and peripheral edema. It has been proposed that these complications arise due to a elevated central venous pressure of the changed circulation.

The hypothesis of the study is that patients with a univentricular circulation have a changed morphology which may be associated with both the degree of lymphatic complications and their physical capacity.

The morphology will be described using T2-weighted non-contrast MRI. The main lymphatic vessel, the thoracic duct, will be rendered and measured using 3D software, and the lymphatic changes characterized by Dr. Yoav Dori (CHOP, Pennsylvania, USA), using a newly published grading system. The above will be correlated with the amount of complications experienced by the patients, and their physical capacity measured by both IPAQ questionaire and CPX test.

The study population consists of patients with a Fontan circulation operated at InCor Heart Institute, São Paulo, Brasil and Hospital das Clínicas, Ribeirão Preto, Brasil. Exclusion criteria were mental illness, genetic syndromes and age \<18 years. The patient group will be compared with age, gender and weight matched healthy controls.

Eligibility criteria: Inclusion Criteria:

- Fontan Circulation

Exclusion Criteria:

* Age \<18 years
* Mental illness
* Genetic syndromes

Primary outcomes:
measure: Lymphatic classification
timeFrame: 1 hour
measure: Relative measure of tortuosity
timeFrame: 1 hour
measure: Measurement of thoracic duct volume
timeFrame: 1 hour
measure: Measurement of thoracic duct diameter
timeFrame: 1 hour
measure: The International Physical Activity Questionnaire (IPAQ-SF)
timeFrame: 1 hour
measure: CPX heart rate
timeFrame: 1 hour
measure: CPX Vo2
timeFrame: 1 hour
Secondary outcomes:
Other outcomes:
Locations:
city: Aarhus N
country: Denmark
facility: Department of Cardiothoracic and Vascular Surgery
Study officials:
References: