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Survival of Double Inlet Left Ventricle Patients Without Fontan Circulation (DILV-S)

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details.
 
ClinicalTrials.gov Identifier: NCT03667144
Recruitment Status : Unknown
Verified September 2018 by Sophie Meyer, University of Groningen.
Recruitment status was:  Not yet recruiting
First Posted : September 12, 2018
Last Update Posted : September 12, 2018
Sponsor:
Information provided by (Responsible Party):
Sophie Meyer, University of Groningen

Brief Summary:

Patients with univentricular hearts are currently palliated with the Fontan procedures. This results in an unphysiologic circulation with poor long-term survival. On the other hand there is a small, selected subgroup of patients with univentricular hearts of the double inlet left ventricle (DILV) type that survives up to old age without ever having undergone any Fontan procedures. Considering the relatively bleak data on long-term survival of DILV patients palliated with the Fontan procedure, it seems highly relevant to investigate and determine factors that allow a selection of unoperated patients to live to a comparatively high age without apparent major complaints. This might have an impact on how (and if, at all) certain univentricular patients are selected for operation in the future. For some, a different surgical procedure, or even therapeutic nihilism might be a more viable alternative.

The investigators hypothesise that favourable intracardiac streaming plays a role in making these patients viable: certain intracardiac anatomical characteristics allow for favourable flow patterns.

The primary objective of this study is to describe the intracardiac anatomy and hemodynamics of DILV patients without Fontan circulation using 4D MRI. Furthermore, the functional status and quality of life of these patients will be assessed.


Condition or disease
Double Inlet Left Ventricle

Detailed Description:

Rationale: Patients with univentricular hearts are currently palliated with a sequence of operations known as the Fontan procedure. This results in an un-physiologic circulation with poor long-term survival. On the other hand there is a small, selected subgroup of patients with univentricular hearts of the double inlet left ventricle (DILV) type that survives up to old age without ever having undergone any Fontan procedure. Considering the relatively bleak data on long-term survival of DILV patients palliated with the Fontan procedure, it is highly relevant to investigate and determine factors that allow for a selection of unoperated patients to live to a comparatively high age without apparent major complaints. This might have an impact on how (and if, at all) certain univentricular patients will be selected for operation in the future. For some, a different surgical procedure, or even therapeutic nihilism might be a more viable alternative. The investigators hypothesize that favourable intracardiac streaming plays a role in making the unpalliated patients viable: certain intracardiac anatomical characteristics allow for favourable flow patterns, resulting in higher systemic oxygen saturations than expected by mixing pulmonary and systemic venous returns.

Objective: The primary objective of this study is to describe the intracardiac anatomy and hemodynamics of DILV patients without Fontan circulation using 4D MRI. Furthermore, the functional status and quality of life of these patients will be assessed and compared to a matched cohort of Fontan patients.

Study design: This will be an observational, cross-sectional study. The investigators aim to include all DILV patients without Fontan circulation known at the congenital heart disease referral centers in the Netherlands and Flanders. The Dutch patients will undergo one 4D MRI, a physical examination, cardiopulmonary exercise testing and have a blood sample taken at the University Medical Center Utrecht. The patients from the Flemish referral centers will undergo the same protocol at the University Medical Center Leuven. Furthermore, patients over the age of 18 will be asked to fill out a quality of life questionnaire.

Study population: Included will be patients over the age of 12 with the diagnosis double inlet left ventricle, which have not undergone the Fontan trajectory. For the 4D MRI, patients with an MRI non-compatible pacemaker in situ will be excluded.

Main study parameters/endpoints: This is primarily an explorative study aiming to describe the anatomy of unpalliated DILV hearts of patients without Fontan palliation. With 4D MRI, the hearts will be grouped according to the angular relationship of the two lines connecting the atrioventricular and the ventriculo-arterial valves. This angle can be divided into three categories: 1) parallel (i.e. 0°), 2) perpendicular (i.e. 90°) or 3) an in-between variant. Accordingly, flow patterns, ranging from linear to turbulent will be measured and correlated to the anatomical variants.

Nature and extent of the burden and risks associated with participation, benefit and group relatedness: The burden and risks associated with participation in this study are minimal. Patients need to visit the site of investigation only once, and the tests done at the visit (i.e. 4D MR imaging, exercise and pulmonary function testing and blood sample) are part of standard medical care and as such pose negligible risk on the patients. The quality of life survey is not part of standard medical care and might be confrontational on a psychological level. It is, however, a widely used and scientifically approved survey, which aims to keep this burden very low. Due to the extremely low prevalence of the condition to be studied, it is of great importance to include every single patient, including children under the age of 18. Excluding children from this study would further decrease the sample size to a significantly smaller number, making it difficult to draw conclusions.

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Study Type : Observational
Estimated Enrollment : 15 participants
Observational Model: Cohort
Time Perspective: Cross-Sectional
Official Title: Survival of Double Inlet Left Ventricle Patients Without Fontan Circulation: Intracardiac Anatomy and Hemodynamics, Functional Status and Quality of Life
Estimated Study Start Date : December 3, 2018
Estimated Primary Completion Date : June 1, 2019
Estimated Study Completion Date : June 1, 2019

Resource links provided by the National Library of Medicine

MedlinePlus related topics: Anatomy




Primary Outcome Measures :
  1. Angle between atrioventricular and ventriculoarterial valves [ Time Frame: 1 day ]
    This is primarily an explorative study aiming to describe the anatomy of unpalliated DILV hearts. With 4D MRI, the hearts will be grouped according to the angular relationship of the atrioventricular to the ventriculo-arterial valves. This angle can be divided into three categories: 1) parallel (i.e. 0°), 2) perpendicular (i.e. 90°) or 3) an in-between variant. Accordingly, flow patterns, ranging from linear to turbulent will be measured and correlated to the anatomical variants.


Secondary Outcome Measures :
  1. New York Heart Association Functional Classification [ Time Frame: 1 day ]
    NYHA functional class I to IV

  2. Peak VO2 [ Time Frame: 1 day ]
    peak oxygen uptake (VO2index) during exercise as well as peak VO2 as percentage of predicted (VO2 pred)

  3. Peripheral oxygen saturation [ Time Frame: 1 day ]
    Oxygen saturation at rest, measured with oximetry

  4. Scores for Quality of life Adults [ Time Frame: 1 day ]

    Quality of life survey overall scores, and scores in the subcategories measured with the Short Form (SF)36 (For patients 18 and older). The SF-36 consists of eight scaled scores, which are the weighted sums of the questions in their section. Each scale is directly transformed into a 0-100 scale on the assumption that each question carries equal weight. The lower the score the more disability. The higher the score the less disability i.e., a score of zero is equivalent to maximum disability and a score of 100 is equivalent to no disability. The eight sections are:

    vitality physical functioning bodily pain general health perceptions physical role functioning emotional role functioning social role functioning mental health


  5. Scores for Quality of life [ Time Frame: 1 day ]
    Quality of life survey overall scores, and scores in the subcategories measured with PedsQL (for patients younger than 18). The 23-item PedsQL Generic Core Scales were designed to measure the core dimensions of health as delineated by the World Health Organization, as well as role (school) functioning. The 4 Multidimensional Scales are: Physical Functioning (8 items) Emotional Functioning (5 items), Social Functioning (5 items), School Functioning (5 items) The summary scores are: Total Scale Score (23 items), Physical Health Summary Score (8 items), Psychosocial Health Summary Score (15 items)

  6. Hematocrit [ Time Frame: 1 day ]
    from venous blood

  7. Haemoglobin [ Time Frame: 1 day ]
    from venous blood

  8. Creatinin [ Time Frame: 1 day ]
    from venous blood

  9. eGFR [ Time Frame: 1 day ]
    from venous blood

  10. high sensitive Troponin T [ Time Frame: 1 day ]
    from venous blood

  11. NT-proBNP [ Time Frame: 1 day ]
    from venous blood

  12. LDH [ Time Frame: 1 day ]
    from venous blood

  13. ASAT [ Time Frame: 1 day ]
    from venous blood

  14. ALAT [ Time Frame: 1 day ]
    from venous blood

  15. Alkaline phosphatase [ Time Frame: 1 day ]
    from venous blood

  16. Gamma-GT [ Time Frame: 1 day ]
    from venous blood

  17. Galectin 3 [ Time Frame: 1 day ]
    from venous blood

  18. Bilirubin [ Time Frame: 1 day ]
    from venous blood


Other Outcome Measures:
  1. Demographics [ Time Frame: 1 day ]
    sex, age, weight, height

  2. Highest Level of Education [ Time Frame: 1 day ]
    Highest level of education as well as their current Profession and their current work Status will be asked of all participants on the Basis of a questionnaire

  3. Current Medication use [ Time Frame: 1 day ]
    Participants will be asked to provide the names and the dosage of all medications they are currently using .


Biospecimen Retention:   Samples With DNA
Venous blood samples for biomarker analysis


Information from the National Library of Medicine

Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the contacts provided below. For general information, Learn About Clinical Studies.


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Ages Eligible for Study:   12 Years and older   (Child, Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population
Included will be patients over the age of 12 with the diagnosis double inlet left ventricle, which have not undergone the Fontan trajectory. For the 4D MRI, patients with an MRI non-compatible pacemaker in situ will be excluded.
Criteria

Inclusion Criteria:

  • Diagnosis double inlet left ventricle, established by echocardiograph or MRI
  • Not undergone Fontan trajectory in the past
  • Be of the age of 12 or older
  • Written informed consent

Exclusion Criteria:

• previous (partial) cavopulmonary connection

The following subjects will be excluded from the MRI part of the study, but will still be asked to participate in all the other parts:

• MRI non-compatible pacemaker in situ


Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT03667144


Contacts
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Contact: Sophie L Meyer, BSc 004917681033255 s.l.meyer@umcg.nl

Sponsors and Collaborators
University of Groningen
Investigators
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Principal Investigator: Tjark Ebels, MD PhD University Medical Center Groningen
Publications:

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Responsible Party: Sophie Meyer, Prof. dr. Tjark Ebels, University of Groningen
ClinicalTrials.gov Identifier: NCT03667144    
Other Study ID Numbers: NL59516.042.17
First Posted: September 12, 2018    Key Record Dates
Last Update Posted: September 12, 2018
Last Verified: September 2018
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: No

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Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No
Keywords provided by Sophie Meyer, University of Groningen:
Double inlet left ventricle without Fontan circulation