Multimodality biomarkers for noninvasive assessment of the Fontan patient
Study ID: STU 122016-037
We plan to conduct a single center prospective longitudinal study and enroll approximately 50 Fontan patients and 20 controls of a similar age, gender, BMi and physical activity level between the ages of 10-40 years. There will be an interim review after studying 5 subjects under the following protocols to ensure feasibility of the project. This review will be done with Dr Maria Bano (Pi), Dr Richard Kirk, Dr Tarique Hussain. We will enroll from pediatric cardiology and heart failure clinics and inpatient care units at Children's Health Dallas, adult congenital heart disease clinics and inpatient care units at Clements university Hospital and the Clinical Heart Center. This study will evaluate the multimodality biomarkers for assessment of Fontan circulation including heart, liver and lung and change in these markers with exercise training. The Pi will evaluate the inclusion and exclusion criteria for each patient and this information will be recorded in a screening log. if the patient is a potential participant, the entire study protocol will first be discussed with the attending Cardiologist. Then the patient /legal guardian will be approached by the attending Cardiologist and if interested, the Pi or a co-investigator will contact the potential participant. Patient will have blood drawn for assessment of renal function (see exclusion criteria for details) and ruling out pregnancy for all female participants of child bearing age prior to enrollment. it will be stressed that the decision to participate or not participate in the study will not affect the patient's care and the patient or caregiver may withdraw from the study at any time without consequences. if they agree to participate, they will be given the written consent form and this will be reviewed with them. if they agree to the study and provide written consent, they will be enrolled. once informed consent is available we plan on scheduling a 2 day long research visit (Visit 1) within 14 days of the screening visit. . The first day of testing will occur at Children's Health, Dallas and the second day of testing will be completed at institute for exercise and environmental Medicine, Dallas. Patient will go home after the first day of testing and return for the 2nd day of testing. on the first day of the research visit the patient will start the day with a magnetic resonance imaging of the Fontan circulation. This will include imaging of the heart, lung and liver as detailed below. This study will be conducted according to standard clinical protocol used at Children's Health Dallas for imaging patients with congenital heart disease and some additional research sequences detailed below. We plan to include specific imaging for tissue characterization and assessment of myocardial fibrosis, liver fibrosis and disproportionate pulmonary blood flow. These additional imaging sequences will follow FDa guidelines for novel imaging techniques in congenital heart disease. We will then draw blood (approximately 10 ml) for assessment of serum biomarkers and circulating microRnas of interest described later. This blood will be stored in the bio-specimen repository at Children's Health lab and batch tested per protocol below. The patient will go home and return for day 2 of exercise testing to ieeM. They will return 1 month from Visit 1 to repeat exercise testing at ieeM and establish reproducibility of the testing results. These patients will then start a 3-6 month long cardiac rehabilitation program detailed below. Frequent phone follow up (conducted by the research staff) will ensure adherence. after the 3-6 month study period we will bring the patients back for a follow up 2 day research visit (Visit 3) and repeat all the testing completed at Visit 1.
* Presence of a congenital heart defect that leads to Fontan circulation * Previously performed Fontan surgery * Current age 10 years to 40 years * Informed consent
- Cancer Related
- Healthy Volunteers
- UT Southwestern Principal Investigator
- Maria Bano