Single Ventricle Defect
Conditions
Keywords
Single ventricle, Hypoplastic Left Heart Syndrome, Congenital heart lesion, Right or left ventricle, Cerebral blood flow, Pulmonary blood flow, Staged Fontan reconstruction, hemi Fontan/bidirectional Glenn stages
Brief summary
This study seeks to determine cerebral blood flow changes in single ventricle patients during staged surgical reconstruction as compared with normal children. Two general groups of single ventricle patients will be recruited for this study, corresponding to the two approaches used. An aged-match group of healthy subjects will be included as a control.
Detailed description
This is a prospective, non-randomized, single center study designed to measure and determine the changes in cerebral blood flow in a cohort of single ventricle patients followed across all three stages of surgical (Fontan) reconstruction. In addition, cerebral blood flow response to hypercarbia will be determined after the first 2 operations as a measure of cerebral blood flow reserve and the intactness of the cerebral autoregulatory system. MRI data from this cohort will be compared with that collected in age-matched healthy children and in a cross sectional group of patients who will not necessarily be followed through all stages of surgery, but may undergo one or two of the MRI evaluations depending on their stage of surgery. Neurological exams and EEGs will be performed. Medical history, cardiac catheterization data, where available, will also be collected. Factors such as cardiopulmonary bypass time and socioeconomic class will be controlled for.
Interventions
None listed
Sponsors
National Institutes of Health (NIH)
National Heart, Lung, and Blood Institute (NHLBI)
Children's Hospital of Philadelphia
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
4 Months to 10 Years
Healthy volunteers
No
Inclusion criteria
- Single Ventricle Subjects: * Males or females up to age 10 years. * Any complex congenital heart lesion that has single ventricle physiology. These single ventricle lesions will be of either right or left ventricular morphology. * Ability to undergo an MRI scan of at least 60-90 minutes under general anesthesia or deep sedation. Patients will undergo determination of their physical ability to undergo the MRI prior to being enrolled in the study by parameters such as blood pressure, heart rate, cardiac rhythm, and respiratory rate. MRI involves laying still in the supine position for at least 60-90 minutes. * Parents signing informed consent. Inclusion Criteria - Normal Control Group: * Males or females up to age 10 years. * Normal cerebral anatomy who are normocephalic and who are asymptomatic (e.g. if the scan is performed for headache) at the time of the scan. * Ability to undergo an MRI scan of at least 60-90 minutes under general anesthesia or deep sedation. Normal patients will undergo determination of their physical ability to undergo the MRI prior to being enrolled in the study by parameters such as blood pressure, heart rate, cardiac rhythm, and respiratory rate. MRI involves laying still in the supine position for at least 60-90 minutes. * Parents signing informed consent.
Exclusion criteria
- Single Ventricle Patients : * Any condition judged by the patient's physician that would cause this trial to be detrimental to the patient. * Stenosis of the branch pulmonary arteries or coarctation of the aorta. * Any significant arrhythmias such as supraventricular tachycardia, trigeminy, etc. Occasional premature atrial or ventricular contractions or an occasional missed beat is permitted. * Systemic hypertension. * Primary lung disease (eg bronchopulmonary dysplasia). * Any known significant neurological disease outside of the usual state of single ventricle patients. * Any chromosomal anomalies or other major anomalies which would confound neurological outcome. * A patient with a pacemaker or cardioverter/defibrillator in place. * A ferromagnetic foreign body (with the exception of sternal wires and vascular clips in the thorax). * Parents/guardians or subjects who, in the opinion of the Investigator, may be non-compliant with study schedules or procedures.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Cerebral blood flow changes throughout staged surgical reconstruction in single ventricle patients. | Up to 2 years | Cerebral blood flow & systemic blood flow will be measured via MRI in addition to cerebral anatomy and volume at all 3 stages. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| CBF in single ventricle patients compared to CBF of age-matched normal children. | Up to 2 years | — |
| CBF reserve and intactness of the cerebral regulatory mechanisms after Stage I and hemi Fontan/bidirectional Glenn stage of surgeries. | Up to 2 years | Determine CBF reserve and intactness of the cerebral regulatory mechanisms in patients after Stage I and hemi Fontan/bidirectional Glenn stage of surgeries by obtaining CBF under normal conditions and under conditions of hypercarbia prior to surgery. |
| CBF reserve after Stage 1 compared with CBF reserve in hemi Fontan/bidirectional Glenn stage of surgeries. | Up to 2 years | — |
Other
| Measure | Time frame | Description |
|---|---|---|
| Age related CBF changes between 4-6 months to 3-10 years. | Up to 2 hours | Determine age related CBF changes in a group of normal children from ages 4-6 months to 3-10 years undergoing clinical brain MRIs for other reasons and found not to have pathology. |
Countries
United States
Outcome results
None listed