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The Fontan Procedure is a palliative surgical procedure used in pediatric patients with one functional ventricle. The procedure, a series of stepwise operations that alter cardiorespiratory physiology, separate the systemic and pulmonary circulations to create Fontan physiology, where the systemic venous blood flows passively and without ventricular thrust into the pulmonary circulation. The hallmark of the Fontan circulation is a sustained, abnormally elevated central venous pressure combined with decreased cardiac output, especially during periods of increased demands. Results of several studies in Fontan patients have shown reduced parasympathetic and sympathetic activity compared to controls. In children with congenital heart disease, a differential diagnosis of autonomic dysfunction may be part of their pathophysiology, a compensatory mechanism, a consequence of surgical procedures or a combination of these.
In children, measurement of ANS function is equally important. Children with single ventricle physiology (and other cardiac conditions) have routine surveillance and cardiac magnetic resonance (CMR) imaging to monitor for disease progression. While autonomic data is routinely collected and is available from these scans, these data are rarely, collected and analyzed; however, our group has shown feasibility. Therefore, autonomic data is usually unavailable in children. Despite the availability of agerelated normal values, the predictive power of autonomic activity is understudied in children and there are no published studies of quantification of autonomic data in this population.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Fontan | Patients undergoing cardiac MRI for Fontan, |
| |
| Healthy controls | Patients undergoing thoracic MRI for chest wall deformity. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| VU-AMS device | Other | Patients will wear the VU-AMS monitor prior to MRI. |
|
| Measure | Description | Time Frame |
|---|---|---|
| 80% availability of respiratory sinus arrhythmia from cardiac MRI (magnetic resonance imaging) | Data collected during MRI | Through MRI completion up to one hour |
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| Measure | Description | Time Frame |
|---|---|---|
| Describe difference in respiratory sinus arrhythmia in participants with Fontan | Collection of data from the cardiac MRI | Through MRI completion up to one hour |
| Describe difference in respiratory sinus arrhythmia in participants with pectus |
Inclusion Criteria:
Exclusion Criteria:
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Patients presenting for clinically indicated CMR scans with planned cine sequence and use of a respiratory bellows.
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| Name | Affiliation | Role |
|---|---|---|
| Pornswan Ngamprasertwong, MD | Children's Hospital Medical Center, Cincinnati | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Cincinnati Children's Hospital Medical Center | Cincinnati | Ohio | 45229 | United States |
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| ID | Term |
|---|---|
| D005660 | Funnel Chest |
| D006330 | Heart Defects, Congenital |
| ID | Term |
|---|---|
| D001848 | Bone Diseases, Developmental |
| D001847 | Bone Diseases |
| D009140 | Musculoskeletal Diseases |
| D009139 | Musculoskeletal Abnormalities |
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Collection of data from the cardiac MRI
| Through MRI completion up to one hour |
| Compare estimate of pre-ejection period (PEP) | Collection of data from VU-AMS monitor | Prior to MRI scan up to 10 minutes |
| Compare estimate of pre-ejection period (PEP) | Collection of data from the cardiac MRI | Through MRI completion up to one hour |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D018376 | Cardiovascular Abnormalities |
| D002318 | Cardiovascular Diseases |
| D006331 | Heart Diseases |