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This study is designed to learn more about children who have blockage of the left side of their heart. The goal is to determine how much the heart muscle has thickened before surgery and how it changes in the months after surgery. Investigators are also looking for blood tests that may help them predict which patients will have the most thickening pre-operatively and the best return towards normal after surgery. The findings of this study will help the investigators develop new tests to monitor affected patients and develop new therapies to help minimize heart thickening.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Persistent hypertrophy | Elevated cardiac mass at follow-up time point as measured by Echocardiographic and Serologic evaluations. |
| |
| Normal geometry | Normal cardiac mass at follow-up time point as measured by Echocardiographic and Serologic evaluations. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Echocardiogram | Procedure | 2 and 3-dimensional echocardiographic images will be obtained to estimate LV mass, ejection fraction (Simpson's biplane), fractional shortening (M mode Doppler), and pulse-wave estimated pressure gradient across the outflow obstruction. |
| Measure | Description | Time Frame |
|---|---|---|
| Left ventricular mass | Left ventricular mass will be measured by 2D echocardiography at 1 year after surgical correction. | 1 year after surgery |
| Change in left ventricular mass | Left ventricular mass will be measured by 2D echocardiography prior to surgery, immediately after surgery, and will be compared to that measured at 1 year after surgical correction. | Within 1 week prior to surgery, 24-72 hrs after surgery, 1 year after surgery |
| Measure | Description | Time Frame |
|---|---|---|
| Relative wall thickness | A more detailed measure of relative wall thickness (RWT) will be assessed by echocardiography at 1 year after surgical correction. | 1 year after surgery |
| Left ventricular mass |
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Inclusion Criteria:
Exclusion Criteria:
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Potential subjects will be identified from the cardiac surgical and clinic schedules at Children's Hospital Colorado (CHCO).
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| Name | Affiliation | Role |
|---|---|---|
| Benjamin S Frank, MD | University of Colorado, Denver | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Children's Hospital Colorado | Denver | Colorado | 80045 | United States |
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| ID | Term |
|---|---|
| D001017 | Aortic Coarctation |
| D001024 | Aortic Valve Stenosis |
| D006332 | Cardiomegaly |
| ID | Term |
|---|---|
| D006330 | Heart Defects, Congenital |
| D018376 | Cardiovascular Abnormalities |
| D002318 | Cardiovascular Diseases |
| D006331 | Heart Diseases |
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Plasma and tissue samples from patients will be stored for the duration of the study. Patients may choose to consent to have these samples stores beyond the completion of primary analysis for testing in future studies.
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| Serology | Biological | protein analysis targeting several potential protein biomarkers for cardiac remodeling and hypertrophy as well as targeting known miRNAs. |
|
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Left ventricular mass will be measured by 3D echocardiography at 1 year after surgical correction.
| 1 year after surgery |
| Change in relative wall thickness | Relative wall thickness will be measured by 2D echocardiography prior to surgery and at 1 year after surgical correction. | Within 1 week prior to surgery and 1 year after surgery |
| Change in relative wall thickness | Relative wall thickness will be measured by 2D echocardiography 24-72 hours and at 1 year after surgical correction. | 24-72 hours and 1 year after surgery |
| Change in left ventricular mass | Change in left ventricular mass will be measured by 3D echocardiography 24-72 hours and 1 year after surgical correction. | 24-72 hours and 1 year after surgery |
| Change in left ventricular mass | Change in left ventricular mass will be measured by 3D echocardiography prior to surgery and at 1 year after surgical correction. | Within 1 week prior to surgery and 1 year after surgery |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D000082862 | Aortic Valve Disease |
| D006349 | Heart Valve Diseases |
| D014694 | Ventricular Outflow Obstruction |
| D006984 | Hypertrophy |
| D020763 | Pathological Conditions, Anatomical |
| D013568 | Pathological Conditions, Signs and Symptoms |