Not provided
Not provided
Not provided
Not provided
| ID | Type | Description | Link |
|---|---|---|---|
| U10HL068270 | U.S. NIH Grant/Contract | View source |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Class |
|---|---|
| National Heart, Lung, and Blood Institute (NHLBI) | NIH |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
The purpose of this study is to compare direct and indirect measures of right ventricular (RV) systolic and diastolic function between 11 year old subjects who had been randomly assigned to receive a right ventricle to pulmonary artery shunt (RVPAS) vs. a modified Blalock-Taussig shunt (MBTS) at the time of the Norwood operation.
The Single Ventricle Reconstruction (SVR) trial was the first multicenter, randomized clinical trial to compare two operations in the field of congenital heart disease.8,11 Children with HLHS and other related single RV lesions were enrolled and randomized to receive either a MBTS or a RVPAS at the time of the initial Norwood procedure. This landmark study provided extraordinary insight not only into the consequences of both shunt types, but also into the course, treatment responses and short- and mid-term outcomes for these medically complex patients. Through the SVR Trial and SVR Extension Study (SVR II), outcomes, including but not limited to the primary outcome of transplant-free survival, have now been evaluated in this patient cohort when the last enrolled patient reached 12 months and again at 3 years of age. While early post-operative transplant-free survival during the interstage period7 and at one year8 was better for those children randomized to a RVPAS, survival by the 3-year evaluation appeared equivalent between the two shunt types. Moreover, RVEF was somewhat diminished and the number of interventions was higher in the RVPAS group.12 These findings raised concern that the RV dysfunction in the RVPAS group may be progressive, leading to significantly worse long-term outcomes; if so, the benefits of the RVPAS for short-term survival may be outweighed by longer-term morbidity and mortality. Thus, the optimal surgical approach for newborns with HLHS and related single RV lesions remains unclear.
The Pediatric Heart Network (PHN) Investigators have a unique opportunity and responsibility to analyze the effect of the type of systemic-to-pulmonary artery shunt placed during the Norwood procedure on longer-term survival, as well as to define its effect on other long-term outcomes in this multi-institutional cohort of exquisitely characterized subjects with single RV lesions. As subjects enrolled in the SVR cohort approach a decade of age, the investigators aim: 1) to determine if shunt type at the time of Norwood operation is associated with any long-term differences in cardiac function, survival, or contributors to quality of life; and 2) to characterize long-term outcomes and determine risk factors other than shunt type for adverse long-term outcomes in children with HLHS and other related single ventricle anomalies.
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Transplant-free survivors | Transplant-free survivors of the SVR cohort (All SVR survivors are eligible to be followed for vital status.) |
Not provided
| Measure | Description | Time Frame |
|---|---|---|
| RVEF at 10-12 Years on MRI | RV ejection fraction (RVEF) at 10-12 years, as measured by cardiac magnetic resonance (CMR). | assessed between 10-12 years of age, an average of 53.2 days from consent |
| Measure | Description | Time Frame |
|---|---|---|
| Death or Cardiac Transplantation | The incidence of death or cardiac transplantation between those randomized to receive a RVPAS vs. a MBTS at the time of the Norwood operation. | 11 years ± 1 year to 16 years |
| The Exercise Tolerance Between Those Randomized to a RVPAS vs. a MBTS. |
Not provided
Inclusion Criteria:
Exclusion Criteria:
Not provided
Not provided
All SVR study cohort members will be contacted to assess for vital status. Transplant free survivors will be approached to participate in the in-person assessment.
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 40671650 | Derived | Miller TA, Sharma B, Gongwer R, Trachtenberg FL, Newburger JW, Goldberg CS, Gustafson KE, Gaynor JW, Votava-Smith JK, Lambert LM, Sananes R, Kral MC, Tsang R, Heinrich KP, Cnota J, Shah A, Ilardi D; Pediatric Heart Network Investigators. Neurodevelopmental Outcomes in Early Adolescence: The Pediatric Heart Network's Single Ventricle Reconstruction Trial. Circulation. 2025 Oct 28;152(17):1246-1261. doi: 10.1161/CIRCULATIONAHA.125.074523. Epub 2025 Jul 17. |
Not provided
Not provided
Not provided
Not provided
Not provided
| ID | Title | Description |
|---|---|---|
| FG000 | mBTTS | Blalock Taussig Thomas shunt at Norwood |
| FG001 | RVPAS | right-ventricular-to-pulmonary-artery shunt at Norwood |
| Title | Milestones | Reasons Not Completed | ||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Overall Study |
|
Not provided
Not provided
| ID | Title | Description |
|---|---|---|
| BG000 | mBTTS | Blalock Taussig Thomas shunt at Norwood |
| BG001 | RVPAS | right-ventricular-to-pulmonary-artery shunt at Norwood |
| Units | Counts |
|---|---|
| Participants |
|
| Title | Description | Population Description | Parameter Type | Dispersion Type | Unit of Measure | Calculate Percentage | Denominator Units Selected | Denominators | Classes |
|---|---|---|---|---|---|---|---|---|---|
| Age, Categorical | Count of Participants |
| Type | Title | Description | Population Description | Reporting Status | Anticipated Posting Date | Parameter Type | Dispersion Type | Unit of Measure | Calculate Percentage | Time Frame | Units Analyzed | Denominator Units Selected | Arm/Group Information | Denominators | Classes | Analyses | |||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Primary | RVEF at 10-12 Years on MRI | RV ejection fraction (RVEF) at 10-12 years, as measured by cardiac magnetic resonance (CMR). | participants who underwent cardiac MRI | Posted | Mean | Standard Deviation | percentage of blood pumped | assessed between 10-12 years of age, an average of 53.2 days from consent |
|
through study completion, an average of 5.3 years from consent
through study completion
Not provided
| ID | Title | Description | Deaths (Affected) | Deaths (At Risk) | Serious Events (Affected) | Serious Events (At Risk) | Other Events (Affected) | Other Events (At Risk) |
|---|---|---|---|---|---|---|---|---|
| EG000 | mBTTS | Blalock Taussig Thomas shunt at Norwood | 3 |
Not provided
Not provided
We cannot exclude enrollment bias or survival bias.
| Title | Organization | Phone | Extension | |
|---|---|---|---|---|
| Dr. Felicia Trachtenberg | Carelon Research | Felicia.Trachtenberg@carelon.c | Felicia.Trachtenberg@carelon.com |
Not provided
| Type | Includes Protocol | Includes SAP | Includes ICF | Document Label | Document Date | Document Uploaded Date | Document File Name |
|---|---|---|---|---|---|---|---|
| Prot_SAP | Yes | Yes | No | Study Protocol and Statistical Analysis Plan | May 28, 2024 | Feb 25, 2025 | Prot_SAP_000.pdf |
| ICF | No | No | Yes | Informed Consent Form | May 28, 2024 | Feb 25, 2025 | ICF_001.pdf |
Not provided
Not provided
Not provided
Not provided
Participants and their parents who have not yet contributed to the SVR Extension biorepository will be offered the opportunity to provide saliva for extraction and storage of DNA. If study participants have consented to participate in the biorepository through the SVR Extension study but not yet contributed a sample, they will be asked to contribute at the time of the SVR III participation. Families who were not previously approached, or who declined participation in the biorepository earlier, may be offered the opportunity to participate at the time of the SVR III participation.
Exercise capacity: Maximal Oxygen Consumption (VO2max), maximal work rate, and anaerobic threshold |
| 11 years ± 1 year to 16 years |
| Atrial and Ventricular Arrhythmias | The incidence of arrhythmias between those randomized to a RVPAS vs. a MBTS. | 11 years ± 1 year to 16 years |
| The Neurodevelopmental Outcomes at 11 Years of Age in Those Randomized to a RVPAS vs. a MBTS | Achievement, as measured by the Wechsler Individual Achievement Tests (WIAT) (Math and Reading); Intelligence, as measured by the Wechsler Intelligence Scale for Children (WISC); Other domains of neurodevelopmental function including assessment of language, executive function, visual spatial skills, motor function, memory, social skills, behavior, health-related quality of life (HRQoL) and adaptive function. | 11 years ± 1 year |
| Risk Stratification Models | Develop risk stratification models for 1) cardiac outcomes, 2) transplant-free survival, and 3) neurodevelopmental outcomes. | 11 years ± 1 year to 16 years |
| Biospecimens | Collect specimens from subjects and their parents to further develop the biologic specimen repository. | 11 years ± 1 year to 16 years |
| BG002 | Total | Total of all reporting groups |
| Participants |
|
| Sex: Female, Male | Count of Participants | Participants |
|
| Ethnicity (NIH/OMB) | Count of Participants | Participants |
|
| Race/Ethnicity, Customized | Count of Participants | Participants |
|
| Units | Counts |
|---|
| Participants |
|
|
| Secondary | Death or Cardiac Transplantation | The incidence of death or cardiac transplantation between those randomized to receive a RVPAS vs. a MBTS at the time of the Norwood operation. | Not Posted | 11 years ± 1 year to 16 years | Participants |
| Secondary | The Exercise Tolerance Between Those Randomized to a RVPAS vs. a MBTS. | Exercise capacity: Maximal Oxygen Consumption (VO2max), maximal work rate, and anaerobic threshold | Not Posted | 11 years ± 1 year to 16 years | Participants |
| Secondary | Atrial and Ventricular Arrhythmias | The incidence of arrhythmias between those randomized to a RVPAS vs. a MBTS. | Not Posted | 11 years ± 1 year to 16 years | Participants |
| Secondary | The Neurodevelopmental Outcomes at 11 Years of Age in Those Randomized to a RVPAS vs. a MBTS | Achievement, as measured by the Wechsler Individual Achievement Tests (WIAT) (Math and Reading); Intelligence, as measured by the Wechsler Intelligence Scale for Children (WISC); Other domains of neurodevelopmental function including assessment of language, executive function, visual spatial skills, motor function, memory, social skills, behavior, health-related quality of life (HRQoL) and adaptive function. | Not Posted | 11 years ± 1 year | Participants |
| Secondary | Risk Stratification Models | Develop risk stratification models for 1) cardiac outcomes, 2) transplant-free survival, and 3) neurodevelopmental outcomes. | Not Posted | 11 years ± 1 year to 16 years | Participants |
| Secondary | Biospecimens | Collect specimens from subjects and their parents to further develop the biologic specimen repository. | Not Posted | 11 years ± 1 year to 16 years | Participants |
| 109 |
| 0 |
| 109 |
| 0 |
| 109 |
| EG001 | RVPAS | right-ventricular-to-pulmonary-artery shunt at Norwood | 3 | 128 | 0 | 128 | 0 | 128 |
Not provided
Not provided