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| Name | Class |
|---|---|
| Stanford University | OTHER |
| Children's Hospital of Philadelphia | OTHER |
| New York Presbyterian Hospital | OTHER |
| University of Texas Southwestern Medical Center |
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This is a retrospective cohort study of pediatric hypertrophic cardiomyopathy (HCM) patients using chart and registry review methodology. The studies objective is to develop and validate a sudden cardiac death (SCD) risk calculator that is age-appropriate for children with HCM that includes clinical and genetic factors.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Hypertrophic Cardiomyopathy |
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| Measure | Description | Time Frame |
|---|---|---|
| Number of Participants With a Composite Sudden Cardiac Death Event | The composite SCD event includes post diagnosis SCD, aborted SCD (including ventricular fibrillation, sustained ventricular tachycardia), primary ICD insertion with appropriate shock, secondary ICD insertion | Time to a composite sudden cardiac death event during 5-year follow-up |
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Inclusion Criteria:
Exclusion Criteria:
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Individuals meeting eligibility criteria at first presentation were included.
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 32418493 | Result | Miron A, Lafreniere-Roula M, Steve Fan CP, Armstrong KR, Dragulescu A, Papaz T, Manlhiot C, Kaufman B, Butts RJ, Gardin L, Stephenson EA, Howard TS, Aziz PF, Balaji S, Ladouceur VB, Benson LN, Colan SD, Godown J, Henderson HT, Ingles J, Jeewa A, Jefferies JL, Lal AK, Mathew J, Jean-St-Michel E, Michels M, Nakano SJ, Olivotto I, Parent JJ, Pereira AC, Semsarian C, Whitehill RD, Wittekind SG, Russell MW, Conway J, Richmond ME, Villa C, Weintraub RG, Rossano JW, Kantor PF, Ho CY, Mital S. A Validated Model for Sudden Cardiac Death Risk Prediction in Pediatric Hypertrophic Cardiomyopathy. Circulation. 2020 Jul 21;142(3):217-229. doi: 10.1161/CIRCULATIONAHA.120.047235. Epub 2020 May 18. |
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This was a multicenter retrospective observational cohort study of pediatric patients with clinically diagnosed childhood-onset HCM. The primary cohort included patients from 11 sites participating in the PRIMaCY study (Precision Medicine for Cardiomyopathy), an international registry of patients with pediatric HCM launched in 2017. These included 4 Canadian, 6 US, and 1 Australian site. For model validation, we used data from the Sarcomeric Human Cardiomyopathy Registry.
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| ID | Title | Description |
|---|---|---|
| FG000 | Hypertrophic Cardiomyopathy | Patients with a clinical diagnosis of HCM defined as having an LV posterior wall or septal thickness z score ≥2. |
| Title | Milestones | Reasons Not Completed | ||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Overall Study |
|
Participants with a clinical diagnosis of HCM
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| ID | Title | Description |
|---|---|---|
| BG000 | Hypertrophic Cardiomyopathy | Patients included were those with a clinical diagnosis of HCM defined as having LV posterior wall or septal thickness z score ≥2, age <18 years at the time of diagnosis, absence of a SCD event before diagnosis, and at least 1 follow-up assessment after diagnosis. |
| Units | Counts |
|---|---|
| Participants |
|
| Title | Description | Population Description | Parameter Type | Dispersion Type | Unit of Measure | Calculate Percentage | Denominator Units Selected | Denominators | Classes |
|---|---|---|---|---|---|---|---|---|---|
| Age, Continuous | Median |
| 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 | Number of Participants With a Composite Sudden Cardiac Death Event | The composite SCD event includes post diagnosis SCD, aborted SCD (including ventricular fibrillation, sustained ventricular tachycardia), primary ICD insertion with appropriate shock, secondary ICD insertion | Patients with a clinical diagnosis of HCM defined as having an LV posterior wall or septal thickness z score ≥2, age <18 years at the time of diagnosis, absence of a sudden cardiac death event before diagnosis, and at least 1 follow-up assessment after diagnosis. | Posted | Count of Participants | Participants | Time to a composite sudden cardiac death event during 5-year follow-up |
|
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Adverse events were not captured as this was a retrospective observational cohort study
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| ID | Title | Description | Deaths (Affected) | Deaths (At Risk) | Serious Events (Affected) | Serious Events (At Risk) | Other Events (Affected) | Other Events (At Risk) |
|---|---|---|---|---|---|---|---|---|
| EG000 | Hypertrophic Cardiomyopathy | Patients with a clinical diagnosis of HCM defined as having an LV posterior wall or septal thickness z score ≥2. |
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The study has limitations inherent to any retrospective analysis, that includes missing data, survivor bias, and lower uptake of genetic testing in the earlier era. Missing echocardiographic data were addressed through a re-review of echocardiograms where available and through imputation where this was not possible. We deliberately did not perform an echocardiographic core laboratory analysis because a real-world, point-of-care tool has to rely on locally acquired data.
| Title | Organization | Phone | Extension | |
|---|---|---|---|---|
| Seema Mital, Staff Cardiologist | Hospital for Sick Children | 416-813-7418 | seema.mital@sickkids.ca |
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| Type | Includes Protocol | Includes SAP | Includes ICF | Document Label | Document Date | Document Uploaded Date | Document File Name |
|---|---|---|---|---|---|---|---|
| Prot | Yes | No | No | Study Protocol | Nov 30, 2018 | Dec 21, 2020 | Prot_000.pdf |
| SAP | No | Yes | No | Statistical Analysis Plan | Jul 21, 2020 | Mar 10, 2021 | SAP_001.pdf |
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| ID | Term |
|---|---|
| D002312 | Cardiomyopathy, Hypertrophic |
| D016757 | Death, Sudden, Cardiac |
| ID | Term |
|---|---|
| D009202 | Cardiomyopathies |
| D006331 | Heart Diseases |
| D002318 | Cardiovascular Diseases |
| D001020 | Aortic Stenosis, Subvalvular |
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| OTHER |
| University of Michigan | OTHER |
| Children's Hospital Medical Center, Cincinnati | OTHER |
| Provincial Health Services Authority British Columbia | OTHER |
| Stollery Children's Hospital | OTHER |
| Children's Hospital of Eastern Ontario | OTHER |
| Royal Children's Hospital | OTHER |
| Children's Healthcare of Atlanta | OTHER |
| Primary Children's Hospital | OTHER |
| Baylor College of Medicine | OTHER |
| The Cleveland Clinic | OTHER |
| Monroe Carell Jr. Children's Hospital at Vanderbilt | OTHER |
| Children's Hospital Colorado | OTHER |
| Indiana University Health | OTHER |
| OHSU Doernbecher Children's Hospital | OTHER |
| Children's Hospital Los Angeles | OTHER |
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| years |
|
| Sex: Female, Male | Count of Participants | Participants |
|
| Race and Ethnicity Not Collected | Race and Ethnicity were not collected from any participant. | Count of Participants | Participants |
|
| Region of Enrollment | Number | participants |
|
| Units | Counts |
|---|---|
| Participants |
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| 0 |
| 0 |
| 0 |
| 0 |
| 0 |
| 0 |
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| D001024 |
| Aortic Valve Stenosis |
| D000082862 | Aortic Valve Disease |
| D006349 | Heart Valve Diseases |
| D006323 | Heart Arrest |
| D003645 | Death, Sudden |
| D003643 | Death |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |