Evidence map›Paper›PMID 32418493›Full record

ArticleCirculation2020

A Validated Model for Sudden Cardiac Death Risk Prediction in Pediatric Hypertrophic Cardiomyopathy.

Anastasia Miron, Myriam Lafreniere-Roula, Chun-Po Steve Fan, Katey R Armstrong, Andreea Dragulescu, Tanya Papaz, Cedric Manlhiot, Beth Kaufman, Ryan J Butts, Letizia Gardin and 32 more

Registry-linked trialOpen access · bronzeAbstract readMulticenter Study
PubMed Full text at PMC Publisher
In one paragraph

Article in Circulation, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT04036799 (Development of a Risk Calculator to Predict Sudden Cardiac Death in Children With Hypertrophic Cardiomyopathy), which is not on this map. Cited by 86 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
86citing papers in PubMed, 2 pooled it
18.9field-weighted citation impact, top 1% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT04036799 active not recruitingnot on this map

Development of a Risk Calculator to Predict Sudden Cardiac Death in Children With Hypertrophic Cardiomyopathy

TypeobservationalSponsorThe Hospital for Sick ChildrenRan2017 to 2026Enrolled572ConditionsHypertrophic Cardiomyopathy, Sudden Cardiac Death
3 · Its place in the literature

Who cites it

86 citing papers in PubMed, 2 syntheses or guidelines pooled it, 219 citations in OpenAlex.

  1. Pooled it
  2. Guideline
  3. Trial
  4. Article
  5. Article
  6. Review
  7. Article
  8. Review
  9. Review
  10. Review
  11. Article
  12. Genetics of Sudden Cardiac Death.Diseases (Basel, Switzerland) · 2025
    Review
  13. Article
  14. Article
  15. Hypertrophic cardiomyopathy.Nature reviews. Disease primers · 2025
    Review
  16. Article
  17. Article
  18. Article
  19. Article
  20. Article

26 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

42 authors at 20 institutions in 6 countries.

Anastasia MironDivision of Cardiology (A.M., T.P., S.M.), Hospital for Sick Children, Toronto, Ontario, Canada.
Myriam Lafreniere-RoulaTed Rogers Computational Program, Ted Rogers Center for Heart Research, The Hospital for Sick Children, University Health Network, Toronto, Ontario, Canada (M.L.-R., C.-P, S.F.).
Chun-Po Steve FanTed Rogers Computational Program, Ted Rogers Center for Heart Research, The Hospital for Sick Children, University Health Network, Toronto, Ontario, Canada (M.L.-R., C.-P, S.F.).
Katey R ArmstrongDivision of Pediatric Cardiology, Department of Pediatrics, British Columbia Children's Hospital, Vancouver, Canada (K.R.A.).
Andreea DragulescuDepartment of Cardiology (A.D., V.B.L., L.N.B., A.J., E.J.-St-M.), Hospital for Sick Children, Toronto, Ontario, Canada.
Tanya PapazDivision of Cardiology (A.M., T.P., S.M.), Hospital for Sick Children, Toronto, Ontario, Canada.
Cedric ManlhiotDepartment of Pediatrics, Johns Hopkins Medical Center, Baltimore, MD (C.M.).
Beth KaufmanDepartment of Pediatrics, Lucile Packard Children's Hospital Stanford, Palo Alto, CA (B.K.).
Ryan J ButtsDivision of Pediatric Cardiology, Department of Pediatrics, Children's Medical Center of Dallas, TX (R.J.B.).
Letizia GardinDepartment of Cardiology, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada (L.G.).
Elizabeth A StephensonDepartment of Cardiology, Labatt Family Heart Center, Hospital for Sick Children, University of Toronto, Ontario, Canada (E.A.S., S.M.).
Taylor S HowardDepartment of Pediatrics, Section of Pediatric Cardiology, Baylor College of Medicine, Texas Children's Hospital (T.S.H.).
Pete F AzizCenter for Pediatric and Congenital Heart Disease, Pediatric Electrophysiology and Pacing, Cleveland Clinic Children's Hospital, OH (P.F.A.).
Seshadri BalajiDepartment of Pediatrics, Division of Cardiology, Oregon Health & Science University, OHSU Doernbecher Children's Hospital, Portland (S.B.).
Virginie Beauséjour LadouceurDepartment of Cardiology (A.D., V.B.L., L.N.B., A.J., E.J.-St-M.), Hospital for Sick Children, Toronto, Ontario, Canada.
Lee N BensonDepartment of Cardiology (A.D., V.B.L., L.N.B., A.J., E.J.-St-M.), Hospital for Sick Children, Toronto, Ontario, Canada.
Steven D ColanDepartment of Cardiology, Boston Children's Hospital, MA (S.D.C.).
Justin GodownDepartment of Pediatrics, Division of Pediatric Cardiology, Monroe Carrell Jr Children's Hospital at Vanderbilt, Nashville, TN (J.G.).
Heather T HendersonPediatrics, Medical University of South Carolina, Charleston (H.T.H.).
Jodie InglesAgnes Ginges Center for Molecular Cardiology at Centenary Institute, The University of Sydney, New South Wales, Australia (J.I., C.S.).
Aamir JeewaDepartment of Cardiology (A.D., V.B.L., L.N.B., A.J., E.J.-St-M.), Hospital for Sick Children, Toronto, Ontario, Canada.
John L JefferiesDivision of Adult Cardiovascular Diseases, University of Tennessee Health Science Center, Memphis (J.L.J.).
Ashwin K LalDivision of Pediatric Cardiology, University of Utah Primary Children's Hospital, Salt Lake City (A.K.L.).
Jacob MathewDepartment of Cardiology, The Royal Children's Hospital, Melbourne, Victoria, Australia (J.M., R.G.W.).
Emilie Jean-St-MichelDepartment of Cardiology (A.D., V.B.L., L.N.B., A.J., E.J.-St-M.), Hospital for Sick Children, Toronto, Ontario, Canada.
Michelle MichelsDepartment of Cardiology, Thoraxcenter, Erasmus MC Rotterdam, South Holland, Netherlands (M.M.).
Stephanie J NakanoDepartment of Pediatrics, Division of Cardiology, Children's Hospital Colorado, Aurora (S.J.N.).
Iacopo OlivottoReferral Center for Cardiomyopathies, Careggi University Hospital, Florence, Italy (I.O.).
John J ParentDepartment of Pediatrics, Riley Children's Hospital, Indianapolis, IN (J.J.P.).
Alexandre C PereiraLaboratory of Genetics and Molecular Cardiology, Heart Institute (InCor), University of Sao Paulo Medical School, Brazil (A.C.P.).
Christopher SemsarianAgnes Ginges Center for Molecular Cardiology at Centenary Institute, The University of Sydney, New South Wales, Australia (J.I., C.S.).
Robert D WhitehillDivision of Cardiology, Children's Healthcare of Atlanta, GA (R.D.W.).
Samuel G WittekindThe Heart Institute, Cincinnati Children's Hospital, OH (S.G.W., C.V.).
Mark W RussellPediatrics, C.S. Mott Children's Hospital, Ann Arbor, MI (M.W.R.).
Jennifer ConwayDivision of Pediatric Cardiology, Department of Pediatrics, Stollery Children's Hospital, Edmonton, AB, Canada (J.C.).
Marc E RichmondDivision of Pediatric Cardiology, Department of Pediatrics, Columbia University College of Physicians and Surgeons/Morgan Stanley Children's Hospital, New York, NY (M.E.R.).
Chet VillaThe Heart Institute, Cincinnati Children's Hospital, OH (S.G.W., C.V.).
Robert G WeintraubDepartment of Cardiology, The Royal Children's Hospital, Melbourne, Victoria, Australia (J.M., R.G.W.).
Joseph W RossanoDivision of Cardiology, Children's Hospital of Philadelphia, PA (J.W.R.).
Paul F KantorDivision of Cardiology, Children's Hospital of Los Angeles, CA (P.F.K.).
Carolyn Y HoCardiovascular Division, Brigham and Women's Hospital, Boston, MA (C.Y.H.).
Seema MitalDivision of Cardiology (A.M., T.P., S.M.), Hospital for Sick Children, Toronto, Ontario, Canada.
Hospital for Sick Children · CACentenary Institute · AUCincinnati Children's Hospital Medical Center · USRoyal Children's Hospital · AUTed Rogers Centre for Heart Research · CAAzienda Ospedaliero-Universitaria Careggi · ITBaylor College of Medicine · USBC Children's Hospital · CABoston Children's Hospital · USBrigham and Women's Hospital · USChildren's Healthcare of Atlanta · USChildren's Hospital Colorado · USChildren's Hospital of Eastern Ontario · CAChildren's Hospital of Los Angeles · USChildren's Hospital of Philadelphia · USChildren's Medical Center · USCleveland Clinic · USC. S. Mott Children's Hospital · USDoernbecher Children's Hospital · USErasmus University Rotterdam · NL

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundHypertrophic cardiomyopathy is the leading cause of sudden cardiac death (SCD) in children and young adults. Our objective was to develop and validate a SCD risk prediction model in pediatric hypertrophic cardiomyopathy to guide SCD prevention strategies.

methodsIn an international multicenter observational cohort study, phenotype-positive patients with isolated hypertrophic cardiomyopathy <18 years of age at diagnosis were eligible. The primary outcome variable was the time from diagnosis to a composite of SCD events at 5-year follow-up: SCD, resuscitated sudden cardiac arrest, and aborted SCD, that is, appropriate shock following primary prevention implantable cardioverter defibrillators. Competing risk models with cause-specific hazard regression were used to identify and quantify clinical and genetic factors associated with SCD. The cause-specific regression model was implemented using boosting, and tuned with 10 repeated 4-fold cross-validations. The final model was fitted using all data with the tuned hyperparameter value that maximizes the c-statistic, and its performance was characterized by using the c-statistic for competing risk models. The final model was validated in an independent external cohort (SHaRe [Sarcomeric Human Cardiomyopathy Registry], n=285).

resultsOverall, 572 patients met eligibility criteria with 2855 patient-years of follow-up. The 5-year cumulative proportion of SCD events was 9.1% (14 SCD, 25 resuscitated sudden cardiac arrests, and 14 aborted SCD). Risk predictors included age at diagnosis, documented nonsustained ventricular tachycardia, unexplained syncope, septal diameter

conclusionOur study provides a validated SCD risk prediction model with >70% prediction accuracy and incorporates risk factors that are unique to pediatric hypertrophic cardiomyopathy. An individualized risk prediction model has the potential to improve the application of clinical practice guidelines and shared decision making for implantable cardioverter defibrillator insertion. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT0403679.

Indexed as

Models, StatisticalAdolescentAge FactorsAlgorithmsCardiomyopathy, HypertrophicChildDeath, Sudden, CardiacFemaleHumansMalePublic Health SurveillanceReproducibility of ResultsRetrospective StudiesRisk AssessmentRisk Factorscardiomyopathiescardiomyopathy, hypertrophicdeath, sudden, heartdefibrillators, implantablehypertrophypediatrics

Identifiers

PMID32418493
PMCPMC7365676
OpenAlexW3024147252

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC-ND
Read underepoch 390

Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.