Evidence map›Paper›PMID 34718528›Full record

ArticleEuropean journal of preventive cardiology2022

External validation of the HCM Risk-Kids model for predicting sudden cardiac death in childhood hypertrophic cardiomyopathy.

Gabrielle Norrish, Chen Qu, Ella Field, Elena Cervi, Diala Khraiche, Sabine Klaassen, Tiina H Ojala, Gianfranco Sinagra, Hirokuni Yamazawa, Chiara Marrone and 17 more

Open access · bronzeAbstract read
In one paragraph

Article in European journal of preventive cardiology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
25citing papers in PubMed, 1 pooled it
6.6field-weighted citation impact, top 2% 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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

25 citing papers in PubMed, 1 synthesis or guideline pooled it, 63 citations in OpenAlex.

  1. Guideline
  2. Article
  3. Article
  4. Article
  5. Review
  6. Review
  7. Review
  8. Hypertrophic cardiomyopathy.Nature reviews. Disease primers · 2025
    Review
  9. Review
  10. Article
  11. Review
  12. Review
  13. Article
  14. Article
  15. Article
  16. Performance of the PRIMaCY sudden death risk prediction model for childhood hypertrophic cardiomyopathy: implications for implantable cardioverter-defibrillator decision-making.Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2023
    Article
  17. Review
  18. Spotlight on the 2022 ESC guideline management of ventricular arrhythmias and prevention of sudden cardiac death: 10 novel key aspects.Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2023
    Review
  19. Should lethal arrhythmias in hypertrophic cardiomyopathy be predicted using non-electrophysiological methods?Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2023
    Article
  20. Review
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

27 authors at 20 institutions in 10 countries.

Gabrielle NorrishCentre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London WC1N 3JH, UK.ORCID 0000-0003-2064-237X
Chen QuDepartment of Statistical Science, University College London, London, UK.
Ella FieldCentre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London WC1N 3JH, UK.
Elena CerviCentre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London WC1N 3JH, UK.
Diala KhraicheNecker - Enfants Malades hospital, Paris, France.
Sabine KlaassenDepartment of Paediatric Cardiology, Charite - Universitatsmedizin Berlin, Berlin, Germany.
Tiina H OjalaDepartment of Paediatric Cardiology, New Children's Hospital, University of Helsinki, Helsinki, Finland.ORCID 0000-0001-9092-7763
Gianfranco SinagraHeart Muscle Disease Registry Trieste, University of Trieste, Trieste, Italy.
Hirokuni YamazawaDepartment of Paediatrics, Faculty of Medicine and Graduate school of Medicine, Hokkaido University Hospital, Sapporo, Japan.
Chiara MarroneFondazione Toscana G Monasterio, Massa-Pisa, Italy.
Anca PopoiuDepartment of Paediatrics, Children's Hospital 'Louis Turcanu', University of Medicine and Pharmacy "Victor Babes" Timisoara, Timisoara, Romania.
Fernando CentenoRio Hortega University Hospital, Valladolid, Spain.
Sylvie SchouveyHospital Saint Joseph, Marseille, France.
Iacopo OlivottoCardiomyopathy Unit, Careggi University Hospital, Florence, Italy.ORCID 0000-0003-1751-9266
Sharlene M DayDepartment of Internal Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Steve ColanBoston Children's Hospital, Harvard Medical School, Boston, MA, USA.
Joseph RossanoChildren's Hospital of Philadelphia, Philadelphia, PA, USA.
Samuel G WittekindCincinnati Children's Hospital Medical Center, Heart Institute, Cincinnati, OH, USA.
Sara SaberiDepartment of Internal Medicine-Cardiology, University of Michigan, Ann Arbor, MI, USA.
Mark RussellDepartment of Internal Medicine-Cardiology, University of Michigan, Ann Arbor, MI, USA.
Adam HelmsDepartment of Internal Medicine-Cardiology, University of Michigan, Ann Arbor, MI, USA.
Jodie InglesCardio Genomics Program at Centenary Institute, The University of Sydney, Sydney, Australia.ORCID 0000-0002-4846-7676
Christopher SemsarianAgnes Ginges Centre for Molecular Cardiology, Centenary Institute, The University of Sydney, Sydney, Australia.
Perry M ElliottInstitute of Cardiovascular Sciences, University College London, London, UK.
Carolyn Y HoCardiovascular Division, Brigham and Women's Hospital, Boston, MA, USA.
Rumana Z OmarDepartment of Statistical Science, University College London, London, UK.
Juan P KaskiCentre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London WC1N 3JH, UK.
Great Ormond Street Hospital · GBUniversity of Michigan · USThe University of Sydney · AUUniversity College London · GBAzienda Ospedaliero-Universitaria Careggi · ITBoston Children's Hospital · USBrigham and Women's Hospital · USChildren's Hospital of Philadelphia · USCincinnati Children's Hospital Medical Center · USFondazione Toscana Gabriele Monasterio · ITHokkaido University Hospital · JPHôpital Saint Joseph · FRHospital Universitario Río Hortega · ESInstitut Necker Enfants Malades · FRMax Delbrück Center · DESt Bartholomew's Hospital · GBUniversity of Helsinki · FIUniversity of Pennsylvania · USUniversity of Trieste · ITVictor Babeș University of Medicine and Pharmacy Timișoara · RO

Funding

British Heart Foundation FS/16/72/32270Medical Research Council MR/T024062/1
6 · The paper itself

Abstract

aimsSudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM). The newly developed HCM Risk-Kids model provides clinicians with individualized estimates of risk. The aim of this study was to externally validate the model in a large independent, multi-centre patient cohort. METHODS AND

resultsA retrospective, longitudinal cohort of 421 patients diagnosed with HCM aged 1-16 years independent of the HCM Risk-Kids development and internal validation cohort was studied. Data on HCM Risk-Kids predictor variables (unexplained syncope, non-sustained ventricular tachycardia, maximal left ventricular wall thickness, left atrial diameter, and left ventricular outflow tract gradient) were collected from the time of baseline clinical evaluation. The performance of the HCM Risk-Kids model in predicting risk at 5 years was assessed. Twenty-three patients (5.4%) met the SCD end-point within 5 years, with an overall incidence rate of 2.03 per 100 patient-years [95% confidence interval (CI) 1.48-2.78]. Model validation showed a Harrell's C-index of 0.745 (95% CI 0.52-0.97) and Uno's C-index 0.714 (95% 0.58-0.85) with a calibration slope of 1.15 (95% 0.51-1.80). A 5-year predicted risk threshold of ≥6% identified 17 (73.9%) SCD events with a corresponding C-statistic of 0.702 (95% CI 0.60-0.81).

conclusionsThis study reports the first external validation of the HCM Risk-Kids model in a large and geographically diverse patient population. A 5-year predicted risk of ≥6% identified over 70% of events, confirming that HCM Risk-Kids provides a method for individualized risk predictions and shared decision-making in children with HCM.

Indexed as

Cardiomyopathy, HypertrophicDeath, Sudden, CardiacAdolescentChildChild, PreschoolCohort StudiesHumansIncidenceInfantRetrospective StudiesRisk AssessmentRisk FactorsHypertrophic cardiomyopathyPaediatricRisk stratificationSudden death

Identifiers

PMID34718528
PMCPMC8967478
OpenAlexW3209636806

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Registered trials

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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.