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Original research
Validation of the hypertrophic cardiomyopathy risk-sudden cardiac death calculator in Asians
  1. You-Jung Choi1,
  2. Hyung-Kwan Kim1,
  3. Sang Chol Lee2,
  4. Jun-Bean Park1,
  5. Inki Moon1,
  6. Jiesuck Park1,
  7. Yong-Jin Kim1,
  8. Dae-Won Sohn1,
  9. Steve Ommen3
  1. 1 Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea
  2. 2 Department of Cardiology and Cardiovascular Imaging Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea
  3. 3 Department of Cardiovascular Disease, Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Professor Hyung-Kwan Kim, Internal Medicine, Seoul National University Hospital, Seoul, 03080, Republic of Korea; cardiman73{at}; Dr Sang Chol Lee, Cardiovascular Medicine/Cardiovascular Imaging Specialist, Sungkyunkwan University School of Medicine/Samsung Medical Center Cardiovascular Imaging Center 135-710, Seoul, Korea; chrislee.echo{at}


Objective The hypertrophic cardiomyopathy (HCM) risk-sudden cardiac death (SCD) calculator endorsed by the 2014 European Society of Cardiology has not been independently validated in the Asians. We aimed to investigate whether the HCM Risk-SCD calculator effectively predicts SCD in Korean HCM population.

Methods An observational, longitudinal cohort study was performed in 730 patients with HCM from 2007 to 2017. The primary endpoint was a composite of SCD and appropriate implantable cardioverter-defibrillator (ICD) therapy.

Results During a follow-up period of 4288 person-years, 16 (2.2%) patients reached the primary endpoint. This validation study revealed a calibration slope of 0.892 and C-statistics of 0.718. The primary endpoint occurred in 1.1% (7/615), 4.6% (3/65) and 12.0% (6/50) of low-risk, intermediate-risk and high-risk groups, respectively. Although most patients (85.2%) without the primary endpoint were classified into the low-risk group, 7 of 11 SCD (63.6%) occurred in the low-risk group. In univariable and multivariable analysis, sex (woman) was significantly associated with the primary endpoint and emerged as independent predictor. The addition of sex to the HCM Risk-SCD calculator significantly improved the predictive value of the primary endpoint (net reclassification improvement 0.557, p=0.015).

Conclusions In the Korean HCM population, the HCM Risk-SCD calculator had a high negative predictive value and accuracy for predicting SCD or appropriate ICD therapy, but misclassified a few patients experiencing the primary endpoint as low-risk or intermediate-risk groups.

  • asia
  • cardiomyopathy, hypertrophic
  • death, sudden, cardiac
  • forecasting
  • risk assessment

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  • H-KK and SCL contributed equally.

  • Contributors YJC, HKK and SCL contributed to the planning of study. YJC, HKK and JBP contributed to the study design. YJC, HKK, SCL and JBP did the statistical analysis. YJC, HKK, SCL and SO contributed to the drafting of the paper. All the authors contributed to the interpretation of results and critical review of the manuscript.

  • Funding This study was supported by the research grant from ChongKunDang 2018 research fund (No. 0620183330 (2017-1493)).

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Ethics approval The institutional review board of each hospital approved the study protocol.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability statement No data are available.

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