@article {Choiheartjnl-2019-315160, author = {You-Jung Choi and Hyung-Kwan Kim and Sang Chol Lee and Jun-Bean Park and Inki Moon and Jiesuck Park and Yong-Jin Kim and Dae-Won Sohn and Steve Ommen}, title = {Validation of the hypertrophic cardiomyopathy risk-sudden cardiac death calculator in Asians}, elocation-id = {heartjnl-2019-315160}, year = {2019}, doi = {10.1136/heartjnl-2019-315160}, publisher = {BMJ Publishing Group Ltd}, abstract = {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.}, issn = {1355-6037}, URL = {https://heart.bmj.com/content/early/2019/08/23/heartjnl-2019-315160}, eprint = {https://heart.bmj.com/content/early/2019/08/23/heartjnl-2019-315160.full.pdf}, journal = {Heart} }