[III-SY09-03] 小児心筋症における心臓突然死の予防
Keywords:hypertrophic cardiomyopathy, sudden cardiac death, risk prediction model
[Aim] Prevention of sudden cardiac death (SCD) is important in hypertrophic cardiomyopathy (HCM). This study focused on verifying the HCM Risk-Kids model (HRK), a risk prediction model published in JAMA cardiology 2019, and searching additional risk factors. [Method] Thirty-six patients with HCM were included. SCD-related events (SCDEs) were defined as VT/Vf, SCD, aborted cardiac arrest, and appropriate ICD therapy. HRK values, maximum annual change of maximum wall thickness (mWT per year), and Log-transformed (Ln) cardiac troponin (cTn) values were compared with and without SCDEs. P<0.05 was statistically significant. [Results] HRK values, mWT per year, and LncTnT values were significantly different with and without SCDEs (p=0.05, p=0.0004, and p=0.03, respectively). ROC analysis resulted in a sensitivity of 100%, and specificity of 38%, for HRK values of 6%/5 years or higher, which should be considered for primary prevention of SCD. The cut-off value for predicting SCDEs was an increase in the wall thickening of 2.3 mm per year (sensitivity 100% and specificity 70%). The ROC curve of cTnT levels to predict SCDEs showed that the cutoff value was 21 pg/ml. The group with a cTnT level of 21 pg/ml or higher had more SCDEs than that of less than 21 pg/ml (log-rank p=0.007). [Discussio Based on comparisons with the Taft HCM Institute, risk prediction of HCM in Japanese patients may be somewhat inaccurate. We believe that the mWT per year and cTnT may be additional risk factors that compensates for inaccuracy in risk assessment model.