Acta Paediatr. 2021 Jul 27. doi: 10.1111/apa.16045. Online ahead of print.
ABSTRACT
AIM: To compare risk-algorithms (HCMRisk-Kids, ECG Risk-score) in hypertrophic cardiomyopathy (HCM) without syndrome association (ns-HCM), and with Noonan-like syndromes (RAS-HCM).
METHODS: A national paediatric HCM-cohort (n=151), presenting <19y of age, mean follow-up 13.3y, from all Swedish centres of Paediatric Cardiology (presenting 1972-2015), with 41 RAS-HCM-patients (61% males), and 110 ns-HCM-patients (68% familial; 65% males). The end-point was a composite of sudden cardiac death and re-suscitated cardiac arrest (SCD/CA). Risk-factors were studied with Cox-hazard regression, and ROC-curve analysis (C-statistic).
RESULTS: There were 33 SCD/CA, 27/110 in ns-HCM and 6/41 in RAS-HCM (p=0.27). In ns-HCM HCMRisk-Kids ≥6% at diagnosis had C-statistic of 0.69 for predicting SCD/CA during first 5y of follow-up, and positive predictive value (PPV) of 22%. After 7y of age (HCMRisk-Kids7plus) C-statistic was 0.76. ECG Risk-score ≥6 at diagnosis had C-statistic 0.87 and PPV of 31%. Independent risk factors for SCD/CA were HCMRisk-Kids7plus score (p=0.005) and ECG risk-score (p<0.001), whereas early beta-blocker dose (p=0.001) and myectomy (p=0.004) reduced risk. The sum of HCMRisk-Kids7yplus and ECG Risk-score7yplus ≥14 best predicted SCD/CA within 5y in ns-HCM with C-statistic of 0.90 [0.83-0.96], sensitivity 100%, and PPV 38%.
CONCLUSIONS: Combining the ECG Risk-score with HCMRisk-Kids improves risk-stratification in ns-HCM, and shows promise in RAS-HCM.
PMID:34314540 | DOI:10.1111/apa.16045
