Ingegerd Östman-Smith1, Gunnar Sjöberg2, Jenny Alenius Dahlqvist3, Per Larsson4, Eva Fernlund5,6. 1. Department of Pediatric Cardiology, Queen Silvia Children's Hospital, Sahlgrenska Academy, Gothenburg University, Gothenburg, Sweden. 2. Department of Women's and Children's Health, Karolinska Institute, Stockholm, Sweden. 3. Department of Clinical Sciences, Umeå University, Umeå, Sweden. 4. Department of Pediatric Cardiology, Uppsala University, Children's Hospital, Uppsala, Sweden. 5. Department of Clinical and Experimental Medicine, Linköping University, Division of Pediatrics, Crown Princess Victoria Children's Hospital, Linköping University Hospital, Linköping, Sweden. 6. Department of Clinical Sciences Lund, Lund University, Skåne University Hospital, Pediatric Cardiology, Lund, Sweden.
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. This article is protected by copyright. All rights reserved.
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. This article is protected by copyright. All rights reserved.
Entities:
Keywords:
Beta-blocker; Hypertrophic cardiomyopathy; Noonan syndrome; Risk factors; Sudden death
Authors: Cheuk To Chung; George Bazoukis; Sharen Lee; Ying Liu; Tong Liu; Konstantinos P Letsas; Antonis A Armoundas; Gary Tse Journal: Int J Arrhythmia Date: 2022-04-01