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. 2022 Jan-Feb:63:15-21.
doi: 10.1016/j.hjc.2021.06.005. Epub 2021 Jun 17.

Validation of the new American College of Cardiology/American Heart Association Guidelines for the risk stratification of sudden cardiac death in a large Mediterranean cohort with Hypertrophic Cardiomyopathy

Thomas Zegkos  1 , Georgios Tziomalos  2 , Despoina Parcharidou  2 , Dimitris Ntelios  2 , Christos A Papanastasiou  2 , Efstratios Karagiannidis  2 , Thomas Gossios  2 , Pavlos Rouskas  2 , Sotiris Katranas  2 , Stilianos Paraskevaidis  2 , Haralambos Karvounis  2 , Georgios Efthimiadis  2
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Free article

Validation of the new American College of Cardiology/American Heart Association Guidelines for the risk stratification of sudden cardiac death in a large Mediterranean cohort with Hypertrophic Cardiomyopathy

Thomas Zegkos et al. Hellenic J Cardiol. 2022 Jan-Feb.
Free article

Abstract

Background: The aim of our study was to assess the performance of the new American College of Cardiology (ACC)/American Heart Association (AHA) Guidelines, with respect to sudden cardiac death (SCD) prevention, in comparison with the established risk score of the European Society of Cardiology (hypertrophic cardiomyopathy [HCM] Risk-SCD), in a large Mediterranean cohort of HCM patients.

Methods: The clinical and imaging characteristics of 784 HCM patients (mean age at first evaluation 52 ± 16 years, 67.2% males) were analyzed retrospectively. The sensitivity, specificity, and negative predictive value for SCD events of the presence of ≥1 risk factor for SCD according to the ACC/AHA Guidelines 2020 and of the HCM Risk-SCD≥6% and HCM Risk-SCD≥4% were estimated during follow-up.

Results: During follow-up, 47 (6%) patients suffered an SCD event. The presence of ≥1 major risk factor for SCD according to the new ACC/AHA Guidelines had 96% sensitivity (95% CI 85.5-99.5%) with modest specificity of 59% (95% CI 55-62.2%) and negative predictive value of 99.5% (95% CI 98.2-99.9%). On the contrary, HCM- Risk-SCD≥6% had a relatively low sensitivity (32%, 95% CI 19.1-47.1%) and high specificity of 95% (95% CI 93.1-96.4%), whereas, HCM-Risk-SCD≥4% had sensitivity of 60% (95% CI 44-74%) and specificity of 83.9% (95% CI 80-85.6%). Both the HCM Risk-SCD cut-off values demonstrated lower negative predictive value but higher accuracy than the ACC/AHA algorithm for SCD prediction.

Conclusion: The novel ACC/AHA proposed algorithm identifies most of the patients with an SCD event with the cost of numerous defibrillator implantations. HCM-Risk-SCD demonstrated higher specificity, whereas its sensitivity and negative predictive value are modest.

Keywords: hypertrophic cardiomyopathy; implantable cardioverter defibrillator; primary prevention; risk stratification; sudden death.

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Conflict of interest statement

Conflict of interest None declared by all authors.

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