Comparison of the Prognostic Usefulness of the European Society of Cardiology and American Heart Association/American College of Cardiology Foundation Risk Stratification Systems for Patients With Hypertrophic Cardiomyopathy

Abstract

Implantable cardiodefibrillators (ICDs) have proven benefit in preventing sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HC), making risk stratification essential. Data on the predictive accuracy on the European Society of Cardiology (ESC) risk scoring system have been conflicting. We independently evaluated the ESC risk scoring system in our cohort of patients with HC from a large tertiary center and compared this with previous guidance by the American College of Cardiology Foundation and Heart Association (ACCF/AHA). Risk factor profiles, 5-year SCD risk estimates, and ICD recommendations, as defined by the ACCF/AHA and ESC guidelines, were retrospectively ascertained for 288 HC patients with and without SCD or equivalent events at our center. In the SCD group (n = 14), a significantly higher proportion of patients would not have met the criteria for an ICD implant using the ESC scoring algorithm compared with ACCF/AHA guidance (43% vs 7%, p = 0.029). In those without SCD events (n = 274), a larger proportion of individuals not requiring an ICD was identified using the ESC risk score model compared with the ACCF/AHA model (82% vs 57%; p < 0.0001). Based on risk stratification criteria alone, 5 more individuals with a previously aborted SCD event would not have received an ICD with the ESC risk model compared with the ACCF/AHA risk model. In conclusion, we found that the current ESC scoring system potentially leaves more high-risk patients unprotected from sudden death in our cohort of patients.

Effectiveness of the 2014 European Society of Cardiology guideline on sudden cardiac death in hypertrophic cardiomyopathy: a systematic review and meta-analysis

“…One study included patients with a history of ventricular fibrillation and was included in the meta-analysis once the authors provided the required SCD endpoint data for patients without a history of cardiac arrest 26. Another study did not report SCD endpoints within 5 years of baseline evaluation; the authors were contacted but as data were not provided the study was excluded 22.…”

Assessing right ventricular deformation in hypertrophic cardiomyopathy patients with preserved right ventricular ejection fraction: a 3.0-T cardiovascular magnetic resonance study

“…Increase in the left ventricular out flow gradient is an important marker of development of LVOTO in patients with HCM 2 . Previous studies have shown that patients with HCM who have LVOTO are at a higher risk of sudden cardiac death or progression to severe congestive symptoms 28,29 . Studies have also shown that inter-ventricular septum is shared by two chambers and that the LV motion may affect RV motion by direct mechanical interaction 6 .…”

Increasing evidence that risk scores underperform in predicting sudden death in hypertrophic cardiomyopathy

“…On the other hand, as well noted by Choi et al 5 as well as other investigators,8 9 the ESC risk score is associated with relatively high specificity, implying that by using this prediction strategy, the number of ICD implants could be reduced in low-risk patients and ICD overuse decreased. However, the number of ICDs needed to appropriately treat one patient for VT/VF is similar between the ESC and ACC/AHA strategies and does not different from randomised clinical trials of ICDs in non-HCM diseases 5.…”

“…Furthermore, in a recent meta-analysis of nine studies (not including the recent Tufts experience) comprising close to 10 000 patients with HCM from diverse regions and ethnicities internationally, the ESC risk score was associated with sensitivities as low as 41% for predicting individual patients with HCM who suffered a SCD event 6. Therefore, the totality of these broad and expansive experiences demonstrate that the ESC risk score does not reliably identify an important and substantial subset of at-risk patients with HCM who likely would be excluded from an ICD implant recommendation and remain at risk for potentially life-threatening SCD events 5–8…”