What does this research mean for the field?

In a cohort of patients with apical hypertrophic cardiomyopathy, over 10% experienced an event of all-cause death or ICD shock, with poor predictive performance of risk scores. Novelty: ClaimNovelty.CONFIRMATORY. Consensus alignment: ConsensusAlignment.NEUTRAL.

What question did this study set out to answer?

Evaluate the predictive value of the HCM Risk-SCD score and aHCM-specific risk score in apical hypertrophic cardiomyopathy.

February 8, 2026

Sudden cardiac death HCM risk scores in apical hypertrophic cardiomyopathy: an unmet need in clinical practice

Key Result

HCM Risk-SCD (AUC 0.39) and aHCM-specific scores (AUC 0.53) poorly predicted death or ICD shocks in apical HCM over 58 months follow-up.

Key Points

Evaluate the predictive value of the HCM Risk-SCD score and aHCM-specific risk score in apical hypertrophic cardiomyopathy.
Retrospective study of patients diagnosed with apical hypertrophic cardiomyopathy.
Data collected at baseline for risk score calculation.
Used HCM Risk-SCD score and aHCM-specific score based on various clinical variables.
Follow-up during which primary endpoint events were recorded.
105 out of 579 patients were diagnosed with aHCM and enrolled.
During follow-up, 15 patients experienced an event defined as the primary endpoint.
Older age and left atrial volume index were predictive of the primary endpoint.
The HCM Risk-SCD and aHCM-specific scores performed poorly, indicating inadequate risk stratification.

Structured PICO

Do the ESC HCM Risk-SCD score and a novel aHCM-specific risk score accurately predict the composite of all-cause death, appropriate ICD shock, or heart transplantation in patients with apical hypertrophic cardiomyopathy?

Population

105 patients with apical hypertrophic cardiomyopathy (aHCM), mean age 69±14 years, 64% female.

Intervention

ESC HCM Risk-SCD score and a novel aHCM-specific risk score

Outcome

Composite of all-cause death, appropriate defibrillator shock (implantable cardioverter-defibrillator [ICD] shocks or resuscitated SCD), or heart transplantationcomposite

Current risk stratification tools, including the ESC HCM Risk-SCD score and a novel aHCM-specific score, perform poorly in predicting adverse outcomes in patients with apical hypertrophic cardiomyopathy.

Main Result

Absolute Event Rate: 0% vs 0%

Abstract

Abstract Introduction The concept that apical hypertrophic cardiomyopathy (aHCM) is associated with a lower risk of sudden cardiac death (SCD) has often been challenged. Prediction tools have yet to be validated in aHCM. Accordingly, we aimed to evaluate the predictive value of HCM Risk-SCD score (ESC) and a new aHCM-specific risk score in our cohort of aHCM. Methods Retrospective study of consecutive patients diagnosed with aHCM, with at least a yearly follow-up in our center. Data of interest to calculate risk scores was collected at baseline, considered as the date of aHCM imaging diagnosis. The ESC HCM Risk-SCD score was calculated using the 7-variable online tool. The aHCM-specific score (JACC Advances, 2024), ranging from 0-8 points, comprises 5 variables: age, creatinine, left atrial volume index (LAVI), right ventricular systolic pressure and apical aneurysm (any volume). The primary endpoint was a composite of all-cause death, appropriate defibrillator shock [implantable cardioverter-defibrillator (ICD) shocks or resuscitated SCD), or heart transplantation. Results Among 579 patients with hypertrophic cardiomyopathy, 105 (18%) had aHCM and were enrolled [mean age 69±14 years, 64% female, maximal left ventricular thickness 16 (14-19) mm, median LAVI 44 (35-46) mL/m2; 8, 5, 25 and 6 patients with a SCD family history, non-sustained VT, unexplained syncope and apical aneurysm, respectively). An ICD was implanted as primary prevention of SCD in 17 (16%) patients. During a median follow-up of 58 (26-96) months, 15 patients met an event of the primary endpoint (12 deaths, 3 ICD shocks). In univariate analysis, older age (HR 1.10 per year, CI 1.02-1.14; p=0.002) and LAVI (HR 1.04 per mL/m2; CI 1.04-1.07; p=0.004) were the only variables imputed into one of the scores predicting the primary endpoint. The HCM Risk-SCD (AUC 0.39, p=0.377) and the novel aHCM-specific score (AUC 0.53; p=0.759) performed poorly to predict the primary endpoint. The categorization of patients, as per the HCM Risk-SCD score as low (4%), intermediate (4-6%) and high-risk (6%), or as per the aHCM-specific score (0, 0-2 and ≥3, respectively), did not improve discrimination. Conclusions In our cohort of patients with aHCM, more than 1 in every 10 patients died or had an ICD shock. The HCM Risk-SCD and the novel aHCM-specific score were poor discriminators for the likelihood of the primary endpoint. This study underscores the importance of further research to improve risk stratification in aHCM.

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