SPC365

Sudden Cardiac Death HCM Risk Scores in Apical Hypertrophic Cardiomyopathy: an unmet need in clinical practice

Session: SESSÃO DE POSTERS 44 - ARRITMIAS VENTRICULARES
Speaker: Ana Rita Bello

Congress: CPC 2025
Topic: F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme: 17. Myocardial Disease
Subtheme: 17.2 Myocardial Disease – Epidemiology, Prognosis, Outcome
Session Type: Cartazes
FP Number: ---
Authors: Ana Rita Bello; Maria Rita Lima; Rita Almeida Carvalho; Débora Correia; Joana Certo Pereira; Rita Amador; Gonçalo Cunha; Catarina Brízido; Sérgio Maltês; Bruno Rocha; Carlos Aguiar

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif"><u><span style="font-size:11.0pt">Introduction</span></u><span style="font-size:11.0pt">: </span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif"><span style="font-size:11.0pt">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.</span></span></span></p>

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<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif"><u><span style="font-size:11.0pt">Methods</span></u><span style="font-size:11.0pt">: </span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif"><span style="font-size:11.0pt">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.</span></span></span></p>

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<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif"><u><span style="font-size:11.0pt">Results</span></u><span style="font-size:11.0pt">: </span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif"><span style="font-size:11.0pt">Among 579 patients with hypertrophic cardiomyopathy, 105 (18%) had aHCM and were enrolled [mean age 69&plusmn;14 years, 64% female, maximal left ventricular thickness 16 (14-19) mm, mean LAVI 44 (35-46) mL/m<sup>2</sup>; 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. </span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif">&nbsp;</span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif"><span style="font-size:11.0pt">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/m<sup>2</sup>; 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 (&lt;4%), intermediate (4-6%) and high-risk (&gt;6%), or as per the aHCM-specific score (0, 0-2 and </span><span style="font-size:11.0pt"><span style="font-family:&quot;Arial&quot;,sans-serif">&ge;</span></span><span style="font-size:11.0pt">3, respectively), did not improve discrimination.</span></span></span></p>

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<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif"><u><span style="font-size:11.0pt">Conclusions</span></u><span style="font-size:11.0pt">: </span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif"><span style="font-size:11.0pt">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.</span></span></span></p>