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A Portuguese risk prediction score for sudden cardiac death in hypertrophic cardiomyopathy
Session:
CO 13 - Miocárdio e Pericárdio
Speaker:
Catarina Ruivo
Congress:
CPC 2018
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
17. Myocardial Disease
Subtheme:
17.2 Myocardial Disease – Epidemiology, Prognosis, Outcome
Session Type:
Comunicações Orais
FP Number:
---
Authors:
Catarina Ruivo; Fernando Montenegro Sá; Joana Correia; Em nome dos investigadores do Registo Nacional de Miocardiopatia Hipertrófica
Abstract
<p><strong>Background</strong><strong>:</strong> The 2014 European Society of Cardiology guidelines of hypertrophic cardiomyopathy (HCM) use a clinical risk prediction model for sudden cardiac death (SCD), based on the HCM Risk-SCD study, which did not include Portugal as a participating European country. However, this risk stratification strategy is not able to predict SCD with absolute certainty. The aim of this study was to develop a new SCD risk prediction score using the Portuguese registry and compare the accuracy of both Portuguese and European scores.</p> <p><strong>Methods and Results:</strong> The Portuguese risk prediction score was derived from a retrospective, multicenter national registry. The cohort consisted of 1022 consecutive HCM patients (mean age of 53.2±16.4 years). During a follow-up period median of 5 years, 19 patients (1.9%) died suddenly or had an appropriate implantable cardioverter defibrillator (ICD) shock. The score was developed from the entire data set using the Cox proportional hazards model. Four pre-specified predictors: (1) history of unexplainable syncope, (2) interventricular septum thickness ≥ 19 mm, (3) signs of heart failure and (4) fragmented QRS complexes (RSR´ pattern) seen on a 12-lead electrocardiogram were independently associated with SCD/appropriate ICD shock (p<0.05). These predictors were included in the final Portuguese score to estimate individual probabilities of SCD at 5 years. Receiver operating characteristic (ROC) curves and the area under curve (AUC) were calculated for the Portuguese and European scores. The AUC was 0.82 (95% CI: 0.71-0.92, p<0.001) for the Portuguese score which performed significantly better than the European score [AUC of 0.72 (95% CI: 0.53-0.90, p=0.038); ROC curves are shown in Figure 1].</p> <p><strong>Conclusion:</strong> The Portuguese risk prediction score allow to calculate the individual risk estimate, which contributes to the clinical decision-making process (ICD implantation for primary prevention of SCD). While the unexplainable syncope and the left ventricular wall thickness remain known SCD risk factors, the fragmented QRS has been recently associated with myocardial fibrosis, ischemic scaring and higher risk of SCD in HCM patients. Comparing with the European score, the Portuguese score seems to improve the accuracy of risk stratification of patients with HCM and it includes only four independent predictors of SCD, which may be easier to apply.</p>
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