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Clin Cardiol. 2017 Oct;40(10):853-860. doi: 10.1002/clc.22735. Epub 2017 Jun 14.

ESC sudden-death risk model in hypertrophic cardiomyopathy: Incremental value of quantitative contrast-enhanced CMR in intermediate-risk patients.

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Department of Cardiology, University Hospital Ramón y Cajal, Madrid, Spain.
Department of Medicine, University of Alcalá, Madrid, Spain.
CIBERCV, Instituto de Salud Carlos III (ISCIII), Spain.
Department of Radiology, University Hospital Ramón y Cajal, Madrid, Spain.
Department of Cardiology, University Hospital Dr. Negrín, Gran Canaria, Spain.



Hypertrophic cardiomyopathy (HCM) remains the most common cause of sudden cardiac death (SCD) in the young; however, current strategies do not identify all HCM patients at risk. A novel validated algorithm was proposed by the last European Society of Cardiology guidelines to guide implantable cardioverter-defibrillator (ICD) therapy. Recently, extensive myocardial fibrosis was independently associated with increased risk of SCD events. This study aimed to establish the relation between myocardial fibrosis (late gadolinium enhancement [LGE] extension) and the novel SCD risk-prediction model in a real population of HCM to evaluate its potential additional value in the different risk groups.


There is a significant association between LGE extension and the novel SCD risk calculator that may help conflicting ICD decisions.


Seventy-seven patients with HCM underwent routine clinical evaluation, echocardiography, and cardiac magnetic resonance study. Their SCD risk at 5 years was calculated using the new model.


Extension of LGE positively correlated with SCD risk prediction (r = 0.7, P < 0.001). Low-, intermediate-, and high-risk groups according to the model showed significantly different extent of LGE (5% ± 6% vs 18% ± 9% vs 17% ± 4%; P < 0.001). Four patients (6%) in the low-risk group and 5 (62%) in the intermediate-risk group showed extensive areas of LGE. All patients except 1 (86%) at highest risk (n = 6) showed extensive areas of LGE.


LGE extension is concordant with the novel SCD-risk model defining low- and high-risk groups; it may provide additional information, allowing better discrimination to support implantable cardioverter-defibrillator decision. LGE quantification holds promise for SCD stratification in HCM.


Cardiac Magnetic Resonance; Hypertrophic Cardiomyopathy; Late Gadolinium Enhancement; Risk Stratification; Sudden Cardiac Death

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