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

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


Background: 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.

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

Methods: 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.

Results: 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.

Conclusions: 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.

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

Publication types

  • Observational Study

MeSH terms

  • Adult
  • Aged
  • Cardiomyopathy, Hypertrophic / complications
  • Cardiomyopathy, Hypertrophic / diagnostic imaging*
  • Cardiomyopathy, Hypertrophic / mortality
  • Cardiomyopathy, Hypertrophic / therapy
  • Clinical Decision-Making
  • Contrast Media / administration & dosage*
  • Death, Sudden, Cardiac / etiology*
  • Death, Sudden, Cardiac / prevention & control
  • Decision Support Techniques*
  • Defibrillators, Implantable
  • Echocardiography
  • Electric Countershock / instrumentation
  • Female
  • Humans
  • Magnetic Resonance Imaging, Cine*
  • Male
  • Middle Aged
  • Organometallic Compounds / administration & dosage*
  • Predictive Value of Tests
  • Prognosis
  • Risk Assessment
  • Risk Factors


  • Contrast Media
  • Organometallic Compounds
  • gadobutrol