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ESC sudden-death risk model in hypertrophic cardiomyopathy: Incremental value of quantitative contrast-enhanced CMR
Rocio Hinojar1,2, José Luis Zamorano1,2,3, Ariana Gonzalez Gómez1
1Department of Cardiology, University Hospital Ramón y Cajal, Madrid, Spain.
Insights
Myocardial fibrosis, measured by late gadolinium enhancement (LGE) extension, correlates with sudden cardiac death (SCD) risk in hypertrophic cardiomyopathy (HCM). This finding aids in implantable cardioverter-defibrillator (ICD) decisions for HCM patients.
Area of Science:
- Cardiology
- Medical Imaging
- Genetics
Background:
- Hypertrophic cardiomyopathy (HCM) is a primary cause of sudden cardiac death (SCD) in young individuals.
- Current risk stratification methods for SCD in HCM patients are insufficient.
- Myocardial fibrosis, indicated by late gadolinium enhancement (LGE), is a newly identified risk factor for SCD.
Purpose of the Study:
- To investigate the relationship between the extent of myocardial fibrosis (LGE) and a novel SCD risk prediction model in HCM patients.
- To assess the additional value of LGE in refining SCD risk stratification and guiding implantable cardioverter-defibrillator (ICD) decisions.
Main Methods:
- Seventy-seven HCM patients underwent clinical evaluation, echocardiography, and cardiac magnetic resonance imaging.
- Late gadolinium enhancement (LGE) extension was quantified using cardiac MRI.
- A novel SCD risk prediction model was applied to calculate 5-year SCD risk.
Main Results:
- A significant positive correlation was observed between LGE extension and SCD risk prediction (r = 0.7, P < 0.001).
- Distinct LGE extents were found across low- (5% ± 6%), intermediate- (18% ± 9%), and high-risk (17% ± 4%) groups (P < 0.001).
- Extensive LGE was present in 6% of the low-risk, 62% of the intermediate-risk, and 86% of the high-risk groups.
Conclusions:
- LGE extension is consistent with the novel SCD risk model, effectively differentiating risk groups.
- Quantification of LGE provides valuable supplementary information for improved SCD risk stratification in HCM.
- LGE assessment can aid in making more informed decisions regarding ICD implantation in HCM patients.
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.
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