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

Abstract

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.

Electrocardiography scar quantification correlates with scar size of hypertrophic cardiomyopathy seen by multidetector computed tomography

“…In the current study, patients with other causes of fibrosis (eg, coronary disease or a previous myectomy) were excluded. HCM is generally a low‐event‐rate disease; therefore, demonstrating novel risk markers is challenging . We included patients with a high risk of arrhythmic events (ie, ≥2 traditional SCD risk factors) with the aim of increasing the prevalence of fibrosis…”

Clinical and echocardiographic parameters as risk factors for atrial fibrillation in patients with hypertrophic cardiomyopathy

“…In our cohort, almost 69.8% HCM patients had a positive family history, while 32.6% had a family history of SCD. This is admittedly more than in most other cohorts . A reason for this may be the fact that we are a referral center, actively screening even distantly living families of HCM patients referred to us.…”

CMR-Based Risk Stratification of Sudden Cardiac Death and Use of Implantable Cardioverter–Defibrillator in Non-Ischemic Cardiomyopathy

“…The ESC 2014 guidelines stated that although LGE imaging might be useful in predicting cardiovascular mortality, the data did not allow using LGE in standardized risk prediction for SCD [177]. In a study of Hinojar et al, the extent of LGE was an independent predictor between ESC low-and high-risk groups with nearly all of the high-risk patients showing an LGE extent > 15%, but they did not support distinguishing intermediate from high-risk patients [191].…”