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

• Published in: Clinical cardiology (Mahwah, N.J.) Vol. 40; no. 10; pp. 853 - 860
• Main Authors: Hinojar, Rocio, Zamorano, José Luis, Gonzalez Gómez, Ariana, Plaza Martin, Maria, Esteban, Amparo, Rincón, Luis Miguel, Portugal, Juan Carlos, Jimenez Nácher, José Julio, Fernández‐Golfín, Covadonga
• Format: Journal Article
• Language: English
• Published: New York Wiley Periodicals, Inc 01-10-2017; John Wiley & Sons, Inc
• Subjects: Adult; Aged; Cardiac Magnetic Resonance; Cardiomyopathy; Cardiomyopathy, Hypertrophic - complications; Cardiomyopathy, Hypertrophic - diagnostic imaging; Cardiomyopathy, Hypertrophic - mortality; Cardiomyopathy, Hypertrophic - therapy; Clinical Decision-Making; Clinical Investigations; Contrast Media - administration & dosage; Death, Sudden, Cardiac - etiology; Death, Sudden, Cardiac - prevention & control; Decision Support Techniques; Defibrillators; Defibrillators, Implantable; Echocardiography; Electric Countershock - instrumentation; Female; Humans; Hypertrophic Cardiomyopathy; Late Gadolinium Enhancement; Magnetic Resonance Imaging, Cine; Male; Middle Aged; Organometallic Compounds - administration & dosage; Predictive Value of Tests; Prognosis; Risk Assessment; Risk Factors; Risk Stratification; Sudden Cardiac Death; Risk Stratification; Sudden Cardiac Death; Cardiac Magnetic Resonance; Late Gadolinium Enhancement; Hypertrophic Cardiomyopathy
• ISSN: 0160-9289; 1932-8737
• DOI: 10.1002/clc.22735

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.