Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology · 2026
Revisiting risk stratification in hypertrophic cardiomyopathy after the recent guidelines - Late gadolinium enhancement remains tough to beat.
СтатьяНаблюдательное исследованиеМногоцентровое исследованиеНазвание и аннотация: Перевод в очереди
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Amador Rita, Pereira Joana, Freitas Pedro, Lima Rita, Paiva Mariana, Gomes Daniel, Eduardo Rochite Carlos, Arteaga Edmundo, Carmo Pedro, Cavaco Diogo, Adragão Pedro, Ferreira António
Аннотация
INTRODUCTION AND OBJECTIVES: Recommendations for implantable cardioverter-defibrillator (ICD) implantation to prevent sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM) have evolved over time, with late gadolinium enhancement (LGE) emerging as an important additional risk marker. Our study aimed to evaluate changes in the accuracy and discriminative performance of international guidelines for ICD recommendations and to determine whether LGE can improve this risk stratification.
METHODS: We conducted a multicenter retrospective observational study of HCM patients who underwent cardiac magnetic resonance (CMR) imaging for diagnosis or risk assessment. ICD eligibility was determined based on the ESC guidelines (2014, 2022, 2023) and the American College of Cardiology (ACC) guidelines (2011, 2020, 2024). Our primary endpoint was a composite of sudden cardiac death (SCD), appropriate ICD discharge, or sustained ventricular tachycardia (VT).
RESULTS: A total of 531 patients (median age 49 years; 57% male) were included. During a median follow-up of 49 months, twenty-eight events occurred (15 SCDs, six appropriate ICD discharges, seven sustained VTs). Over time, both European and American guidelines demonstrated improved sensitivity, positive predictive value, and discriminatory ability, while maintaining a high negative predictive value. The agreement between the recent ESC and ACC guidelines was moderate (κ≈0.6, p<0.001). Late gadolinium enhancement (LGE) was present in 80% of patients and LGE% independently predicted arrhythmic events (adjusted hazard ratio 1.09 per 1% increase; p<0.001). A burden ≥8% was the best predictor of events, while the absence of LGE identified patients without arrhythmic outcomes.
CONCLUSIONS: The extent of LGE burden improves risk stratification for SCD, with the complete absence of LGE indicating a very low-risk subgroup. LGE thresholds should be individualized by integrating imaging results, clinical factors and the patient's context.
METHODS: We conducted a multicenter retrospective observational study of HCM patients who underwent cardiac magnetic resonance (CMR) imaging for diagnosis or risk assessment. ICD eligibility was determined based on the ESC guidelines (2014, 2022, 2023) and the American College of Cardiology (ACC) guidelines (2011, 2020, 2024). Our primary endpoint was a composite of sudden cardiac death (SCD), appropriate ICD discharge, or sustained ventricular tachycardia (VT).
RESULTS: A total of 531 patients (median age 49 years; 57% male) were included. During a median follow-up of 49 months, twenty-eight events occurred (15 SCDs, six appropriate ICD discharges, seven sustained VTs). Over time, both European and American guidelines demonstrated improved sensitivity, positive predictive value, and discriminatory ability, while maintaining a high negative predictive value. The agreement between the recent ESC and ACC guidelines was moderate (κ≈0.6, p<0.001). Late gadolinium enhancement (LGE) was present in 80% of patients and LGE% independently predicted arrhythmic events (adjusted hazard ratio 1.09 per 1% increase; p<0.001). A burden ≥8% was the best predictor of events, while the absence of LGE identified patients without arrhythmic outcomes.
CONCLUSIONS: The extent of LGE burden improves risk stratification for SCD, with the complete absence of LGE indicating a very low-risk subgroup. LGE thresholds should be individualized by integrating imaging results, clinical factors and the patient's context.