Prognostic factors in hypertrophic cardiomyopathy in children: An MRI based study. (1st October 2022)

• Record Type: Journal Article
• Authors: Ali, Lamia Ait; Marrone, Chiara; Martins, Duarte Saraiva; Khraiche, Diala; Festa, Pierluigi; Martini, Nicola; Santoro, Giuseppe; Todiere, Giancarlo; Panaioli, Elena; Bonnet, Damien; Boddaert, Nathalie; Aquaro, Giovanni Donato; Raimondi, Francesca
• Is Part Of: International journal of cardiology. Volume 364(2022)
• Page Start: 141
• Page End: 147
• Publication Date: 2022-10-01
• Subjects: Hypertrophic cardiomyopathy -- Cardiac magnetic resonance -- Sudden cardiac death -- Children -- Late gadolinium enhancement -- Fibrosis; HCM Hypertrophic cardiomyopathy -- SCD sudden cardiac death -- CHF congestive heart failure -- CMR cardiac magnetic resonance -- LGE late gadolinium enhancement technique -- LV left ventricle -- BSA body surface área -- LVOT left ventricular outflow tract -- ICD implantable cardioverter defibrillator -- AUC area under the curve -- LVEF left ventricular ejection fraction -- LVEDV left ventricular end diastolic volume -- LVESV left ventricular end systolic volume -- LVH left ventricular hypertrophy; Cardiology -- Periodicals; Electronic journals; 616.12
• DOI: 10.1016/j.ijcard.2022.06.043
• Languages: English
• ISSNs: 0167-5273

Abstract

Abstract: Background: Clinical and prognostic role of cardiac magnetic resonance (CMR) in adult population with hypertrophic cardiomyopathy (HCM) have been largely assessed. We sought to investigate the role of CMR for predicting cardiovascular events in children with HCM. Methods: CMR was performed in 116 patients with HCM (37 sarcomeric mutations, 31 other mutations, mean age 10.4 ± 4.3 yrs). CMR protocol included cine imaging for evaluation of morphology and function and late gadolinium enhancement (LGE). Hard cardiac events (sustained VT, resuscitated cardiac arrest, sudden cardiac death, end-stage heart failure, heart transplant and appropriate ICD intervention) were recorded through a median follow-up of 4 (1–7) years. Results: During follow-up 21 heart cardiac events occurred. At maximal-rank statistic the optimal cut-point for LGE extent for predicting events was ≥2%. Syncope, non-sustained ventricular tachycardia (NSVT) and LGE extent ≥2% were independent predictors of events. At Harrel's C statistic combination of LGE extent ≥2% and syncope was the strongest model for predicting events. HR of patients with LGE extent ≥2% and no history of syncope was 3.6 (1.1–12.2) that increased to 37.6 (5.4–161) in those with LGE extent ≥2% and syncope. The median time dependent AUC of LGE extent (0.88, 95% CI 0.86–0.89) was significantly higher than that of syncope (0.63, 95% CI 0.61–0.66, p < 0.0001) and NSVT (0.52, 95% CI 0.50–0.53, p < 0.0001). Conclusions: In children with HCM, LGE and syncope were independent predictors of hard cardiac events at follow-up. Highlights: We present the results of a multicentric pediatric cohort of HCM submitted to CMR with a median follow-up of 4 years. Syncope, non-sustained ventricular tachycardia (NSVT) and LGE extent ≥2% were independent predictors of events. Combination of LGE and syncope was the best model for predicting events. LGE extent alone was the strongest predictor.