Late gadolinium enhancement role in arrhythmic risk stratification of patients with LMNA cardiomyopathy: results from a long-term follow-up multicentre study. Issue 12 (30th September 2020)

• Record Type: Journal Article
• Authors: Peretto, Giovanni; Barison, Andrea; Forleo, Cinzia; Di Resta, Chiara; Esposito, Antonio; Aquaro, Giovanni Donato; Scardapane, Arnaldo; Palmisano, Anna; Emdin, Michele; Resta, Nicoletta; Santoni, Anna; Guaricci, Andrea Igoren; Santobuono, Vincenzo Ezio; Pepe, Martino; Favale, Stefano; Ferrari, Maurizio; Benedetti, Sara; Della Bella, Paolo; Sala, Simone
• Is Part Of: Europace. Volume 22:Issue 12(2020)
• Page Start: 1864
• Page End: 1872
• Publication Date: 2020-09-30
• Subjects: LMNA mutation -- Cardiomyopathy -- Sudden cardiac death -- Cardiac magnetic resonance -- Late gadolinium enhancement -- Ventricular arrhythmias -- Risk stratification; Arrhythmia -- Treatment -- Periodicals; Cardiac pacing -- Periodicals; Catheter ablation -- Periodicals; Heart -- Physiology -- Periodicals; Electrophysiology -- Periodicals; 617.4120645
• DOI: 10.1093/europace/euaa171
• Languages: English
• ISSNs: 1099-5129

Abstract

Abstract: Aims: We aimed at addressing the role of late gadolinium enhancement (LGE) in arrhythmic risk stratification of LMNA-associated cardiomyopathy (CMP). Methods and results: We present data from a multicentre national cohort of patients with LMNA mutations. Of 164 screened cases, we finally enrolled patients with baseline cardiac magnetic resonance (CMR) including LGE sequences [ n = 41, age 35 ± 17 years, 51% males, mean left ventricular ejection fraction (LVEF) by echocardiogram 56%]. The primary endpoint of the study was follow-up (FU) occurrence of malignant ventricular arrhythmias [MVA, including sustained ventricular tachycardia (VT), ventricular fibrillation, and appropriate implantable cardioverter-defibrillator (ICD) therapy]. At baseline CMR, 25 subjects (61%) had LGE, with non-ischaemic pattern in all of the cases. Overall, 23 patients (56%) underwent ICD implant. By 10 ± 3 years FU, eight patients (20%) experienced MVA, consisting of appropriate ICD shocks in all of the cases. In particular, the occurrence of MVA in LGE+ vs. LGE− groups was 8/25 vs. 0/16 ( P = 0.014). Of note, no significant differences between LGE+ and LGE− patients were found in currently recognized risk factors for sudden cardiac death (male gender, non-missense mutations, baseline LVEF <45% and non-sustained VT), all P -value >0.05. Conclusions: In LMNA-CMP patients, LGE at baseline CMR is significantly associated with MVA. In particular, as suggested by this preliminary experience, the absence of LGE allowed to rule-out MVA at 10 years mean FU.