Late gadolinium enhancement role in arrhythmic risk stratification of patients with LMNA cardiomyopathy: Results from a long-term follow-up multicentre study

Italian Network for Laminopathies (NIL)

Research output: Contribution to journalArticlepeer-review

Abstract

Aims We aimed at addressing the role of late gadolinium enhancement (LGE) in arrhythmic risk stratification of LMNA-associated cardiomyopathy (CMP). Methods and We present data from a multicentre national cohort of patients with LMNA mutations. Of 164 screened cases, we results 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 LGEþ vs. LGE- groups was 8/ 25 vs. 0/16 (P = 0.014). Of note, no significant differences between LGEþ 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.

Original languageEnglish
Pages (from-to)1864-1872
Number of pages9
JournalEuropace
Volume22
Issue number12
DOIs
Publication statusPublished - Dec 1 2020

Keywords

  • Cardiac magnetic resonance
  • Cardiomyopathy
  • Late gadolinium enhancement
  • LMNA mutation
  • Risk stratification
  • Sudden cardiac death
  • Ventricular arrhythmias

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

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