Open Access

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

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Date: 30 September 2020
Journal: EP Europace Journal , Volume 22 , Issue 12 , Pages 1864 - 1872
Authors: G. Peretto , A. Barison , C. Forleo , C. Di Resta , A. Esposito , G. Aquaro , A. Scardapane , A. Palmisano , M. Emdin , N. Resta , A. Santoni , A. Guaricci , V. Santobuono , M. Pepe , S. Favale , M. Ferrari , S. Benedetti , P. Della Bella , S. Sala

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AbstractAims

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.

About the contributors

Giovanni Peretto

Role: Author

Andrea Barison

Role: Author

Cinzia Forleo

Role: Author