LETTER TO THE EDITOR
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doi:10.1093/europace/euaa374
Lamin cardiomyopathy risk
stratification: Authors’ reply
I would like to thank Dr Barriales-Villa et al. for
their interest in our work. There are no doubts
that arrhythmic risk stratification in LMNA car-
diomyopathy is still challenging. The prognostic
impact of reduced left ventricular ejection frac-
tion (LVEF) and non-sustained ventricular tachy-
cardias (NSVT) has been confirmed by several
studies, including the REDLAMINA cited by the
authors.1,2 Conversely, the evidence is weaker
for other traditional risk factors like male gen-
der and non-missense mutations.1,2 Although
efforts have been made to integrate all the
known prognostic determinants under a single
multi-parametric score,3 there is still no recog-
nized role for cardiac magnetic resonance in ar-
rhythmic risk stratification. To our knowledge,
we published the first study in LMNA cardiomy-
opathy, showing an association between late
gadolinium enhancement (LGE) and the occur-
rence of major ventricular arrhythmias during
follow-up.4 In particular, the high negative pre-
dictive value documented for LGE should be
Figure 1. Multi-parametric arrhythmic risk stratification in LMNA cardiomyopathy. LMNA, lamin A/C gene; SCD, sudden cardiac death.
Published on behalf of the European Society of Cardiology. All rights reserved. V
contextualized to the study population, charac-
terized by a low baseline risk profile.4 Results
should not be improperly generalized. For in-
stance, patients with reduced LVEF and docu-
mented NSVT might be at high risk of
malignant ventricular arrhythmias independently
of LGE.1 Instead, LGE may be particularly help-
ful to improve risk stratification in otherwise
low-risk subjects, as those with early-stage car-
diomyopathy and preserved systolic function.
Consistently, it has been recently suggested to
consider LVEF as a continuous risk-modifier var-
iable,3 and to shift the prognostic cutoff from
the current 45% to an earlier one, as high as
50%.2,5 Within an updated multi-parametric
model, LGE is not expected to replace the tra-
ditional risk factors, but simply to play as an ad-
ditional prognostic marker (Figure 1). A further
contribution to risk stratification may come
from extra-cardiac laminopathies, as already
shown in patients with associated neuromuscu-
lar manifestations.5 Since the disease is rare and
complex, multicentre and multidisciplinary stud-
ies are strongly advocated to improve patient
selection for primary prevention cardioverter-
defibrillator implant.
Conflict of interest: none declared.
C The Author(s) 2020. For permissions, please email: journals.permissions@oup.com.
References
1. Barriales-Villa R, Ochoa JP, Larra~ naga-Moreira JM.
Lamin cardiomyopathy risk stratification. Europace
2020.
2. Peretto G, Sala S, Benedetti S, Di Resta C, Gigli L,
Ferrari M et al. Updated clinical overview on cardiac
laminopathies: an electrical and mechanical disease.
Nucleus 2018;9:380–91.
3. Wahbi K, Ben Yaou R, Gandjbakhch E, Anselme F,
Gossios T, Lakdawala NK et al. Development and vali-
dation of a new risk prediction score for life-
threatening ventricular tachyarrhythmias in laminopa-
thies. Circulation 2019;140:293–302.
4. Peretto G, Barison A, Forleo C, Di Resta C, Esposito
A, Aquaro GD et al. Late gadolinium enhancement role
in arrhythmic risk stratification of patients with LMNA
cardiomyopathy: results from a long-term follow-up
multicentre study. Europace 2020; doi:
10.1093/europace/euaa171.
5. Peretto G, Di Resta C, Perversi J, Forleo C, Maggi L,
Politano L et al.; on behalf of the Italian Network for
Laminopathies (NIL). Cardiac and neuromuscular fea-
tures of patients with LMNA-related cardiomyopathy.
Ann Intern Med 2019;171:458.
Giovanni Peretto1,2*
1
Department of Arrhythmology and Cardiac
Electrophysiology, IRCCS San Raffaele Hospital,
Milan, Italy; and 2School of Medicine, San Raffaele
Vita-Salute University, Milan, Italy
Corresponding author. Tel: þ39 02 2643 7484;
fax: þ39 02 2643 7326. E-mail address:
peretto.giovanni@hsr.it