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Multicenter Study
. 2024 May 14;83(19):1841-1851.
doi: 10.1016/j.jacc.2024.02.041.

Magnetic Resonance Imaging Characterization and Clinical Outcomes of Dilated and Arrhythmogenic Left Ventricular Cardiomyopathies

Matteo Castrichini  1 , Antonio De Luca  2 , Giulia De Angelis  2 , Raquel Neves  3 , Alessia Paldino  2 , Matteo Dal Ferro  2 , Giulia Barbati  4 , Kristen Medo  5 , Andrea Barison  6 , Chrysanthos Grigoratos  6 , Marta Gigli  2 , Davide Stolfo  7 , Francesca Brun  2 , Daniel W Groves  5 , Robert Quaife  5 , Ramone Eldemire  5 , Sharon Graw  5 , Jeffrey Addison  5 , Giancarlo Todiere  6 , Ignazio Alessio Gueli  6 , Nicoletta Botto  6 , Michele Emdin  8 , Giovanni Donato Aquaro  9 , Ramin Garmany  3 , Naveen L Pereira  3 , Matthew R G Taylor  5 , Michael J Ackerman  3 , Gianfranco Sinagra  2 , Luisa Mestroni  5 , John R Giudicessi  3 , Marco Merlo  10
Affiliations
Multicenter Study

Magnetic Resonance Imaging Characterization and Clinical Outcomes of Dilated and Arrhythmogenic Left Ventricular Cardiomyopathies

Matteo Castrichini et al. J Am Coll Cardiol. .

Abstract

Background: Nondilated left ventricular cardiomyopathy (NDLVC) has been recently differentiated from dilated cardiomyopathy (DCM). A comprehensive characterization of these 2 entities using cardiac magnetic resonance (CMR) and genetic testing has never been performed.

Objectives: This study sought to provide a thorough characterization and assess clinical outcomes in a large multicenter cohort of patients with DCM and NDLVC.

Methods: A total of 462 patients with DCM (227) or NDLVC (235) with CMR data from 4 different referral centers were retrospectively analyzed. The study endpoint was a composite of sudden cardiac death or major ventricular arrhythmias.

Results: In comparison to DCM, NDLVC had a higher prevalence of pathogenic or likely pathogenic variants of arrhythmogenic genes (40% vs 23%; P < 0.001), higher left ventricular (LV) systolic function (LV ejection fraction: 51% ± 12% vs 36% ± 15%; P < 0.001) and higher prevalence of free-wall late gadolinium enhancement (LGE) (27% vs 14%; P < 0.001). Conversely, DCM showed higher prevalence of pathogenic or likely pathogenic variants of nonarrhythmogenic genes (23% vs 12%; P = 0.002) and septal LGE (45% vs 32%; P = 0.004). Over a median follow-up of 81 months (Q1-Q3: 40-132 months), the study outcome occurred in 98 (21%) patients. LGE with septal location (HR: 1.929; 95% CI: 1.033-3.601; P = 0.039) was independently associated with the risk of sudden cardiac death or major ventricular arrhythmias together with LV dilatation, older age, advanced NYHA functional class, frequent ventricular ectopic activity, and nonsustained ventricular tachycardia.

Conclusions: In a multicenter cohort of patients with DCM and NDLVC, septal LGE together with LV dilatation, age, advanced disease, and frequent and repetitive ventricular arrhythmias were powerful predictors of major arrhythmic events.

Keywords: LGE; SCD; arrhythmias; dilated cardiomyopathy; nondilated left ventricular cardiomyopathy.

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Conflict of interest statement

Funding Support and Author Disclosures This work was supported by the Paul and Ruby Tsai Family Hypertrophic Cardiomyopathy Career Development Award (Dr Giudicessi); the National Institutes of Health/National Heart, Lung, and Blood Institute R01HL147064 and R01HL164634 (Drs Mestroni and Taylor). The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Figures

FIGURE 1
FIGURE 1. Cumulative Incidence of the Composite Endpoint (SCD/MVA) According to LGE
(A) Patients with LGE were at higher risk of events compared to those without LGE (P < 0.001). (B) Patients with any septal LGE showed a greater risk of events compared to those with free-wall LGE (P = 0.025) and without LGE (P < 0.001). (C) No significant difference between patients with isolated or combined septal LGE (P = 0.13). No corrections for multiple testing were applied. CIF = cumulative incidence function; LGE = late gadolinium enhancement; MVA = major ventricular arrhythmias; SCD = sudden cardiac death.
FIGURE 2
FIGURE 2. Cumulative Incidence of Composite Endpoint (SCD/MVA) Comparing DCM vs NDLVC
A worse outcome was observed for DCM patients when compared to NDLVC patients (P = 0.0003). DCM = dilated cardiomyopathy; NDLVC = nondilated left ventricular cardiomyopathy; other abbreviations as in Figure 1.
FIGURE 3
FIGURE 3. Cumulative Incidence of SCD/MVA According to Phenotype and LGE Positivity
DCM patients with LGE showed the highest risk of arrhythmic events, whereas NDLVC patients without LGE showed the lowest risk. No corrections for multiple testing were applied. Abbreviations as in Figure 1 and 2.
CENTRAL ILLUSTRATION
CENTRAL ILLUSTRATION. Characterization and Clinical Outcomes of Dilated Cardiomyopathy and Nondilated Left Ventricular Cardiomyopathy
In a multicenter cohort of patients with dilated and nondilated LV cardiomyopathies, LV dilatation as well as the presence and septal location of LGE were independent predictors of major arrhythmic events. Patients with LV dilatation and LGE showed the greater risk of events, whereas those with NDLVC without LGE showed the lowest risk. CMR = cardiac magnetic resonance; DCM = dilated cardiomyopathy; LGE = late gadolinium enhancement; LV = left ventricular; MVA = major ventricular arrhythmias; NDLVC = nondilated left ventricular cardiomyopathy; P/LP = pathogenic or likely pathogenic; SCD = sudden cardiac death.
See this image and copyright information in PMC

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