Late gadolinium enhanced cardiovascular magnetic resonance of lamin A/C gene mutation related dilated cardiomyopathy

Holmström, Miia; Kivistö, Sari; Heliö, Tiina; Jurkko, Raija; Kaartinen, Maija; Antila, Margareta; Reissell, Eeva; Kuusisto, Johanna; Kärkkäinen, Satu; Peuhkurinen, Keijo; Koikkalainen, Juha; Lötjönen, Jyrki; Lauerma, Kirsi-Maria Susanna

doi:10.1186/1532-429x-13-30

Cited by 99 publications

(76 citation statements)

References 29 publications

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“…15 In contrast, a previous MRI study in patients with the lamin A/C mutation and DCM described a predominance of basal and midseptal LV involvement, correlated with conduction abnormalities. 16 The higher proportion of anteroseptal patients fulfilling diagnostic criteria for DCM in our data may relate to more extensive endocardial scarring …”

Section: Discussionmentioning

confidence: 89%

Catheter Ablation of Ventricular Arrhythmia in Nonischemic Cardiomyopathy

Olóriz

Silberbauer

Maccabelli

et al. 2014

Circ: Arrhythmia and Electrophysiology

158

104

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Background-The aim was to relate distinct scar distributions found in nonischemic cardiomyopathy with ventricular tachycardia (VT) morphology, late potential distribution, ablation strategy, and outcome. Methods and Results-Eighty-seven patients underwent catheter ablation for drug-refractory VT. Based on endocardial unipolar voltage, 44 were classified as predominantly anteroseptal and 43 as inferolateral. Anteroseptal patients more frequently fulfilled diagnostic criteria for dilated cardiomyopathy (64% versus 36%), associated with more extensive endocardial unipolar scar (41 [22-83] versus 9 [1-29] cm 2 ; P<0.001). Left inferior VT axis was predictive of anteroseptal scar (positive predictive value, 100%) and right superior axis for inferolateral (positive predictive value, 89%). Late potentials were infrequent in the anteroseptal group (11% versus 74%; P<0.001). Epicardial late potentials were common in the inferolateral group (81% versus 4%; P<0.001) and correlated with VT termination sites (κ=0.667; P=0.014), whereas no anteroseptal patient had an epicardial VT termination (P<0.001). VT recurred in 44 patients (51%) during a median follow-up of 1.5 years. Anteroseptal scar was associated with higher VT recurrence (74% versus 25%; log-rank P<0.001) and redo procedure rates (59% versus 7%; log-rank P<0.001). After multivariable analysis, clinical predictors of VT recurrence were electrical storm (hazard ratio, 3.211; P=0.001) and New York Heart Association class (hazard ratio, 1.608; P=0.018); the only procedural predictor of VT recurrence was anteroseptal scar pattern (hazard ratio, 5.547; P<0.001). Conclusions-Unipolar low-voltage distribution in nonischemic cardiomyopathy allows categorization of scar pattern as inferolateral, often requiring epicardial ablation mainly based on late potentials, and anteroseptal, which frequently involves an intramural septal substrate, leading to a higher VT recurrence. (Circ Arrhythm Electrophysiol. 2014;7:414-423.)

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Section: Discussionmentioning

confidence: 89%

Catheter Ablation of Ventricular Arrhythmia in Nonischemic Cardiomyopathy

Olóriz

Silberbauer

Maccabelli

et al. 2014

Circ: Arrhythmia and Electrophysiology

158

104

View full text Add to dashboard Cite

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“…Hearts from mice with an Lmna mutation that causes cardiomyopathy have marked fibrosis and evidence of enhanced TGF- and CTGF activity (42,43). Hearts of human subjects with cardiomyopathy caused by LMNA mutations similarly have prominent fibrosis (44,45). Adventitial fibrosis in coronary arteries has been reported in children with Hutchinson-Gilford progeria syndrome, which is caused by LMNA mutation (46).…”

Section: Ecm Is Altered In Dermal Fibroblasts From Patients With Lmnamentioning

confidence: 99%

Extracellular matrix remodeling and transforming growth factor-β signaling abnormalities induced by lamin A/C variants that cause lipodystrophy

Dour

Béréziat

et al. 2017

Journal of Lipid Research

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“…However, typical septal and inferolateral scars have been described in various diseases, such as myocarditis, 11 arrhythmogenic RV cardiomyopathy/dysplasia with LV involvement, 12 laminopathy, 13 and Anderson-Fabry disease.…”

Section: Typical Arrhythmogenic Substratesmentioning

confidence: 99%

Contrast-Enhanced MRI–Derived Scar Patterns and Associated Ventricular Tachycardias in Nonischemic Cardiomyopathy

Piers

Tao

Taxis

et al. 2013

Circ: Arrhythmia and Electrophysiology

159

121

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Background-There are limited data on typical arrhythmogenic substrates and associated ventricular tachycardias (VT) in patients with nonischemic cardiomyopathy. The substrate location may have implications for the ablation strategy. Methods and Results-Nineteen consecutive patients with nonischemic cardiomyopathy (age 58±14 years, 79% men, left ventricular ejection fraction 41±11%) who underwent contrast-enhanced MRI and VT ablation were included. On the basis of 3-dimensional contrast-enhanced MRI-derived scar reconstructions, 8 patients (42%) had predominant basal anteroseptal scar, 9 patients (47%) had predominant inferolateral scar, and 2 patients (11%) had other scar types. Three distinct VT morphologies (≥1 of 3 inducible in 16/19 patients) were associated with underlying scar type. In 9 patients with anteroseptal scar-related VT (8/9 predominant scar, 1/9 nonpredominant), ablation target sites (defined as sites with ≥11/12 pacemap, concealed entrainment or VT termination during ablation) were located in the aortic root and/or anteroseptal left ventricular endocardium in 8 patients (89%) and in the anterior cardiac vein in 1 patient (11%), with additional target sites at the right ventricular septum in 2 patients (22%) and at the epicardium in 1 patient (11%). In contrast, in 8 patients with predominant inferolateral scar-related VT, target sites were located at the epicardium in 5 patients (63%) and in the endocardial inferolateral left ventricle in 3 patients (37%). Conclusions-Two typical scar patterns (anteroseptal and inferolateral) account for 89% of arrhythmogenic substrates in patients with nonischemic cardiomyopathy. Three distinct VT morphologies are highly suggestive of the presence of these scars. Anteroseptal scars were, in general, most effectively approached from the aortic root or anteroseptal left ventricular endocardium, whereas inferolateral scars frequently required an epicardial approach. (Circ Arrhythm Electrophysiol. 2013;6:875-883.)

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Late gadolinium enhanced cardiovascular magnetic resonance of lamin A/C gene mutation related dilated cardiomyopathy

Cited by 99 publications

References 29 publications

Catheter Ablation of Ventricular Arrhythmia in Nonischemic Cardiomyopathy

Catheter Ablation of Ventricular Arrhythmia in Nonischemic Cardiomyopathy

Extracellular matrix remodeling and transforming growth factor-β signaling abnormalities induced by lamin A/C variants that cause lipodystrophy

Contrast-Enhanced MRI–Derived Scar Patterns and Associated Ventricular Tachycardias in Nonischemic Cardiomyopathy

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