The extent and location of late gadolinium enhancement predict defibrillator shock and cardiac mortality in patients with non-ischaemic dilated cardiomyopathy

Barison, Andrea; Aimo, Alberto; Mirizzi, Gianluca; Castiglione, Vincenzo; Ripoli, Andrea; Panchetti, Luca; Rossi, Andrea; Giannoni, Alberto; Startari, Umberto; Aquaro, Giovanni Donato; Emdin, Michele; Piacenti, Marcello

doi:10.1016/j.ijcard.2020.02.028

Cited by 19 publications

(21 citation statements)

References 23 publications

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“…The relationship between LGE extent and increased risk for VT may represent an increased susceptibility to reentrant ventricular arrhythmias. For our study, LGE extent was evaluated by median of the cohort ( 30 , 31 ), per incremental increase ( 28 ), and by number of segments ( 27 ) as utilized in previous studies. LGE extent was consistently significant using these three definitions in our cohort of NICM patients but using only one definition (LGE ≥ median) in ICM patients.…”

Section: Discussionmentioning

confidence: 99%

“…In patients with NICM, the impact of LGE extent in the literature, while overall strongly correlative to worse outcomes, is highly variable. In our study, LGE greater than the median of 10% and >15% when stratified per 5% increase were significantly associated with risk for VT. LGE cut-offs in the literature found to have prognostic value have been reported from just 1, 5% ( 29 ), 8% ( 31 ) to 13% ( 28 ). The variability in these findings is likely a reflection of different patient populations, methodology for LGE quantification, and most importantly, the heterogenous composition of the various cardiomyopathies that make up NICM.…”

Section: Discussionmentioning

confidence: 99%

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Impact of late gadolinium enhancement extent, location, and pattern on ventricular tachycardia and major adverse cardiac events in patients with ischemic vs. non-ischemic cardiomyopathy

Tat

Ball

Camren

et al. 2022

Front. Cardiovasc. Med.

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BackgroundLeft ventricular late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) has been associated with increased risk for life-threatening ventricular tachyarrhythmias. The differences in association between LGE characteristics and prognosis in patients with ischemic (ICM) vs. non-ischemic (NICM) cardiomyopathy is incompletely understood.MethodsA total of 168 consecutive patients who underwent CMR imaging with either ICM or NICM were included in our study. LGE extent, location and pattern were examined for association to the primary endpoint of ventricular tachycardia (VT) and secondary endpoint of major adverse cardiac events (MACE).ResultsOf 68 (41%) patients with ICM and 97 (59%) patients with NICM, median LGE mass was 15% (IQR 9–28) for the ICM group and 10% (IQR 6–15) for the NICM group. On multivariate analysis for both groups, LGE characteristics were prognostic while LVEF was not. In patients with ICM, septal and apical segment LGE, and involvement of multiple walls predicted both endpoints on multivariate analysis. LGE extent (≥median) and inferior wall LGE independently predicted the primary endpoint. In patients with NICM, anterior, inferior and apical segment LGE, and involvement of multiple walls predicted both endpoints on multivariate analysis. LGE extent (≥median, number of LGE segments, LGE stratified per 5% increase) and midwall LGE were independent predictors of the primary endpoint.ConclusionsAlthough LGE was an independent predictor of prognosis in both groups, LGE extent, location, and pattern characteristics were more powerful correlates to worse outcomes in patients with NICM than ICM.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Impact of late gadolinium enhancement extent, location, and pattern on ventricular tachycardia and major adverse cardiac events in patients with ischemic vs. non-ischemic cardiomyopathy

Tat

Ball

Camren

et al. 2022

Front. Cardiovasc. Med.

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“…Cardiac magnetic resonance (1.5 T, Signa Artist, GE Healthcare, Waukesha, WI, USA) was performed to assess biventricular systolic function (steady-state free precession cine sequences) and late enhancement imaging (T1-weighted gradient-echo inversion-recovery sequence acquired 10–20 min after 0.2 mmol/Kg gadoteric acid injection) [ 16 ].…”

Section: Methodsmentioning

confidence: 99%

Partial Lipodystrophy and LMNA p.R545H Variant

Magno

Ceccarini

Barison

et al. 2021

JCM

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Laminopathies are disorders caused by LMNA gene mutations, which selectively affect different tissues and organ systems, and present with heterogeneous clinical and pathological traits. The molecular mechanisms behind these clinical differences and tissue specificity have not been fully clarified. We herein examine the case of a patient carrying a heterozygous LMNA c.1634G>A (p.R545H) variant with a mild, transient myopathy, who was referred to our center for the suspicion of lipodystrophy. At physical examination, an abnormal distribution of subcutaneous fat was noticed, with fat accumulation in the anterior regions of the neck, resembling the fat distribution pattern of familial partial lipodystrophy type 2 (FPLD2). The R545H missense variant has been found at very low allelic frequency in public databases, and in silico analysis showed that this amino acid substitution is predicted to have a damaging role. Other patients carrying the heterozygous LMNA p.R545H allele have shown a marked clinical heterogeneity in terms of phenotypic body fat distribution and severity of organ system involvement. These findings indicate that the LMNA p.R545H heterozygous variant exhibits incomplete penetrance and highly variable expressivity. We hypothesized that additional genetic factors, epigenetic mechanisms, or environmental triggers might explain the variable expressivity of phenotypes among various patients.

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“…CMR is an established technique for the assessment of both LV and RV volumes and regional/global systolic function, and can be used to determine the etiology of ventricular dysfunction in dilated and other cardiomyopathies [33]. In adult patients with DCM, CMR studies are indicated to differentiate ischemic versus non-ischemic etiologies; determine ventricular size, function, and mass; detect intracardiac thrombus; and assess for LGE as a marker of fibrosis and for prognostic stratification [34,35]. Children with non-ischemic DCM may have a non-coronary pattern of LGE with patchy or longitudinal mid-wall enhancement, subepicardial, or diffuse subendocardial distribution, but LGE is found less commonly than in adults with this disease [36,37].…”

Section: Goals Of Examinationmentioning

confidence: 99%

SCMR expert consensus statement for cardiovascular magnetic resonance of acquired and non-structural pediatric heart disease

Dorfman

Geva

Samyn

et al. 2022

Journal of Cardiovascular Magnetic Resonance

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Cardiovascular magnetic resonance (CMR) is widely used for diagnostic imaging in the pediatric population. In addition to structural congenital heart disease (CHD), for which published guidelines are available, CMR is also performed for non-structural pediatric heart disease, for which guidelines are not available. This article provides guidelines for the performance and reporting of CMR in the pediatric population for non-structural (“non-congenital”) heart disease, including cardiomyopathies, myocarditis, Kawasaki disease and systemic vasculitides, cardiac tumors, pericardial disease, pulmonary hypertension, heart transplant, and aortopathies. Given important differences in disease pathophysiology and clinical manifestations as well as unique technical challenges related to body size, heart rate, and sedation needs, these guidelines focus on optimization of the CMR examination in infants and children compared to adults. Disease states are discussed, including the goals of CMR examination, disease-specific protocols, and limitations and pitfalls, as well as newer techniques that remain under development.

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The extent and location of late gadolinium enhancement predict defibrillator shock and cardiac mortality in patients with non-ischaemic dilated cardiomyopathy

Cited by 19 publications

References 23 publications

Impact of late gadolinium enhancement extent, location, and pattern on ventricular tachycardia and major adverse cardiac events in patients with ischemic vs. non-ischemic cardiomyopathy

Impact of late gadolinium enhancement extent, location, and pattern on ventricular tachycardia and major adverse cardiac events in patients with ischemic vs. non-ischemic cardiomyopathy

Partial Lipodystrophy and LMNA p.R545H Variant

SCMR expert consensus statement for cardiovascular magnetic resonance of acquired and non-structural pediatric heart disease

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