Isolated Noncompaction of the Left Ventricular Myocardium in Adults: A Systematic Overview

Bhatia, Nisha L.; Tajik, A. Jamil; Wilansky, Susan; Steidley, D. Eric; Mookadam, Farouk

doi:10.1016/j.cardfail.2011.05.002

Cited by 144 publications

(144 citation statements)

References 43 publications

Supporting

Mentioning

127

Contrasting

Unclassified

Order By: Relevance

“…Although early reports suggested that ventricular arrhythmias were uncommon in adults with LVNC, a recent review found the incidence to be as high as 33%. 17 In pediatric patients, ventricular arrhythmias are indeed a relatively common complication of LVNC, affecting 17% of the children in the present study cohort. Additionally, ventricular arrhythmias continue to be a major cause of mortality, with the onset of VT being an indicator of poor prognosis.…”

Section: Discussionmentioning

confidence: 99%

Mortality and Sudden Death in Pediatric Left Ventricular Noncompaction in a Tertiary Referral Center

et al. 2013

View full text Add to dashboard Cite

Background-Left ventricular noncompaction is a cardiomyopathy characterized by excessive trabeculation of the left ventricle, progressive myocardial dysfunction, and early mortality. Left ventricular noncompaction has a heterogeneous clinical presentation that includes arrhythmia and sudden cardiac death. Methods and Results-We retrospectively reviewed all children diagnosed with left ventricular noncompaction at TexasChildren's Hospital from January 1990 to January 2009. Patients with congenital cardiac lesions were excluded. Two hundred forty-two children were diagnosed with isolated left ventricular noncompaction over the study period. Thirtyone (12.8%) died, and 13 (5.4%) were received a transplant. One hundred fifty (62%) presented with or developed cardiac dysfunction. The presence of cardiac dysfunction was strongly associated with mortality (hazard ratio, 11; P<0.001).ECG abnormalities were present in 87%, with ventricular hypertrophy and repolarization abnormalities occurring most commonly. Repolarization abnormalities were associated with increased mortality (hazard ratio, 2.1; P=0.02). Eighty children (33.1%) had an arrhythmia, and those with arrhythmias had increased mortality (hazard ratio, 2.8; P=0.002). Forty-two (17.4%) had ventricular tachycardia, with 5 presenting with resuscitated sudden cardiac death. In total, there were 15 cases of sudden cardiac death in the cohort (6.2%). Nearly all patients with sudden death (14 of 15) had abnormal cardiac dimensions or cardiac dysfunction. No patient with normal cardiac dimensions and function without preceding arrhythmias died. Conclusions-Left ventricular noncompaction has a high mortality rate and is strongly associated with arrhythmias in children. Preceding cardiac dysfunction or ventricular arrhythmias are associated with increased mortality. Children with normal cardiac dimensions and normal function are at low risk for sudden death. Brescia et al Mortality and Pediatric LVNC 2203the ventricular cavity, as demonstrated by color Doppler; and (3) a 2-layered structure of the endocardium with a noncompacted to compacted ratio >2.0. 8 Patients were included in the cohort only if both echocardiogram reviewers agreed on the diagnosis. Patients with LVNC and associated congenital heart disease or known metabolic syndromes (nonisolated LVNC) were excluded. Only patients primarily followed up at Texas Children's Hospital for LVNC were included; patients referred for isolated second opinions or only seen for single visits (without longitudinal follow-up) were excluded. The present study was approved by the Baylor College of Medicine Institutional Review Board, and individual consent was waived. Medical records were reviewed to document clinical presentation and course. Serial echocardiograms were analyzed for measures of systolic function (shortening fraction and ejection fraction), and cardiac dimensions were measured by M-mode echocardiography. Ejection fraction was calculated by the Simpson biplane method. Cardiac systolic dysfunction was defined as an...

show abstract

Section: Discussionmentioning

confidence: 99%

Mortality and Sudden Death in Pediatric Left Ventricular Noncompaction in a Tertiary Referral Center

et al. 2013

View full text Add to dashboard Cite

show abstract

“…Pojęcie niescalenia odnosi się do obecności wyraźnego beleczkowania i głębokich zachyłków międzybeleczkowych w LV i/lub RV, często w połączeniu z cienką zbitą nasierdziową warstwą mięśnia sercowego [422]. U niektórych pacjentów niescalenie wiąże się z rozstrzenią komory i dysfunkcją skurczową.…”

Section: Niescalenie Mięśnia Lewej Komoryunclassified

“…Opisywanymi wskaźnikami predykcyjnymi zwiększonej śmiertelności są starszy wiek, wymiar końcoworozkurczowy LV w momencie ujawnienia się choroby, objawowa HF, utrwalone lub przetrwałe AF, blok odnogi pęczka Hisa oraz współistniejące choroby nerwowo-mięśniowe, ale nie ma zbyt wielu danych wskazujących na to, że samo niescalenie mięśnia LV jest wskazaniem do wszczepienia ICD [422][423][424][425]. Podejmując decyzje dotyczące leczenia za pomocą ICD, należy się kierować ciężkością dysfunkcji skurczowej LV oraz występowaniem utrwalonej VA według tych samych kryteriów, co w przypadku DCM (patrz punkt 7.1.…”

Section: Niescalenie Mięśnia Lewej Komoryunclassified

2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death

Priori

Blomström‐Lundqvist²,

Mazzanti³

et al. 2015

Kardiol Pol

233

337

View full text Add to dashboard Cite

“…2010; Bhatia et al. 2011). Left ventricular non‐compaction is associated with congenital cardiac disorders, other cardiomyopathies, and some neuromuscular diseases (Jenni et al.…”

Section: Introductionmentioning

confidence: 99%

The pathogenicity of genetic variants previously associated with left ventricular non‐compaction

Abbasi

Jabbari

et al. 2015

Molec Gen & Gen Med

View full text Add to dashboard Cite

BackgroundLeft ventricular non‐compaction (LVNC) is a rare cardiomyopathy. Many genetic variants have been associated with LVNC. However, the number of the previous LVNC‐associated variants that are common in the background population remains unknown. The aim of this study was to provide an updated list of previously reported LVNC‐associated variants with biologic description and investigate the prevalence of LVNC variants in healthy general population to find false‐positive LVNC‐associated variants.Methods and ResultsThe Human Gene Mutation Database and PubMed were systematically searched to identify all previously reported LVNC‐associated variants. Thereafter, the Exome Sequencing Project (ESP) and the Exome Aggregation Consortium (ExAC), that both represent the background population, was searched for all variants. Four in silico prediction tools were assessed to determine the functional effects of these variants. The prediction results of those identified in the ESP and ExAC and those not identified in the ESP and ExAC were compared. In 12 genes, 60 LVNC‐associated missense/nonsense variants were identified. MYH7 was the predominant gene, encompassing 24 of the 60 LVNC‐associated variants. The ESP only harbored nine and ExAC harbored 18 of the 60 LVNC‐associated variants. In total, eight out of nine ESP‐positive variants overlapped with the 18 variants identified in ExAC database.ConclusionsIn this article, we identified 9 ESP‐positive and 18 ExAC‐positive variants of 60 previously reported LVNC‐associated variants, suggesting that these variants are not necessarily the monogenic cause of LVNC.

show abstract

Isolated Noncompaction of the Left Ventricular Myocardium in Adults: A Systematic Overview

Cited by 144 publications

References 43 publications

Mortality and Sudden Death in Pediatric Left Ventricular Noncompaction in a Tertiary Referral Center

Mortality and Sudden Death in Pediatric Left Ventricular Noncompaction in a Tertiary Referral Center

2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death

The pathogenicity of genetic variants previously associated with left ventricular non‐compaction

Contact Info

Product

Resources

About