Wide spectrum of presentation and variable outcomes of isolated left ventricular non-compaction

Lofiego, Carla; Biagini, Elena; Pasquale, Ferdinando; Ferlito, Marinella; Rocchi, Guido; Perugini, Enrica; Reggiani, Maria Letizia Bacchi; Boriani, Giuseppe; Leone, Ornella; Çalışkan, Kadir; Cate, Folkert J. ten; Picchio, Fernando Maria; Branzi, Angelo; Rapezzi, Claudio

doi:10.1136/hrt.2006.088229

Cited by 217 publications

(218 citation statements)

References 30 publications

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“…Recent study by Stollberger et al demonstrated that of 106 patients with LVNC who underwent a complete neurologic evaluation, 22 (21%) patients were diagnosed with a well-defined neuromuscular disorder and 68 patients (64%) a neuromuscular disorder of unknown etiology [5]. The rate of neuromuscular disease in this study is higher than previously described in other studies; however, patients in these previous studies were not routinely referred for neurologic evaluation [4,6,26,27]. The presence of a neuromuscular disorder in conjunction with LVNC was found to be associated with a higher mortality rate in this case series, 15% versus 0% [5].…”

Section: Associationscontrasting

confidence: 50%

“…The occurrence rate of VT in adults with LVNC has been highly variable across studies (6-63%) which include a small number of patients, a high percentage of whom also have LV dysfunction as a strong risk factor for VT [3][4][5][6]10,22,25,26]. Thus, whether or not LVNC is an independent risk factor for VT is not known.…”

Section: Arrhythmiamentioning

confidence: 99%

“…However, subsequent adult studies have shown both a lower total rate of systemic embolism (0-15%) and a strong association of embolic events with presence of AF and LV dysfunction [3,6,10,12,19,25,26,31]. One case series of adults compared 30 patients with LVNC to dilated cardiomyopathy control patients and found a lower rate of stroke in the LVNC group [10].…”

Section: Embolizationmentioning

confidence: 99%

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Left Ventricular Non-Compaction: Current Controversy and New Insights

E¹

2015

J Genet Syndr Gene Ther

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Left ventricular non-compaction is an increasingly recognized familial cardiomyopathy characterized morphologically by deep myocardial trabeculations and a highly variable, but potentially aggressive clinical course leading to cardiac death or cardiac transplantation. Great controversy exists regarding the pathophysiology, diagnostic approach, clinical characteristics, and management of the condition. To date, there are no universally accepted precise diagnostic criteria or management guidelines. Recent clinical studies and basic science research have significantly added to our understanding of LVNC in all of these aspects. This article reviews the literature to date on LVNC and addresses current controversies surrounding the pathophysiology, diagnosis, clinical features, and management.

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Section: Associationscontrasting

confidence: 50%

Section: Arrhythmiamentioning

confidence: 99%

Section: Embolizationmentioning

confidence: 99%

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Left Ventricular Non-Compaction: Current Controversy and New Insights

E¹

2015

J Genet Syndr Gene Ther

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“…[5][6][7][8][9][10][11][12][13][14][15] Typical complications include congestive heart failure, ventricular arrhythmias and thromboembolic events. [12][13][14]16,17 Recently, the association of LVNC with metabolic diseases, genetic syndromes and other cardiac abnormalities has been reported. [18][19][20] Genetic studies have shown sarcomere gene mutations in some patients with isolated LVNC similar to those with dilated or hypertrophic cardiomyopathy, and a recent study has found similar mutations in patients with Ebstein anomaly.…”

Section: Since Its First Description Left Ventricular Non-compactionmentioning

confidence: 99%

Left ventricular non‐compaction: Prevalence in congenital heart disease

Stähli

Gebhard

Biaggi

et al. 2013

International Journal of Cardiology

127

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INTRODUCTION: Left ventricular non-compaction cardiomyopathy (LVNC) is a rare cardiomyopathy, originally described as an isolated disease without other structural cardiac abnormalities. The aim of this study was to explore the prevalence of LVNC among adults with different types of congenital heart disease. METHODS: From our databases we identified adults with congenital heart disease who fulfilled diagnostic criteria for LVNC. We report frequencies of associated congenital cardiac defects and the prevalence of LVNC among patients with different congenital heart defects. RESULTS: From a total of 202 patients with LVNC, 24 patients (12%; mean age 32±11years, 19 males) had additional congenital cardiac defects. Associated defects were left ventricular outflow tract abnormalities in 11 patients (46%), including 7 uni-or bicuspid aortic valves; two aortic coarctations; one diffuse aortic hypoplasia and one subaortic stenosis, Ebstein anomaly in 6 patients (25%), tetralogy of Fallot in two (8%), and double outlet right ventricle in one patient (4%). In our cohort, the prevalence of LVNC was highest among patients with Ebstein anomaly (6/40, 15%), followed by aortic coarctation (2/60, 3%), tetralogy of Fallot (3/129, 2%) and uni-or bicuspid aortic valves (7/963, 1%). CONCLUSION: In adults, various forms of congenital heart disease are associated with LVNC, particularly stenotic lesions of the left ventricular outflow tract, Ebstein anomaly, and tetralogy of Fallot. In the future, studying these patients in more depth may provide a better understanding of the interplay between genetic and hemodynamic factors that lead to the phenotype of LVNC.

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“…The electrophysiological study and/or the prophylactic implantation of ICD must be carefully considered. Competitive sports must be avoided and the first-degree relatives must be screened through Doppler echocardiographic studies 10 .…”

Section: Discussionmentioning

confidence: 99%