Examination of Isolated Ventricular Noncompaction (Hypertrabeculation) as a Distinct Entity in Adults

Roberts, William C.; Karia, Sumit; Ko, Jong Mi; Grayburn, Paul A.; George, Betsy Ann; Hall, Shelley; Kuiper, Johannes J.; Meyer, Dan M.

doi:10.1016/j.amjcard.2011.04.027

Cited by 36 publications

(15 citation statements)

References 34 publications

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“…On the other hand, over-diagnosis has been reported especially among patients with hypertrophic cardiomyopathy [5]. Both familial and non-familial forms have been reported in the literature and adult patients with NVM seem to be more common than pediatric cases.…”

Section: Discussionmentioning

confidence: 99%

A very rare case of coexistence of ventricular noncompaction cardiomyopathy, myocardial bridging and atherosclerosis

Erdoğan¹,

Akkaya²,

Bacaksız³

et al. 2013

pwki

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Noncompaction of the ventricular myocardium is a rare congenital heart disease, presumably caused by the intrauterine arrest of the myocardial compaction process at the beginning of fetal development. It could remain asymptomatic or manifest with congestive heart failure, arrhythmias, and systemic thromboemboli. Here we report a 55-year-old man who was admitted to hospital with chest pain and dyspnea, whose further evaluation revealed left ventricular noncompaction cardiomyopathy accompanying myocardial bridging and stenosis of the right coronary artery.

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Section: Discussionmentioning

confidence: 99%

A very rare case of coexistence of ventricular noncompaction cardiomyopathy, myocardial bridging and atherosclerosis

Erdoğan¹,

Akkaya²,

Bacaksız³

et al. 2013

pwki

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“…Indeed, ventricular wall morphogenesis seems to result from a continuous process during which trabecular cells are progressively incorporated into the compact myocardium. This process follows the formation of the ventricular wall from base to apex as demonstrated by the more severe defects observed in the apex region in mutant mice or in human patients suffering from LVNC [39,40]. Mutations in the NKX2-5 gene have been identified in LVNC patients indicating a role for this important cardiac transcription factor in compaction [41,42].…”

Section: Trabecular Specification Into Conductive and Contractile Carmentioning

confidence: 98%

Defects in Trabecular Development Contribute to Left Ventricular Noncompaction

2019

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Left Ventricular Noncompaction (LVNC) is a genetically heterogeneous disorder the etiology of which is still debated. During fetal development trabecular cardiomyocytes contribute extensively to the working myocardium and the ventricular conduction system. The impact of developmental defects in trabecular myocardium in the etiology of LVNC has been debated. Recently we generated new mouse models of LVNC by the conditional deletion of the key cardiac transcription factor encoding gene Nkx2-5 in trabecular myocardium at critical steps of trabecular development. These conditional mutant mice recapitulate pathological features similar to those observed in LVNC patients, including a hypertrabeculated left ventricle with deep endocardial recesses, subendocardial fibrosis, conduction defects, strain defects and progressive heart failure.After discussing recent findings describing the respective contribution of trabecular and compact myocardium during ventricular morphogenesis, this review will focus on new data reflecting the link between trabecular development and LVNC.

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“…In hearts excised at transplantation or at autopsy, LVNC diagnosis is on the basis of the prominent appearance of LV trabeculae and the ratio between the compacted and noncompacted LV wall (13).…”

Section: Anatomy and Pathologymentioning

confidence: 99%

“…Pathoanatomic investigation in autopsy hearts or in hearts excised at transplantation provides data for pathoimaging correlations and assessment of imaging-based diagnoses (13). The most commonly used imaging modalities include echocardiography and cardiac magnetic resonance (CMR).…”

mentioning

confidence: 99%

Left Ventricular Noncompaction

Arbustini

Weidemann

Hall

2014

Journal of the American College of Cardiology

200

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Whether left ventricular noncompaction (LVNC) is a distinct cardiomyopathy or a morphologic trait shared by different cardiomyopathies remains controversial. Current guidelines from professional organizations recommend different strategies for diagnosing and treating patients with LVNC. This state-of-the-art review discusses new insights into the basic mechanisms leading to LVNC, its clinical manifestations, treatment modalities, anatomy and pathology, embryology, genetics, epidemiology, and imaging. Three markers currently define LVNC: prominent left ventricular trabeculae, deep intertrabecular recesses, and a thin compacted layer. Although new genetic data from mice and humans supports LVNC as a distinct cardiomyopathy, evidence for LVNC as a shared morphological trait is not ruled out. Criteria supporting LVNC as a shared morphological trait may depend on consensus guidelines from the multiple professional organizations. Enhanced imaging and increased use of genetics are both predicted to significantly impact our overall understanding of the basic mechanisms causing LVNC and its optimal management.

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Examination of Isolated Ventricular Noncompaction (Hypertrabeculation) as a Distinct Entity in Adults

Cited by 36 publications

References 34 publications

A very rare case of coexistence of ventricular noncompaction cardiomyopathy, myocardial bridging and atherosclerosis

A very rare case of coexistence of ventricular noncompaction cardiomyopathy, myocardial bridging and atherosclerosis

Defects in Trabecular Development Contribute to Left Ventricular Noncompaction

Left Ventricular Noncompaction

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