Left Ventricular Noncompaction: A New Form of Heart Failure

Towbin, Jeffrey A.

doi:10.1016/j.hfc.2010.06.005

Cited by 159 publications

(142 citation statements)

References 109 publications

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“…For instance, left ventricular noncompaction (LVNC) is a cardiomyopathy caused by arrested compaction. It is characterized by excessive trabeculation with deep intertrabecular recesses and thin compact myocardium (6). It usually affects both ventricles, but is more prominent on the left in humans.…”

Section: Introductionmentioning

confidence: 99%

Zinc transporter Slc39a8 is essential for cardiac ventricular compaction

Wen¹,

Li²,

Cheng³

et al. 2018

Journal of Clinical Investigation

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Section: Introductionmentioning

confidence: 99%

Zinc transporter Slc39a8 is essential for cardiac ventricular compaction

Wen¹,

Li²,

Cheng³

et al. 2018

Journal of Clinical Investigation

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“…Concomitant with ventricular septation, the trabeculae start to compact at their base adjacent to the outer myocardium, adding substantially to ventricular wall thickness and coincidently establishing coronary circulation (Wessels and Sedmera, 2003). A significant reduction in trabeculation is closely associated with myocardial growth arrest, whereas persistent trabeculation and/or a reduced level of compaction are associated with left ventricular noncompaction (LVNC) (Towbin, 2010) [also called left ventricular hypertrabeculation (LVHT)] (Finsterer, 2009). LVNC (MIM300183) is a distinct form of inherited cardiomyopathy (Pignatelli et al, 2003;Sandhu et al, 2008).…”

Section: Introductionmentioning

confidence: 99%

Fkbp1a controls ventricular myocardium trabeculation and compaction by regulating endocardial Notch1 activity

et al. 2013

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SUMMARYTrabeculation and compaction of the embryonic myocardium are morphogenetic events crucial for the formation and function of the ventricular walls. Fkbp1a (FKBP12) is a ubiquitously expressed cis-trans peptidyl-prolyl isomerase. Fkbp1a-deficient mice develop ventricular hypertrabeculation and noncompaction. To determine the physiological function of Fkbp1a in regulating the intercellular and intracellular signaling pathways involved in ventricular trabeculation and compaction, we generated a series of Fkbp1a conditional knockouts. Surprisingly, cardiomyocyte-restricted ablation of Fkbp1a did not give rise to the ventricular developmental defect, whereas endothelial cell-restricted ablation of Fkbp1a recapitulated the ventricular hypertrabeculation and noncompaction observed in Fkbp1a systemically deficient mice, suggesting an important contribution of Fkbp1a within the developing endocardia in regulating the morphogenesis of ventricular trabeculation and compaction. Further analysis demonstrated that Fkbp1a is a novel negative modulator of activated Notch1. Activated Notch1 (N1ICD) was significantly upregulated in Fkbp1a-ablated endothelial cells in vivo and in vitro. Overexpression of Fkbp1a significantly reduced the stability of N1ICD and direct inhibition of Notch signaling significantly reduced hypertrabeculation in Fkbp1a-deficient mice. Our findings suggest that Fkbp1a-mediated regulation of Notch1 plays an important role in intercellular communication between endocardium and myocardium, which is crucial in controlling the formation of the ventricular walls.KEY WORDS: FK506 binding protein 12, Ventricular hypertrabeculation/noncompaction, Notch1, Endocardial-myocardial signaling, Mouse

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“…Both DCM and LVNC were included in the same review, because both conditions are still considered idiopathic 6, 14 in most patients. Some of the records were further reviewed regarding echocardiographic examinations and results.…”

Section: Methodsmentioning

confidence: 99%

The highest mortality rates in childhood dilated cardiomyopathy occur during the first year after diagnosis

et al. 2017

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AimThe aim of the study was to assess the incidence, mortality and morbidity of dilated cardiomyopathy (DCM) and noncompaction of the left ventricle (LVNC) in Swedish children.MethodsWe reviewed hospital records of all children with dilated cardiomyopathy (DCM) or left ventricular noncompaction cardiomyopathy (LVNC) up to the age of 18 in the healthcare region of western Sweden from 1991 to 2015.ResultsIn total, 69 cases (61% males) were identified. The combined incidence of DCM and LVNC was 0.77 (95% CI 0.59‐0.96) per 100 000 person years. Children were divided into six groups, and their outcomes were analysed depending on their aetiology. Idiopathic DCM was reported in 43%, and familial dilated and left ventricular noncompaction aetiology was present in 32%. DCM due to various diseases occurred in 8%. DCM associated with neuromuscular diseases was present in 16%. The overall risk of death or receiving transplants in children with idiopathic and familial DCM was 30% over the study period, and 21% died in the first year after diagnosis.ConclusionThe combined incidence of DCM and LVNC was similar to previous reports. Most children with idiopathic DCM presented during infancy, and mortality was highest during the first year after diagnosis.

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Left Ventricular Noncompaction: A New Form of Heart Failure

Cited by 159 publications

References 109 publications

Zinc transporter Slc39a8 is essential for cardiac ventricular compaction

Zinc transporter Slc39a8 is essential for cardiac ventricular compaction

Fkbp1a controls ventricular myocardium trabeculation and compaction by regulating endocardial Notch1 activity

The highest mortality rates in childhood dilated cardiomyopathy occur during the first year after diagnosis

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