LVNC Articles

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Every time we find a new article published about LVNC we will try and post it here.  If you find an article and believe we do not  have it please let us know so we can add it to our growing database!

Isolated Noncompaction of the Left Ventricle in Adults February, 2016:

Isolated left ventricular noncompaction (ILVNC) is a cardiomyopathy that was first described in 1926 as a “spongy myocardium.” The disorder results from intrauterine arrest of compaction of the loose interwoven meshwork of the fetal myocardial primordium and subsequent persistence of deep trabecular recesses in the myocardial wall.

Journal of Medical Cases February, 2015:

 Clinical Dilemma: Recurrent Syncope With Isolated Left Ventricle Non-Compaction Cardiomyopathy and Preserved Ejection        Fraction, a Case Report and Review of the Literature


Our current understanding of isolated left ventricular non-compaction, including diagnostic criteria, management and prognosis, is discussed. The prognosis of the disease is determined by its complications; the death rate and heart transplantation frequency can be as high as 50%.

 Cardiac MR reliably identifies patients with clinically significant left ventricular noncompaction February, 2015:

Trabecular mass quantification at CMR provides an accurate and reliable means of identifying patients with clinically significant LV noncompaction, particularly among patients with a depressed EF and negative echocardiogram.

Left Ventricular Noncompaction (LVNC) February, 2015:

Families with left ventricular noncompaction (LVNC) have been shown to pass the disease on in two different ways, via autosomal dominant or x-linked inheritance.

Summary of “Left Ventricular Noncompaction:

Diagnosing left ventricular noncompaction (LVNC) cardiomyopathy is a challenge for the medical community because the condition shares morphologic features of hypertrophic and dilated cardiomyopathies.

Left Ventricular Noncompaction Cardiomyopathy: 

Left ventricular non-compaction, the most recently classified form of cardiomyopathy, is characterised by abnormal trabeculations in the left ventricle, most frequently at the apex. It can be associated with left ventricular dilation or hypertrophy, systolic or diastolic dysfunction, or both, or various forms of congenital heart disease.

 Mutation as a molecular explanation

A very recent study suggested that HCN4 mutations could be associated with sinusal bradycardia and myocardial non compaction.

Abnormal Activation of TGF Signaling as a Pathogenesis of Left Ventricular Non-compaction Cardiomyopathy December, 2015:

Left ventricular non-compaction (LVNC) is the third most prevalent cardiomyopathy in children and has a unique phenotype with characteristically extensive hypertrabeculation of the left ventricle, similar to the embryonic left ventricle, suggesting a developmental defect of the embryonic myocardium. However, in-depth investigation of this disease has been challenging due to the lack of animal models that can faithfully recapitulate the clinical phenotype of LVNC.

Biventricular Non-Compaction Cardiomyopathy December 7th, 2015:

Left ventricular non-compaction (spongy myocardium) is one of the most misclassified cardiomyopathies. It is characterised by an excessively prominent trabecular meshwork of myocardium and deep intertrabecular myocardium due to an arrest in the compaction process of the myocardial fibres. It could be isolated i.e. without any other structural heart defects or associated with congenital heart defects. The clinical manifestations are variable heart failure, arrhythmia, thromboembolic phenomena depending on extent of non-compaction of cardiac segment.

 Inheritance of LVNC is most often X linked recessive or autosomal dominant. December 12th, 2015:

Inheritance of LVNC is most often X-linked recessive or autosomal dominant, although autosomal recessive and mitochondrial (maternal) inheritance also occur.10, 14, 30 and 79 X-linked LVNC, which is usually associated with the multisystem disorder Barth syndrome (caused by a mutation in the TAZ gene), affects men almost exclusively, although a woman with cardiomyopathy has been described.