LVNCNext

LVNCNextTM is a targeted panel for patients with left ventricular non-compaction (LVNC). Often, LVNC is asymptomatic and sudden death is the first symptom. Therefore, genetic testing may be the most effective way of identifying at-risk individuals or confirming a diagnosis.

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LVNCNextTM is a targeted panel for patients with left ventricular non-compaction (LVNC). Often, LVNC is asymptomatic and sudden death is the first symptom. Therefore, genetic testing may be the most effective way of identifying at-risk individuals or confirming a diagnosis.

LVNCNext is a next generation sequencing (NGS) and deletion/duplication panel of eight genes associated with LVNC: ACTC1, LDB3/ZASP, LMNA, MYBPC3, MYH7, TAZ, TNNT2 and TPM1.  These genes are also included in the comprehensive inherited cardiomyopathy (CMNextTM) and cardiovascular genetics (CardioNext) panels. 

Disease Name 
Left ventricular non-compaction (LVNC)
Disease Information 

LVNC is characterized by excessive and unusual trabeculations within the mature left ventricle. Symptoms in patients vary greatly from asymptomatic to severe heart failure and/or arrhythmias, and include overlapping of left ventricular hypertrophy, dilation, restriction, or various forms of congenital heart disease. In some families, all affected family members have LVNC. However, in other families, some family members have LVNC, while others have dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), or other cardiomyopathies.

Testing Benefits & Indication 
  • Clarify diagnosis and risk for sudden cardiac arrest
  • Target medical management and prevention of cardiac arrest and other complications
  • Offer family members genetic testing (for a familial mutation) and implement medical surveillance to only those that need it
  • Reduce healthcare costs, resources, and anxiety for families
Test Description 

LVNCNext includes 8 genes associated with LVNC: ACTC1, LDB3/ZASP, LMNA, MYBPC3, MYH7, TAZ, TNNT2 and TPM1.  These genes are also included in the comprehensive inherited cardiomyopathy (CMNext) and cardiovascular genetics (CardioNext) panels. Genomic deoxyribonucleic acid (gDNA) is isolated from the patient’s specimen using a standardized kit and quantified. Sequence enrichment of the targeted coding exons and adjacent intronic nucleotides is carried out by a bait-capture methodology using long biotinylated oligonucleotide probes, followed by polymerase chain reaction (PCR) and NGS. Sanger sequencing is performed for any regions missing or with insufficient read depth coverage for reliable heterozygous variant detection. Suspect variant calls are verified by Sanger sequencing.  This assay targets all coding domains, and well into the flanking 5’ and 3’ ends of all the introns and untranslated regions. Gross deletion/duplication analysis for all genes is performed utilizing a targeted chromosomal microarray.

Mutation Detection Rate 

LVNCNext can detect >99.9% of described mutations in the included genes, when present (analytic sensitivity).

Specimen Requirements 

Complete specimen requirements are available here or by downloading the PDF found above on this page.

Turnaround Time 
TEST CODE TEST NAME TURNAROUND TIME (Weeks)
8906 LVNCNext 4-5 
8886 CMNextTM 4-5 
8887 CMNext + TTN 4-5 
8910 CardioNextTM 4-5 
8911 CardioNext + TTN 4-5 
9520 CustomNext-Cardio
Up to 85 cardiovascular genes of your choice
4-5 weeks

 

Specialty 
Genes 
ACTC1
LMNA
MYBPC3
MYH7
TAZ
TNNT2
TPM1
LDB3/ZASP
Tests 
References 
  1. Adapted from Ackerman MJ, et al. HRS/EHRA Expert Consensus Statement on the State of Genetic Testing for the Channelopathies and Cardiomyopathies. Heart Rhythm. 2011 Aug;8(8):1308-39.