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HELIX DIAGNOSTICS

Comprehensive Cardiomyopathy Panel


Panel Description

Cardiomyopathies are a broad spectrum of structural and functional disorders of the heart musculature. There are many different causes of cardiomyopathies, which range from environmental exposures to inherited genetic risk factors. In cases where an external cause is not identified, and/or a family history is suspicious for hereditary risk, diagnostic genetic testing may be indicated.

This panel evaluates 86 genes that have an established, primary association with cardiomyopathy, and several syndromic conditions of which cardiomyopathy is a feature.

Genes Tested (86)

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ABCC9
ACAD9
ACADVL
ACTC1
ACTN2
AGL
ALMS1
ALPK3
BAG3
BMP10
BRAF
CDH2
CPT2
CRYAB
CSRP3
DES
DMD
DNAJC19
DOLK
DSG2
DSG2
DSP
DTNA
ELAC2
EMD
FHL1
FKRP
FKTN
FLNC
GAA
GLA
HCN4
HRAS
JPH2
JUP
KRAS
LAMP2
LMNA
LZTR1
MAP2K1
MAP2K2
MRAS
MTO1
MYBPC3
MYH7
MYL2
MYL3
MYLK3
MYPN
NEXN
NKX2-5
NRAS
PCCA
PCCB
PKP2
PLN
PPA2
PPCS
PRDM16
PRKAG2
PTPN11
RAF1
RBM20
RIT1
RYR2
SCN5A
SGCD
SHOC2
SLC22A5
SOS1
SOS2
SYNE2
TAFAZZIN
TBX20
TCAP
TMEM43
TMEM70
TNNC1
TNNI3
TNNI3K
TNNT2
TPM1
TRIM63
TTN
TTR
VCL

Important Panel Information

Turnaround time: 7-24 days

Preferred specimen: BD Vacutainer Whole Blood K2 EDTA Collection Tube 4mL or Oragene Dx Saliva Collection Kit

Shipping instructions: Specimens to arrive at Helix within 96 hours of collection at ambient temperature.

Hypertrophic Cardiomyopathy (HCM) is a condition in which the heart muscle becomes abnormally thick. This leads to a decrease in the heart's ability to pump blood effectively. This can cause fatigue, shortness of breath, swelling of the legs, heart rhythm abnormalities and, in severe cases, heart failure or sudden cardiac arrest. Dilated Cardiomyopathy (DCM) is a condition in which the heart chambers become enlarged, weakening the heart muscle. In individuals with left ventricular noncompaction (LVNC), LVNC is characterized by endomyocardial trabeculations which can have variable effects on heart musculature, including ventricular dilation. Individuals with DCM or LVNC may be asymptomatic, or may be symptomatic with arrhythmia, left ventricular dysfunction, thromboembolic disease, and/or life-threatening arrhythmias. Arrhythmogenic cardiomyopathy (ACM) is characterized by fibrofatty infiltration of ventricle musculature which may present as arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC) or arrhythmogenic left ventricular cardiomyopathy/dysplasia (ALVC). These findings are associated with increased risk for ventricular dysfunction, and arrhythmogenic events which may result in syncope or, in rare cases, cardiac arrest or sudden death.

It is important to note that in some cases HCM, DCM, LVNC and ARVC may be a feature of a larger syndromic condition. Hereditary forms of these conditions may follow autosomal dominant, autosomal recessive, X-linked or mitochondrial inheritance patterns. This panel does not assess mitochondrial inheritance. Note that some of these genes may also be associated with other unrelated conditions; this means that when undergoing this test, there is a possibility of incidentally detecting carrier status for, or predisposition to, one of these unrelated conditions.

All detected variants are evaluated according to American College of Medical Genetics and Genomics recommendations. Variants are classified based on known, predicted, or possible pathogenicity and reported with interpretive comments detailing their potential or known significance.

Other Tests to Consider

Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) Panel

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Brugada Syndrome Test

Comprehensive Arrhythmias Panel

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Arrhythmogenic Cardiomyopathy Panel

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