Left ventricular non compaction is rare congenital cardiomyopathy with gaining interest due to advancement in imaging modalities for diagnosis and assessment of undulating phenotype

1. LEFT VENTRICULAR NON- COMPACTION
SHORT REVIEW
DR MAHENDRA
CARDIOLOGY,JIPMER
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2. INTRODUCTION
• Spongy appearance of the myocardium first described by
Grant in 1926.
• Developmental considerations-
• development of the myocardial architecture which passes
through four distinct steps
• (i) early heart tube
• (ii) emergence of trabeculations
• (iii) trabecular remodeling
• (iv) development of the multilayered spiral system
• Emergence of trabeculations and trabecular remodeling are
the key steps to understand LVNC
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3. • Emergence of trabeculations-
• emerge after looping of the primitive heart tube at the end of
the fourth week of gestation.
• trabeculation patterns are ventricle-specific
• trabeculations in the LV are generally thicker and the
corresponding intertrabecular spaces are larger at this
embryonic stage.
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4. • Trabecular remodeling-
• remodelling starts after completion of ventricular septation at
8 weeks of gestation in human.
• Increase in ventricular volumes results in compression of the
trabeculations with an increase in the thickness of the
compacted myocardium.
• compaction process coincides with the invasion of epicardial
coronary arteries and vascularization of the myocardium
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5. • progresses from-
• epicardium to the endocardium
• base to the apex
• septum to the free wall in the LV
• more in LV than right ventricle
• time of arrest of normal embryonic myocardial maturation
determines the severity and extension of LVNC
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8. • Left ventricular noncompaction cardiomyopathy rare
congenital disorder characterized by
1. Prominent LV trabeculae
2. Deep intertrabecular recesses communicate with venricular
cavity
3. Thin compacted layer
4. Noncompacted and compacted ratio >2 in end systole
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9. • persistent sinusoids
• describe ventriculo-coronary arterial communications or
intertrabecular spaces connecting the ventricular cavity with
the epicardial coronary artery system through the capillary
bed.
• seen in pulmonary atresia with intact ventricular septum.
• hypertrabeculation-
• increased number of normally formed trabeculations
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12. PREVALENCE
• In adults based on Echo ranges fom 0.014% to 1.3%
• In Australian children with cardiomyopathy prevalence higher-
5% to 9.2%.
• phenotype of noncompaction can vary even within familial
cases and range from clinically benign to fatal.
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13. Genetics of LVNC
• Sporadic and familial form.
• AD more common than X-linked inheritance
• Familial recurrence between 18 and 50%
• Mutations in the G4.5 gene on Xq28 resulting Barth syndrome
with DCM and LVNC in a pediatric population.
• Loci for LVNC were also described on chromosome 1, 5, and
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15. • Acquired NCLV-
• Young athlete
• Pregnancy
• Sickle cell anemia
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19. CLASSIFICATION
• AHA classifies LVNC as a genetic cardiomyopathy.
• ESC and WHO classify LVNC as an unclassified
cardiomyopathy.
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20. Diagnostic criteria-
• Normal variants-
• Boyd et al. reported the frequency and localization of
prominent LV trabeculations at autopsy in 474 normal hearts
• 53% of them exhibited two or more.
• More than three prominent trabeculations were observed in
only 3%.
• but none of the hearts had more than five.
• Most of the trabeculations (85%) were septomarginal bundles
inserting into both the free wall and the septum
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21. Non-compaction
(California criteria)
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23. Zurich criteria
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24. Vienna criteria
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26. Limitations of echocardiographic criteria
• poor correlation between three echocardiographic definitions
• 24% of the study population fulfilled one or more
echocardiographic definitions for LVNC.
• only 30% fulfilled all three criteria.
• 8% of apparently healthy individuals also satisfied one or
more diagnostic criteria for LVNC
• Contrast echocardiogram is useful to better image
intertrabecular spaces
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31. Cardiac magnetic resonance imaging
• Method of choice to confirm or rule out the diagnosis of LVNC
• The NC areas are most commonly found in the apical and
lateral portions of the left ventricle
• ratio of NC to C layers > than 2.3 at end diastole (Petersen et
al.)
• Trabeculated LV mass >20% of global LV mass (Jacquier et al.)
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Zuccarino et al. AJR:204 May 2015

34. • Differential diagnosis-
• Apical form of hypertrophic cardiomyopathy, a combination of
both apical hypertrophic cardiomyopathy and LVNC
• hypertensive cardiomyopathy
• endocardial fibroelastosis
• abnormal chords
• Apical thrombus, or tumours
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35. ASSOCIATED CONDITIONS
• Coronary cameral fistulas
• LVOT abnormalities
• Ebstein’s anomaly
• Bicuspid aortic valve
• Transposition of great vessels
• Metabolic diseases and genetic syndromes, including the
Barth syndrome, the Charco-Marie-Tooth disease
• Muscular dystrophy
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37. MANAGEMENT
• No specific treatment available
• Family members of proband should be screened using ECHO
• Genetic testing may useful in identifying familial forms
• Anticoagulation-(INR 2-3)
1. Decreased systolic function with EF below 40%
2. History of thromboembolism
3. Atrial fibrillation
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38. • ICD/biventricular pacing-
• no robust data available for guideline
• indication for device therapy as per current guideline.
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41. Take home message
• Many pt with trabeculae but normal LV function diagnosed
as NVLC.
• Should not labeled unless truly meet diagnostic criteria.
• Jenni criteria appear to best at present.
• Trabeculation may progress or change under physiologic
condition like pregnancy, serial evaluation is necessary.
• Difference in LV mass and trabecular feature between racial
background
• Screening of relative is crucially important.
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42. THANK YOU
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