Left ventricular non-compaction cardiomyopathy (LVNC) is a rare genetic heart condition characterized by a thickened myocardium with prominent trabeculations. It occurs when the myocardium fails to compact properly during fetal development. LVNC can be isolated or associated with other heart defects, and presents variably from being asymptomatic to causing heart failure, arrhythmias, blood clots, or sudden cardiac death. Diagnosis is made through echocardiogram, cardiac MRI, or CT scan finding an excessive trabeculated myocardium. Treatment focuses on managing complications like heart failure or arrhythmias. Prognosis depends on symptoms and ejection fraction, with mortality over 40% at 5 years in symptomatic patients.

1. Left ventricular non-compaction cardiomyopathy (LVNC)
Ahmed ElBorae, MSc
Assistant lecturer of Cardiology, Cairo University

2. Introduction
• (LVNC) is a rare form of genetic cardiomyopathy characterized by predominant trabecular
sub-endocardial layer at the expense of compacted outer myocardium
• Pathogenesis: Arrest of compaction of inter-trabecular recesses of myocardium , which
normally occur at 8 weeks of gestation (coincides with the invasion of the epicardial coronary
arteries)
Eur Heart J, Volume 32, Issue 12, June 2011, Pages 1446–1456

3. Introduction
• Incidence: Variable 1.3% by Echo to 12 % by CMR ,might be isolated or associated with
various cardiac anomalies or cardiomyopathies in 5%, male > female
• Etiology: Two forms;
- familial > often children> (Autosomal or X-linked, or mitochondrial) > mutation of the G4.5
gene chromosomal 28
- sporadic often adults > mostly sarcomere gene mutation, 40 genes identified (MYH7 40%)
• Clinical presentation: Asymptomatic ,or wide range of symptoms including (Heart failure,
arrhythmia, thrombo-embolism, and SCD)
European Heart Journal, Volume 32, Issue 12, June 2011

4. Diagnosis
• Investigations: Echo various criteria (Non-compacted/compacted > 2 ”Jenni Def.”),
CMR (>2.3)  higher diagnostic yield, LGE is prognostic, exclude mural thrombus,
> 2.2 in MSCT
- Genetic testing: Especially in the presence of positive family history
• D.D: Similar morphology could be observed in patients with chronically
increased left ventricle pressure, athletes, and pregnancy
• Risk stratification: Geno-positive cases carries higher rate of SCD and need for
transplantation, presence of LGE in CMR also carries poorer prognosis
2021 Afify et al. Cureus 13(5): e15112. DOI 10.7759/cureus.15112

5. ESC Heart Failure · December 2021, DOI: 10.1002/ehf2.13694

6. Management
• Treatment:
- No specific TTT, just dealing with complications e.g. GDMT of HF up to device therapy
and transplantation
-Family screening by at least echocardiography is recommended
• Prognosis:
- Depends on symptoms and EF%, mortality > 40% at 5 years in symptomatic patients
-Isolated apical LVNC and preserved EF% have similar outcomes to the general
population
ESC Heart Failure · December 2021, DOI: 10.1002/ehf2.13694

7. Incidence of thromboembolism was 15%
25% were AF, even if CHADVASC 0
70% have LV dysfunction
Anticoagulation ?

8. Thank You
Lower vertebrate normally have LV non-compaction !
Zebra fish heart Frog heart