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Left

Ventricular
noncompaction
RESIDENT

LV noncompaction(LVNC)

LV noncompaction(LVNC)
A morphologic description of the LV with
excessive trabeculation
=Left ventricular hypertrabeculation

Nature 415, 227-233(10 January 2002)

Pathogenesis

Clinical manifestation

Definition of LVNC
Identified as a distinct entity over the past few
decades.
American Heart Association: Primary Genetic
Cardiomyopathy
European Society of Cardiology: Unclassified
Cardiomyopathy
may be a morphological manifestation of
several distinct cardiomyopathies

Echo

Frequent Zone

Diagnosis
Poor agreement between the various diagnostic
criteria
Absence of a non-pathological gold standard
Preponderance towards overdiagnosis

Echo and Cardiac MRI criteria

Stllberger criteria
The presence of multiple echocardiographic
trabeculations
Multiple deep intertrabecular recesses
communicating with the ventricular cavity, and
the recesses demonstrated in the apical or
middle portion of the ventricle
A 2-layered structure of the endocardium with a
noncompacted to compacted ratio >2 (1.4?)

Diagnosis Algorithm

Phenotype
Isolated
Associated with congenital heart disease
LVNC had been identified in patients with
neuromuscular disorders (eg, Charcot-Marie-Tooth
disease type 1A) , mitochondrial disorders ,
metabolic diseases, and genetic syndromes,
including Barth syndrome, Melnick-Needles
syndrome, and nail-patella syndrome
non-isolated LVNC may be found in combination
with septal defects, pulmonic stenosis or
hypoplastic left ventricle

Genetics

Arrhythmias
Most Common :
Ventricular tachycardia (VT) 0~9% sustained VT
Atrial fibrillation (AF) 7~9 %

Systemic thromboembolism
When matched with controls with a similar degree
of LV systolic dysfunction, there was no difference
in the rates of systemic thromboembolism
It appears that the main risk factor for
thromboembolic events in patients with LVNC is
the severity of the underlying systolic dysfunction
Data on asymptomatic patients with preserved
systolic function, no thromboembolic events were
reported during follow-up

Managemnt
No guidelines for the management of patients with
LVNC exist
Genetical and neurological referral
Family counseling and screening
Asymptomatic with normal LV systolic function:
Followed every 23 years with echo and Holter
monitoring
Asymptomatic with LV systolic/diastolic dysfunction:
Heart failure therapy, followed every 1-2 years
Symptomatic patient: Heart failure guideline

Management Algorithm

Heart failure Guideline


Beta-blockers
ACE inhibitors/ARB
Diuretics
Aldosterone antagonists
Implanted automated defibrillators are recommended in those
with EF<35% or those with previously life threatening
arrhythmias
lifelong systemic anticoagulation is indicated to obviate risk for
thromboembolism
12% of patients with LVNC may go on to develop end stage
heart failure and require orthotopic heart transplantation

Summary