left ventricular non-compaction

Left Ventricular non-compaction

RESIDENT 楊宗儒

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 Cardiomyopathymay 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

Stöllberger 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 2–3 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