left ventricular non-compaction
DESCRIPTION
Brief introduction to LV non-compaction, a cardiomyopathy still have much mysteriesTRANSCRIPT
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