left ventricular non-compaction

Left ventricular noncompaction (LVNC) has been recently proposed as a specific form of cardiomyopathy. There have been few pathological series describing gross and microscopic findings of this entity, especially in children. LVNC is frequently associated with other cardiac defects, especially when causing sudden death in infants and children.

Types

 isolated LVNC
 LVNC associated with congenital heart disease

Synopsis

 poorly developed left ventricular (LV) papillary muscles
 noncompact inner LV myocardial layer comprising more than 50% of the LV thickness
 mean age at death/explant was 3.6 years (median, 2.5 months)

Complications

 unexpected death
 heart failure
 endocardial fibroelastosis
 anastomosing or polypoid endocardial trabeculations
 staghorn-shaped, endocardial-lined recesses

Associations

 ventricular septal defect
 anomalous venous pulmonary veins
 coronary ostial stenosis
 histiocytoid cardiomyopathy
 polyvalvar dysplasia
 pulmonary stenosis
 malformed atrioventricular valves

Echographic differential diagnosis

 dilated cardiomyopathy
 hypertrophic cardiomyopathy
 restrictive cardiomyopathy
 endocardial fibroelastosis
 endomyocardial fibrosis
 myocarditis
 thrombus
 localised left ventricular hypertrophy
 left ventricular mass
 myocardial/pericardial disease
 false tendons
 aberrant bands
 thrombi
 apical hypertrophic cardiomyopathy
 fibroma
 obliterative processes
 intramyocardial hematoma
 cardiac metastases
 intramyocardial abscesses

References

 Stollberger C, Finsterer J. Pitfalls in the diagnosis of left ventricular hypertrabeculation/non-compaction. Postgrad Med J. 2006 Oct;82(972):679-83. PMID: 17068279

 Burke A, Mont E, Kutys R, Virmani R. Left ventricular noncompaction: a pathological study of 14 cases. Hum Pathol. 2005 Apr;36(4):403-11. PMID: 15892002