Non Compaction of the LV

Fig. 1. Parietal views of sagittally dissected human embryonic left ventricles showing the process of normal trabecular compaction. (A) Abundant fine trabeculations are present at 6 weeks. (B) The trabeculations start to solidify at their basal area, contributing to added thickness of the compact layer at 12 weeks. (C) The compact layer forms most of the myocardial mass after completion of compaction in the early fetal period.
Reprinted from [46] with permission from BMJ Publishing Group Ltd.
ikeda et al Isolated left ventricular non-compaction cardiomyopathy in adults Journal of Cardiology
Volume 65, Issue 2, February 2015
  • Non-compaction of the left ventricle,
    aka
    • spongiform cardiomyopathy or
      left ventricular non-compaction (LVNC) 
  • is a congenital abnormality
  • caused by
    • embryological arrest of normal myocardial compaction of
  • hypertrophic ventricular trabeculations and
  • deep interventricular recesses.
    • acquired cases have also been reported.

 

 

  • Cardiac MRI
    • modality of choice
    • Size
        • ratio of non-compacted telediastolic myocardium to compacted myocardium of
          • more than 2.3:1 (sensitivity: 86%, specificity: 99%).
    • Position
      • predominantly affects the
        • inferolateral walls and the
        • apex

          LGE in NON COMPACTION
          Short-axis precontrast (A) and delayed enhancement (B) views of a patient with noncompaction cardiomyopathy. On precontrast view, non-compacted layer of LV myocardium is visible. Late gadolinium enhancement sequence demonstrated fibrosis of trabecules of non-compacted layer.
          Panovsky et al The prognostic impact of myocardial late gadolinium enhancement.
          Cardiology in Review · May 2014

Other commonly associated features include:

  • left ventricular dysfunction
    • both systolic and diastolic
  • intraventricular thrombus
  • left atrial enlargement
    • associated with atrial fibrillation

 

The only definitive treatment of left ventricular non-compaction is cardiac transplantation. Otherwise, prevention of both heart failure and thromboembolic events are the main target of treatments.

Possible differential considerations in certain situations include: