So, What exactly is Non Compaction?

Non Compaction is currently considered an "unclassified" cardiomyopathy.  Many patients are specifically diagnosed with Left Ventricular Non Compaction (LVNC) however non compaction can affect other portions of the heart muscle.  The affected heart muscle is thickened by deep trabeculations (or valleys).  The non-compaction actually occurs at 20 weeks in utero as the heart is forming.  It is considered a congenital cardiomyopathy.

Symptoms can include shortness of breath, exercise intolerance, and reduced ejection fraction (EF).  Patients with Non Compaction are considered to be at a greater risk for developing blood clots due to the blood retained in the heart.

Current research studies have shown patients responding well to therapy with specific drugs such as certain ACE inhibitiors and BETA blockers, especially carvedilol.  These drugs have been shown to help regenerate the heart muscle, reduce in size the thickened heart wall, and to improve ejection fraction.  In some cases EF can be restored to normal levels (60% or better).  Aspirin therapy (81 mg/day is typically recommended to help reduce the risk of blood clots.  Some treatments do include actual blood thinning medications depending on the patients individual condition.  In some cases where the heart muscle is severely weakened a heart transplant may be necessary.

Non Compaction has typically been associated with a high mortality rate but that outlook is beginning to change.  With more advanced technology in echo cardiogram and MRI as well as a heightened awareness of this particular type of cardiomyopathy more and more cases are being diagnosed early and correctly.   Many studies have shown that patients with NCC or LVNC have an increased risk of sudden cardiac death due to ventricular fibrillation even if the patient has no documented arrythmia.  Patients may receive implantation of an ICD (implantable cardioverter-defibrillator) to prevent sudden cardiac death.