Noncompaction cardiomyopathy

Non-compaction cardiomyopathy is a rare congenital cardiomyopathy that affects both children and adults. It refers to a type of cardiomyopathy where the myocardial development is hindered while the baby is in the womb during the embryogenesis stage. This hindrance begins to occur around the 8 week mark where previous the myocardium represented a sponge in the sense it was a meshwork of interwoven myocardial fibers. This is beneficial at this stage as there is an increased surface area which allows perfusion of the myocardium from the left ventricular cavity. However as the embryo grows the myocardium should begin to be compact, in this case though the heart fails to fully compact leaving the myocardium to still have a spongy form.

Noncompaction of ventricular myocardium is recently included in the 2006 classification of cardiomyopathies as a genetic cardiomyopathy.

Description

Non-compaction Cardiomyopathy can affect both ventricle walls of the heart separately or together however it generally only affects the left ventricle wall and therefore is sometimes known as left ventricular noncompaction. Due to its recent diagnosis and it being an unclassified Cardiomyopathy according to the WHO, it is not fully understood how common the condition is. Some reports suggest that it is in the order of 0.12 cases per 100,000.

Again due to the recent nature of the condition diagnosis is not definitive and there are on going discussions as to various points such as the ratio of compacted to non-compacted at various age stages. However it is universally understood that non-compaction Cardiomyopathy will by characterized anatomically by deep trabeculations in the ventricular wall, which define recesses communicating with the main ventricular chamber. Major clinical correlates include systolic and diastolic dysfunction, associated at times with systemic embolic events. (Weiford et al.2004)

Miss Diagnosis

In a recent study carried out on 53 patients with the condition in New York, 42 had been diagnosed with a another form of heart disease and only in the most recent 11 cases that ventricular noncompation was diagnosed and this took several echocardiograms to confirm. The most common miss diagnosis were

• dilated cardiomyopathy: 30 Cases
• congenital heart disease: 6 Cases
• ischemic heart disease : 2 Cases
• disease of the heart valves: 2 Cases
• dilated phase hypertensive cardiomyopathy: 1 Case
• restrictive cardiomyopathy: 1 Case

The high number of miss diagnosis can be attributed due to Noncompaction Cardiomyopathy only being first reported in 1990, and so diagnosis is often overlooked or delayed. Advances in medical imaging equipment has made it easier to diagnosis the problem in particle the wider use of MRI's

Images

• 
  Two-dimensional apical four chamber and parasternal short axis images at the level of the ventricles show dilatation of both ventricles, multiple trabeculae and intertrabecular recesses in inferior, lateral, anterior walls, middle and apical portions of the septum and apex of the left ventricle. [1]
• 
  Transthoracic two-dimensional study with color and continuous wave Doppler shows left ventricular noncompaction associated with patent ductus arteriosus (PDA). [2]
• 
  Transthoracic two-dimensional echocardiogram in apical four chamber and parasternal short axis at the level of both ventricles demonstrate dilatation, deep trabeculae and intertrabecular recesses in the inferior, lateral, anterior walls, middle and apical portions of the septum and apex of the left ventricle. [3]
• 
  Two-dimensional parasternal and color Doppler images at the level of both ventricles that show the noncompacted:compacted wall ratio and how the color enters the intertrabecular recesses [4]
• 
  Cross section of the Heart

References

• "Non-compaction of Myocardium Cardiomyopathy". Yale University. Retrieved June 13. {{cite web}}: Check date values in: |accessdate= (help); Unknown parameter |accessyear= ignored (|access-date= suggested) (help)
• "Cardiomyopathy Caused by Isolated Noncompaction of the Left Ventricle in Adults". Medscape Cardiology. Retrieved June 13. {{cite web}}: Check date values in: |accessdate= (help); Unknown parameter |accessyear= ignored (|access-date= suggested) (help)
• "Non-compacted Cardiomyopathy: Clinical-Echocardiographic Study". Medscape Cardiology. Retrieved June 13. {{cite web}}: Check date values in: |accessdate= (help); Unknown parameter |accessyear= ignored (|access-date= suggested) (help)
• "Left Ventriuclar noncompaction" (PDF). Orphanet. Retrieved June 14. {{cite web}}: Check date values in: |accessdate= (help); Unknown parameter |accessyear= ignored (|access-date= suggested) (help)
• "Left Ventricular Non-compaction". Baylor College of Medicine. Retrieved June 15. {{cite web}}: Check date values in: |accessdate= (help); Unknown parameter |accessyear= ignored (|access-date= suggested) (help)
• "Contemporary Definitions and Classification of the Cardiomyopathies". American Heart Association Scientific Statement. Retrieved June 15. {{cite web}}: Check date values in: |accessdate= (help); Unknown parameter |accessyear= ignored (|access-date= suggested) (help)
• "The Failing Heart". Nature. Retrieved June 15. {{cite web}}: Check date values in: |accessdate= (help); Unknown parameter |accessyear= ignored (|access-date= suggested) (help)
• "Noncompaction of the left ventricle: a new cardiomyopathy is presented to the clinician". Sao Paulo Medical Journal. Retrieved June 15. {{cite web}}: Check date values in: |accessdate= (help); Unknown parameter |accessyear= ignored (|access-date= suggested) (help)

• Weiford BC, Subbarao VD, Mulhern KM. 2004. Noncompaction of the ventricular myocardium. Circulation 109(24):2965-71.