Noncompaction cardiomyopathy differential diagnosis

NCC is often missed or misdiagnosed. This can be attributed at least in part to the fact that non-compaction cardiomyopathy was only recently reported as an entity.

The normal heart can have prominent trabeculations, but these usually number less than 3.

In a recent study ^[1] of 53 patients with non-compaction cardiomyopathy, the first 42 were misdiagnosed with another form of heart disease. Improved awareness and improved imaging modalities allowed the correct diagnosis to be made in the last 11 cases. The most common misdiagnoses included:

• 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

Given the apical hypertrophy, the disease may also be mistaken for hypertrophic obstructive cardiomyopathy or apical hypertrophic cardiomyopathy. It can also be confused with arrhythmogenic right ventricular dysplasia, endocardial fibroelastosis, cardiac metastases, and left ventricular thrombus.

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