[Non-compaction cardiomyopathy]

A 49-year-old man with loss of performance, ventricular ectopy and left bundle branch block was referred for diagnostic workup. He had dysmorphic skeletal features with shortening and muscular atrophy of arm and leg and syndactylia.

Echocardiography revealed peculiar hypertrabeculation of left ventricular myocardium which was confirmed by cardiac magnetic resonance imaging. Therefore a diagnosis of non-compaction cardiomyopathy was made. Holter monitoring showed non-sustained ventricular tachycardia, coronary heart disease was excluded by coronary angiography. TREATMENT AUND COURSE: The patient received optimal heart failure drug therapy. Because of malignant ventricular ectopy a biventricular ICD system (CRT-D system) was implanted. Signs and symptoms of heart failure have been stable for four years (NYHA-class II). Repeated sustained ventricular tachycardia were terminated by ICD overstimulation. A history of inadequate ICD shocks was due to incompliance of the patient regarding beta blocker use.

Non-compaction cardiomyopathy is a rare but characteristic cause of heart failure which is associated with potential malignant arryhthmias. Diagnosis is based on echocardiographic workup with typical features. However, because of its low prevalence and awareness deficits in the medical community, non-compaction cardiomyopathy probably is diagnosed too late and not frequently enough.