Isolated non compaction of ventricular myocardium
Enrico Hoffmann a ,Alessia Pepe a ,Massimo Midiri b
Departments of a Cardiology and b Radiology, Medical Faculty, University of Palermo, Italy.
We report two cases of isolated non compaction of the ventricular myocardium (INVM). The first case is a 36 year old man referred to our department because of recurrent syncope and ventricular arrhythmias. The transthoracic echocardiogram (TE) did not contribute to the diagnosis.Cardiac catheterization disclosed grossly and irregularly trabeculated segments along the diaphragmatic, para-apical and postero-lateral regions and a diverticular-like image with systolic retention of contrast medium (Fig.1A). The subsequently performed transesophageal echocardiogram (TEE) revealed an uncertain area of apical irregularity similar to "spongy myocardium" The cardiovascular magnetic resonance (CMR) confirmed deep recesses and prominent trabeculations within the left ventricular wall (Fig.1B).
The second case is a 37 year old man admitted to our department due to symptoms of heart failure.At the age of 32 years he was diagnosed with a dilated cardiomyopathy and a myopathy on unknown origin. On admission the TE, and subsequently TEE,disclosed a left ventricle globally and severely hypocinetic with prominent trabeculations and deep intertrabecular recesses along the postero-lateral wall,especially in the para-apical region (Fig.1C). The coronarography was normal. The CMR confirmed deep recesses and prominent trabeculations within the left ventricular wall,mainly located in the postero-lateral region (Fig.1D).
Although the echocardiography is currently the diagnostic modality of choice for INVM, ventricular angiography and mainly CMR may be necessary to rule out the diagnosis of INVM.In this phase the evidence of clinical cases will probably make faster the classification of INVM as a specific cardiomyopathy.
Figure legend
Fig.1 A, Left ventricular angiogram (left anterior oblique projection)showing the perfusion of deep intertrabecular recesses along the infero-posterior wall and diverticular-like images with systolic retention of contrast medium in the para-apical segment. B, Short-axis T1-weighted spin-echo magnetic resonance imaging through the heart (apical level) showing deep intertrabecular recesses within the left ventricular wall. C, Transesophageal echocardiography:short axis view of left ventricle at the level of the papillary muscles showing multiple prominent trabeculations and deep intertrabecular recesses along the infero-postero-lateral wall. D, Short-axis T1-weighted spin-echo magnetic resonance imaging through the heart (apical level) showing deep intertrabecular recesses within the left ventricular wall,mainly located in the postero-lateral region.
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