Saw-tooth cardiomyopathy

Abstract

We present an unusual case of cardiomyopathy in a two month old male infant with a grade-I systolic murmur. Echocardiographic examination disclosed left ventricular (LV), dysplasia with saw-tooth like inwards myocardial projections extending from the lateral walls towards the LV cavity. There was mild LV systolic dysfunction with apical hypokinesia. Cardiovascular magnetic resonance demonstrated in detail these cross bridging muscular projections originating from the inferior interventricular septum and lateral LV wall, along with areas of hypokinesis at the LV septum and apex in a noncoronary distribution, without any late gadolinium enhancement. We have termed this condition saw-tooth cardiomyopathy because of the very characteristic appearance.

Figure 1

Echocardiogram. Apical 4-chamber view with the transducer angulated towards the inferior LV wall in diastole (A) and systole (B). There are numerous echo dense saw tooth like inwards projections from the inferior interventricular septum and lateral LV wall, clearly seen during both systole and diastole. LV apical dyskinesia was also noted.

Figure 2

CMR. End-diastolic (panels A, C,), and end-systolic (panels B, D) images in the two-chamber and four-chamber orientations, from a free-breathing gradient-echo cine CMR sequence. There are numerous saw-tooth like muscular projections originating from the inferior LV wall, some of them being tethered to the interventricular septum. These myocardial projections have a circular architecture, like successive open rings at the inferior half of the ventricle, spanning the entire heart from the base to the apex.