New Cardiomyopathy
New Cardiomyopathy
Abstract & Commentary
By Michael H. Crawford, MD, Editor
Source: Starmer G, et al. Multimodality imaging of isolated left ventricular apical hypoplasia. Eur Heart J 2012;33:675.
These investigators from Australia present a case of isolated left ventricular (LV) apical hypoplasia with fatty infiltration confirmed by CT and MRI. The left anterior descending artery was small and truncated early on angiography. Echocardiography showed a large banana-shaped right ventricle (RV) that wrapped around the apex of the hypocontractile truncated LV. Also there was apical papillary muscle insertion.
Commentary
Isolated apical LV hypoplasia is a new cardiomyopathy that has been described in about 10 individuals between the ages of 3 to 50 years. Although it can be fatal, most of the reported patients responded to therapy for systolic heart failure and have done relatively well. This case highlights the value of multimodality imaging for identifying the cardinal features of this cardiomyopathy: 1) a truncated spherical LV; 2) fatty infiltration of the LV apex; 3) the banana-shaped RV; 4) apical papillary muscle insertion; and 5) a hypoplastic truncated LAD.
These investigators from Australia present a case of isolated left ventricular (LV) apical hypoplasia with fatty infiltration confirmed by CT and MRI. The left anterior descending artery was small and truncated early on angiography. Echocardiography showed a large banana-shaped right ventricle (RV) that wrapped around the apex of the hypocontractile truncated LV. Also there was apical papillary muscle insertion.Subscribe Now for Access
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