PMC4720816_01
Isolated left ventricular apical hypoplasia in 34-year-old woman.. A. Echocardiogram demonstrates spherical and truncated LV with echo-bright apex (arrow), bulging of interventricular septum towards right ventricle, and elongated RV wrapping around deficient LV apex in parasternal long-axis view. B. Four-chamber view from CMR during diastole and systole shows spherical, truncated LV at apical level with prominent fat (arrows). Papillary muscles originate from flattened anterior apex. RV is elongated and wraps around deficient LV apex. Late gadolinium enhancement revealed no enhancement in apex and ventricular septum of LV. C. Four-chamber view from cardiac MDCT more clearly visualizes extent of fatty material within apical myocardium, interventricular septum and inferior LV wall. Almost all fatty material was confined within myocardium, although small portion of apical fat was contiguous with epicardial fat (arrow). MDCT clearly shows cardiac layers and fat distribution. Fatty tissue is confined within thin myocardial layers of apex and interventricular septum. Note pericardium (arrowheads) and epicardial fat. CMR = cardiac magnetic resonance, LA = left atrium, LV = left ventricle, MDCT = multidetector computed tomography, RV = right ventricle, VR = volume-rendering
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There was a grade II diastolic dysfunction and elevated left ventricular end-diastolic pressure (Fig. 1A). Cardiac magnetic resonance revealed a spherical and truncated LV with rightward bulging of the interventricular septum, as well as prominent fat presence at the apex (Fig. 1B, arrows). There was no delayed myocardial enhancement to indicate myocardial fibrosis or mass (Fig. 1B). The extent to which the fatty material replaced the deficient apical myocardium, interventricular septum and LV inferior wall was better seen on an MDCT (Fig. 1C). Almost all the fatty material was confined within the myocardium; only a small portion of the apical fat was contiguous with epicardial fat (Fig. 1C, arrow).