Multimodality Imaging Demonstrating an Apical Variant Hypertrophic Cardiomyopathy in an Uncommon Pentad

Figures

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  Figure 1.. Three-chamber view reconstructions from diastolic (A) and systolic (C) phases of an electrocardiogram-gated contrast-enhanced cardiac computed tomography demonstrate apical-variant hypertrophic cardiomyopathy with apical pouch-aneurysm. Volume-rendered reconstructions of the left ventricular blood pool during diastole (B) and systole (D) demonstrate obliteration of the mid-ventricular cavity during systole (arrow).

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  Figure 2.. Transthoracic echocardiography: 2-chamber systolic frame (A) showing apical hypertrophy with apical aneurysm (pouch) and mid-cavitary obstruction. Parasternal long axis view (B) shows left ventricle (LV) apical hypertrophy measuring 20 mm. Two-chamber view of LV (C, left) shows “Ace of spades” morphology during systole due to apical hypertrophic cardiomyopathy; color Doppler shows mitral regurgitation (C, right). Two-chamber systolic view (D) shows apical and mid-cavitary pressure gradient with apical and mid-cavitary peak flow of 2, 3 m/s, respectively.

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  Figure 3.. Continuous wave spectral Doppler (A) shows 3 signals: mitral regurgitation peak velocity (6.1 m/s), mid-cavitary peak velocity (3 m/s), and apical left ventricle peak velocity (2 m/s). Polar map of 17 segments strain (B) shows marked reduction (−9.7%) with marked apical and mid-segment involvement. Ascending aortic aneurysm with midlevel diameter of 48 mm (C).

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  Figure 4.. Continuous wave spectral Doppler (3 months postoperatively) shows 3 signals: MR peak velocity (6 m/s), mid-cavitary peak velocity (3 m/s), and apical LV peak velocity (2 m/s).

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  Figure 5.. Continuous wave spectral Doppler (11 months postoperatively) shows 3 signals: mitral regurgitation peak velocity (5.9 m/s), mid-cavitary peak velocity (2.8 m/s), and apical left ventricular peak velocity (2 m/s).

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  Figure 6.. Cross-sectional computed tomography angiography of the chest (11 months postoperatively) showing stable ascending aortic aneurysm with a diameter of 46.6 mm.