Beyond Hypertrophy: A Case of Mid-ventricular Obstructive Hypertrophic Cardiomyopathy With Apical Sequestration

Figures

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  Figure 1. Parasternal long-axis view demonstrating ventricular hypertrophy. Parasternal long-axis (PLAX) transthoracic echocardiographic image showing marked left ventricular hypertrophy involving the mid-ventricular segments. Echogenicity is suboptimal, limiting precise visualization of the apical region, but the degree of hypertrophy is clearly appreciable. This pattern is suggestive of obstructive hypertrophic cardiomyopathy phenotype.

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  Figure 2. Continuous-wave Doppler revealing a systolic mid-cavity “saber-shaped” signal. Continuous-wave Doppler tracing recorded from the apical window showing a characteristic systolic mid-ventricular “saber-shaped” flow signal, reflecting a high-velocity gradient within the mid-cavity. This pattern is typical of mid-ventricular obstruction in hypertrophic cardiomyopathy.

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  Figure 3. Holter monitor recording demonstrating non-sustained ventricular tachycardia. Twenty-four-hour Holter monitor strip showing an episode of NSVT consisting of several beats of a rapid monomorphic ventricular rhythm before spontaneous termination. NSVT: non-sustained ventricular tachycardia; VEB: ventricular ectopic beats (premature ventricular complexes).

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  Figure 4. Two-chamber cine view demonstrating mid-ventricular hypertrophy. Cine steady-state free precession (SSFP) two-chamber view showing marked mid-ventricular hypertrophy with dynamic systolic narrowing of the mid-cavity, resulting in partial functional exclusion of the left ventricular apex. This configuration is characteristic of mid-ventricular obstructive hypertrophic cardiomyopathy.

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  Figure 5. Three-chamber LGE image showing hypertrophic septum fibrosis, apical ischemia, and thrombus. Three-chamber late gadolinium enhancement (LGE) sequence demonstrating transmural enhancement of the apical wall, consistent with ischemic injury within the functionally isolated, sequestrated apex. Intramural enhancement is present in the mid-ventricular septal segments, reflecting fibrosis within the hypertrophied myocardium. The apex, although isolated, does not exhibit features of a true aneurysm, as the apical myocardium remains thick and without focal thinning. A small crescent-shaped apical mural thrombus is visualized as a dark signal void adjacent to the enhanced apical myocardium.