Exploring Morphologic and Functional Variants in Hypertrophic Cardiomyopathy: An Echocardiographic and Doppler Review

Figures

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  Figure 1. Different distribution and location of hypertrophy in hypertrophic cardiomyopathy (HCM) (asterisks): (A) (parasternal long axis) and (B) (parasternal short axis) septal hypertrophy; (C) (parasternal short axis) concentric hypertrophy; (D) (parasternal short axis) lateral hypertrophy; (E) (parasternal long axis) and (F) (contrast echocardiography, apical four-chamber) apical hypertrophy; (G) (apical four-chamber) left ventricular (LV) and right ventricular apical hypertrophy; (H) (subcostal view) lateral hypertrophy. LA: left atrium.

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  Figure 2. Apical HCM with aneurysm. Typical Doppler patterns, contrast echocardiography and cardiac magnetic resonance (CMR) aid in the detection of apical aneurysms. (A) A typical continuous Doppler void pattern (early systolic high velocity towards the LV cavity—3.3 m/s, and early diastolic paradoxical flow—2.2 m/s, known as the ‘lobster claw’ abnormality). (B) Two chamber view with a clear detection of the aneurysm (An). (C) CMR (cine four-chamber view) showing a small aneurysm and (D) same case as demonstrated by contrast echocardiography. (E) CMR (cine three-chamber view) of a larger aneurysm. (F) CMR (late gadolinium enhancement three-chamber view sequence) showing transmural apical scarring (arrows). An: aneurysm.

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  Figure 3. Apical HCM with aneurysm. Modified apical views may be necessary to identify the site of mid-ventricular LV obstruction and confirm the systolic expansion of the apical aneurysm. In this case the Doppler (A) demonstrates an early systolic and early diastolic paradoxical flow (arrows). (B) The colour Doppler facilitates the identification of the mid-ventricular LV obstruction. (C) An adapted two-chamber view shows the obstruction (arrow) and the apical aneurysm (An). Diastolic (D) and systolic (E) frames of the same case (four-chamber view) and diastolic (F) and systolic (G) frames (two-chamber view) demonstrating expansion of the apical cavity in systole.

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  Figure 4. (A) Mid-systolic closure of the aortic box. (B) M-mode of the mitral valve with systolic anterior movement (SAM). (C) Left ventricular outflow tract (LVOT) obstruction with a typical dagger shape morphology. (D) Three-chamber long axis transesophageal echocardiography (TEE) view; the colour Doppler demonstrates the LVOT high velocity and the coexistence of mitral regurgitation (MR) due to SAM. Lower panels: how to calculate and validate the correct LVOT gradient from the MR gradient and manometric blood pressure (scheme of the simple test that may confirm the accuracy of the LVOT gradient measurement). Because the MR gradient reflects the difference between LV systolic pressure and left atrial pressure, it can be used to estimate LV systolic pressure when left atrial pressure is known or assumed. Simultaneously, the cuff-measured systolic blood pressure reflects aortic pressure, provided there is no significant aortic stenosis or LVOT obstruction. In HCM, the difference between the estimated LV systolic pressure and systolic blood pressure should equal the Doppler-derived LVOT gradient. If it does not, the LVOT gradient may be inaccurate, commonly underestimated due to poor…

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  Figure 5. Anomalies of the mitral valve (MV) apparatus. (A) Parasternal long axis view showing an anteriorly displaced and hypertrophied papillary muscle (asterisk). (B,C) Parasternal short axes of the LV: a hypertrophic anterolateral papillary muscle (B, asterisk) and a bifid one (C, arrows). (D) TEE showing the insertion of a head of the anterolateral papillary muscle directly into the mid portion of the anterior mitral valve leaflet (arrow). (E) Four-chamber apical view showing an abnormal apical insertion of the papillary muscle that is also anteriorly displaced (arrows). (F) Three-chamber apical view that allows the measurement of a long anterior mitral leaflet (yellow bar); asterisk showing the anterolateral papillary muscle.