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GUIDELINES Source

  • Nagueh SF, Bierig SM, Budoff MJ, et al. American Society of Echocardiography clinical recommendations for multimodality cardiovascular imaging of patients with hypertrophic cardiomyopathy. J Am Soc Echocardiogr. 2011;24:473–498.

  • Echocardiographic evaluation is compared to other imaging modalities.

  • Myocardial strain.

  • Diastolic function.

  • Imaging in the pediatric population.

Diagnosis, Genetic Testing, and Deformation Patterns Sources
  • Binder J, Ommen SR, Gersh BJ, et al. Echocardiography-guided genetic testing in hypertrophic cardiomyopathy: septal morphological features predict the presence of myofilament mutations. Mayo Clin Proc. 2006;81:459–467.

  • Gersh BJ, Maron BJ, Bonow RO, et al. 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy. Circulation. 2011;124:2761–2796.

  • Geske JB, Bos JM, Gersh BJ, et al. Deformation patterns in genotyped patients with hypertrophic cardiomyopathy. Eur Heart J Cardiovasc Imaging. 2014;15:456–465.

  • Morphology of the interventricular septum was categorized as:

    • - Sigmoid, neutral, apical, and reverse curve.

ECHOCARDIOGRAPHIC FINDINGS

  • This is a genetic disorder of the heart. Family members also may be affected. There is a risk of sudden death.

  • Echocardiographic findings:

    • - Asymmetric left ventricular hypertrophy (Fig. 14-1).

    • - Dynamic left ventricular outflow obstruction (Fig. 14-2).

    • - Mitral systolic anterior motion (Fig. 14-3).

    • - “Depressurizing” mitral regurgitation.

    • - Diastolic relaxation is impaired.

    • - Preserved systolic left ventricular function.

FIGURE 14-1

Asymmetric basal septal hypertrophy.

FIGURE 14-2

Mid-systolic aortic valve closure in dynamic left ventricular outflow obstruction.

FIGURE 14-3

Systolic anterior motion of the mitral valve.

MITRAL REGURGITATION IN HYPERTROPHIC CARDIOMYOPATHY

  • Start by demonstrating systolic anterior motion of the mitral valve on 2D.

  • Color flow should then confirm the sequence of:

    • - Ejection-obstruction-regurgitation (manifested on 2D by the SAM).

  • Characteristic Doppler features of this jet:

    • - Direction: “opposite the culprit leaflet”—since the systolic motion is anterior, the direction of the jet is posterior.

  • Timing:

    • - The predominant regurgitant flow is mid-to-late systolic (rather than holosystolic).

    • - The CW envelope has a characteristic “late-systolic-dagger” shape. Ejection starts, obstruction occurs, and regurgitation peaks in mid to late systole.

  • Velocities:

    • - Since the regurgitation into the left atrium is “depressurizing” the dynamic left ventricular outflow tract obstruction, peak mitral regurgitation velocities are increased.

    • - Instead of the usual 5 m/s, they may exceed 6 m/s (Fig. 14-4).

  • Caution:

    • - It is psychologically tempting to make the amateur mistake of confusing a 7 m/s mitral regurgitation velocity with an unrealistic 196 mm Hg outflow gradient. 4 × 7 × 7 = 196 is mathematically easy, tempting to calculate, but wrong as an outflow gradient. It is too high. Dynamic left ventricular outflow gradients rarely exceed 100 mm Hg.

    • - The true outflow gradient in a patient with ...

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