Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content ++ 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. Graphic Jump LocationView Full Size||Download Slide (.ppt) ++ FIGURE 14-2 Mid-systolic aortic valve closure in dynamic left ventricular outflow obstruction. Graphic Jump LocationView Full Size||Download Slide (.ppt) ++ FIGURE 14-3 Systolic anterior motion of the mitral valve. Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ 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 a mitral regurgitation velocity ... Your Access profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth