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Review

Hypertrophic cardiomyopathy: A complex disease

Laura Young, MD, Nicholas G. Smedira, MD, Albree Tower-Rader, MD, Harry Lever, MD and Milind Y. Desai, MD
Cleveland Clinic Journal of Medicine May 2018, 85 (5) 399-411; DOI: https://doi.org/10.3949/ccjm.85a.17076
Laura Young
Robert and Suzanne Tomsich Department of Cardiovascular Medicine, Heart and Vascular Institute, Cleveland Clinic
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Nicholas G. Smedira
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Albree Tower-Rader
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Harry Lever
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Milind Y. Desai
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    Figure 1

    A, echocardiography, apical 4-chamber view, demonstrates septal hypertrophy (arrow). B, cardiac magnetic resonance imaging of the left ventricular outflow tract also demonstrates septal hypertrophy (arrow). C, echocardiography with continuous-wave Doppler across the left ventricular outflow tract demonstrates a gradient of 70 mm Hg, consistent with obstruction. D, electrocardiography reveals signs of left ventricular hypertrophy by Sokolov-Lynon criteria with S wave depth in V1 plus R wave height in V5 > 35 mm (arrows).

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    Figure 2

    A, echocardiography, apical 4-chamber view, shows apical hypertrophy (arrows). B, cardiac magnetic resonance imaging (4-chamber view) shows apical hypertrophy (red arrows), as well as an apical aneurysm (blue arrow). C, electrocardiography demonstrates giant T-wave inversions in the left precordial leads, characteristic of apical hypertrophic cardiomyopathy (arrows).

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    Figure 3

    Left ventricular outflow tract obstruction due to ventricular septal hypertrophy. The obstruction is dynamic, as the blood flow sweeps the mitral valve toward the septum.

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    Figure 4

    A, echocardiography, apical 4-chamber view, demonstrates a bifid papillary muscle resulting in left ventricular outflow tract obstruction (arrows). B, cardiac magnetic resonance imaging (left ventricular outflow tract view) demonstrates a bifid papillary muscle (arrows). C, an electrocardiogram of a patient with obstruction related to abnormal papillary muscle morphology demonstrates a lack of significant left ventricular hypertrophy. D, continuous-wave Doppler through the left ventricular outflow tract demonstrates a peak gradient of 99 mm Hg, consistent with obstruction, which increases with the Valsalva maneuver to 119 mm Hg (E).

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    Figure 5

    Left ventricular outflow tract (LVOT) obstruction without significant left ventricular hypertrophy. The prominent bifid papillary muscles lead to systolic anterior motion of the mitral valve, causing LVOT obstruction and simultaneous mitral regurgitation.

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    Figure 6

    Reorientation surgery reduces mobility of bifid hypermobile papillary muscles, reducing left ventricular outflow tract (LVOT) obstruction.

Tables

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    TABLE 1

    Differential diagnosis of hypertrophic cardiomyopathy

    Hypertensive cardiomyopathy
    Aortic valvulopathy
    Aortic stenosis
    Supra-aortic or subaortic membranes
    Infiltrative cardiomyopathy
    Amyloidosis
    Fabry disease
    Lysosomal diseases (eg, Danon disease)
    Glycogen storage disorders (eg, Pompe disease)
    Hemochromatosis
    ‘Athlete’s heart’
    Noncompaction cardiomyopathy
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    TABLE 2

    Main causative genes of hypertrophic cardiomyopathy (HCM)

    Sarcomeric proteinsGeneGene prevalence in HCM probands
    Myosin-binding protein CMYPBC315%
    Beta myosin heavy chainMYH715%
    Cardiac troponin TTNNT2  7%
    Alpha-tropomyosinTPM1  7%
    Regulatory myosin light chainMYL2< 5%
    Essential myosin light chainMYL3< 5%
    Cardiac troponin ITNNI3< 5%
    Nonsarcomeric proteinsGeneInheritanceAssociated phenotype
    TransthyretinTTRDominant; 1%–10%Amyloidosis
    Lysosome-associated membrane glycoprotein 2LAMP2X-linked; rareDanon disease
    Alpha-galactosidase AGLAX-linked; 1%–2% of malesFabry disease
    Lysosomal alpha-glucosidaseGAARecessive; rarePompe disease
    FrataxinFXNRecessive; rareFriedrich ataxia
    • Based on information in reference 3.

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    TABLE 3

    Risk-stratification models for primary prevention of sudden cardiac death in hypertrophic cardiomyopathy

    North American modelEuropean model
    An implantable cardioverter-defibrillator (ICD) is reasonable (class IIa recommendation, level of evidence C—limited evidence) if any of the following are present:
    • Family history of sudden death

    • Unexplained syncope

    • Maximum left ventricular wall thickness ≥ 30 mm


    Or if the patient has any other risk factor or modifier for sudden cardiac death and either of the following:
    • Nonsustained ventricular tachycardia

    • Abnormal blood pressure response during exercise (decrease or failure to increase systolic blood pressure ≥ 20 mm Hg during exercise stress test)

    The following factors are used to electronically calculate the 5-year risk of sudden cardiac death:
    • Family history of sudden death

    • Unexplained syncope

    • Maximum left ventricular wall thickness

    • Nonsustained ventricular tachycardia

    • Age

    • Left atrial diameter

    • Left ventricular outflow gradient


    5-year risk < 4%: an ICD is generally not indicated
    5-year risk ≥ 4% to < 6%: an ICD may be considered
    5-year risk ≥ 6%: an ICD should be considered
    • Based on information in references 9 and 30.

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Cleveland Clinic Journal of Medicine: 85 (5)
Cleveland Clinic Journal of Medicine
Vol. 85, Issue 5
1 May 2018
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Hypertrophic cardiomyopathy: A complex disease
Laura Young, Nicholas G. Smedira, Albree Tower-Rader, Harry Lever, Milind Y. Desai
Cleveland Clinic Journal of Medicine May 2018, 85 (5) 399-411; DOI: 10.3949/ccjm.85a.17076

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Hypertrophic cardiomyopathy: A complex disease
Laura Young, Nicholas G. Smedira, Albree Tower-Rader, Harry Lever, Milind Y. Desai
Cleveland Clinic Journal of Medicine May 2018, 85 (5) 399-411; DOI: 10.3949/ccjm.85a.17076
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  • Article
    • ABSTRACT
    • A PLETHORA OF MUTATIONS IN CARDIAC SARCOMERIC GENES
    • LEFT VENTRICULAR OUTFLOW TRACT OBSTRUCTION
    • DIAGNOSTIC EVALUATION
    • GENETIC TESTING, COUNSELING, AND FAMILY SCREENING
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