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Hypertrophic Cardiomyopathy and Mitral Valve Disease

  • Milind DesaiEmail author

Abstract

A 38-year-old man presented with progressively worse exertional dyspnea, effort-related chest tightness, and one episode of exertional syncope. He had a strong history of multiple family members dying suddenly. He also reported intermittent palpitations over the years. On examination, he was found to have a harsh systolic ejection murmur at left upper sternal border that increased with Valsalva and reduced with squatting. Patient also had a moderate intensity systolic precordial murmur. Electrocardiogram revealed normal sinus rhythm with severe left ventricular hypertrophy (LVH) and strain pattern. He was found to have hypertrophic obstructive cardiomyopathy (HOCM) with severe left ventricular outflow tract (LVOT) obstruction. He underwent surgical myectomy with complete relief of symptoms.

Keywords

Left ventricular hypertrophy LVOT obstruction Systolic anterior motion Papillary muscles 

Supplementary material

Video 8.1

Two-dimensional transthoracic echo in the parasternal view demonstrating severely hypertrophied basal interventricular septum with systolic anterior motion (SAM) of the mitral valve. The patient has a characteristic “reverse septal curvature” pattern, which is frequently seen in patients with a genetically transmitted mode of disease (AVI 5952 kb)

Video 8.2

Two-dimensional transthoracic echo with color Doppler in the parasternal view demonstrating severe turbulence of blood flow across the LVOT, due to a combination of SAM of the anterior mitral leaflet and severely hypertrophied basal interventricular septum. Notice the characteristic SAM-related posteriorly directed jet of mitral regurgitation (MR) in the left atrium (AVI 3537 kb)

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Video 8.3 Four-chamber balanced steady-state free precession (b-SSFP) cine cardiac magnetic resonance (CMR) image of the same patient confirming severe basal septal hypertrophy, LVOT obstruction, and posteriorly directed jet of MR. Again, notice the characteristic reverse septal curvature pattern (AVI 1425 kb)
Video 8.4

Two-dimensional transthoracic echocardiogram in the parasternal view demonstrating a severely hypertrophied focal segment of the basal interventricular septum with systolic anterior motion (SAM) of the mitral valve. The patient has a characteristic “sigmoid” pattern, which is frequently seen in elderly patients with long-standing history of uncontrolled hypertension (AVI 6144 kb)

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Video 8.5 Two-dimensional transthoracic echocardiogram in the apical 5-chamber view again demonstrating the characteristic sigmoid-shaped interventricular septum with systolic anterior motion (SAM) of the mitral valve (AVI 2502 kb)
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Video 8.6 Two-dimensional transthoracic echocardiogram in the apical 3-chamber view demonstrating a long anterior mitral leaflet with obstructive SAM. Notice that the basal interventricular septum is normal in thickness (AVI 1709 kb)
Video 8.7

Two-dimensional transthoracic echocardiogram with color Doppler in the same apical 3-chamber view demonstrating severe turbulence of blood flow across the LVOT and characteristic severe posteriorly-directed jet of MR. However, there is no evidence of basal septal hypertrophy (AVI 0 bytes)

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Video 8.8 Two-dimensional transthoracic echocardiogram in the apical 2-chamber view demonstrating a long posterior mitral leaflet with SAM. Notice that the basal interventricular septum is normal in thickness (AVI 2035 kb)
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Video 8.9 Two-dimensional transthoracic echocardiogram in the same apical 2-chamber view demonstrating severe SAM with septal contact, following inhalation of amyl nitrite (AVI 128 kb)
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Video 8.10 Two-dimensional transthoracic echo with color Doppler in the parasternal long-axis view, demonstrating flow turbulence across LVOT suggestive of significant LVOT obstruction. Additionally, there is an anteriorly directed jet of MR, which is atypical for being SAM-related. In HOCM patients, presence of an anteriorly directed jet of MR should always raise a suspicion of intrinsic mitral valve (especially posterior leaflet) pathology (AVI 1459 kb)
Video 8.11

Two-dimensional transthoracic echocardiogram in the apical 3-chamber view demonstrating a very hypertrophied anterolateral papillary muscle. Notice that the mitral leaflet lengths are normal and the basal interventricular septum is normal in thickness (AVI 5927 kb)

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Video 8.12 Two-dimensional transthoracic echocardiogram in the apical 3-chamber view in the same patient following inhalation of amyl nitrite. Notice severe SAM with septal contact (AVI 0 bytes)
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Video 8.13 Two-dimensional transthoracic echocardiogram in the apical 3-chamber view at peak upright bicycle exercise in the same patient, demonstrating severe SAM with septal contact (AVI 1847 kb)
Video 8.14

Two-dimensional transesophageal images (mid-esophageal view) in the same patient after administration of intravenous Isoprenaline, demonstrating severe SAM with septal contact (AVI 7703 kb)

Video 8.15

Two-dimensional Color Doppler transesophageal images (mid-esophageal view) in the same patient after administration of intravenous Isoprenaline demonstrating severe posteriorly directed SAM-related MR and turbulence across the LVOT suggestive of LVOT obstruction (AVI 3759 kb)

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Video 8.16 Four-chamber b-SSFP cine CMR image of the same patient confirming a very hypertrophied anterolateral papillary muscle and absence of basal septal hypertrophy (AVI 1290 kb)
Video 8.17

Two-dimensional transthoracic echocardiogram in the apical 3-chamber view obtained following surgical correction of LVOT obstruction (papillary muscle reorientation). Notice the bright suture material in the mid portion of the LV cavity. Also there is no LVOT obstruction (AVI 10920 kb)

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Video 8.18 Two-dimensional transthoracic echocardiogram in the apical 3-chamber view demonstrating a bifid and hypermobile anterolateral papillary muscle. Notice that the mitral leaflet lengths are normal and the basal interventricular septum is normal in thickness (AVI 2290 kb)
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Video 8.19 Two-dimensional transthoracic echocardiogram in the apical 3-chamber view at peak treadmill exercise, demonstrating severe SAM (AVI 0 bytes)
Video 8.20

Three-chamber b-SSFP cine CMR image of the same patient confirming a bifid and hypermobile anterolateral papillary muscle and absence of basal septal hypertrophy (AVI 0 bytes)

Suggested Reading

  1. Desai MY, Ommen SR, McKenna WJ, Lever HM, Elliott PM. Imaging phenotype versus genotype in hypertrophic cardiomyopathy. Circ Cardiovasc Imaging. 2011;4(2):156–68.PubMedCrossRefGoogle Scholar
  2. Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, et al. 2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Developed in collaboration with the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol. 2011;58(25):e212–60.PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag London 2015

Authors and Affiliations

  1. 1.Department of Cardiovascular MedicineCleveland ClinicClevelandUSA

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