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Hypertrophic Cardiomyopathy

  • Anita Sadeghpour
  • Azin Alizadehasl
Chapter

Abstract

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disorder (autosomal dominant transmission), with primary involvement of the cardiac muscle. If associated with left ventricular outflow tract (LVOT) obstruction, it is called hypertrophic obstructive cardiomyopathy (HOCM).

HCM is characterized by localized or generalized unexplained left ventricular hypertrophy (LVH) in the non-dilated ventricle in the absence of any cardiac or systemic disease that can result in this magnitude of hypertrophy.

Usually, LV wall thickness is ≥15 mm in adults and ratio of IVS thickness to posterior wall thickness is ≥1.3/1. Systolic anterior motion (SAM) is the abnormal movement (sharp bending) of the anterior mitral valve (MV) leaflet toward the interventricular septum during systole.

LVOT obstruction happens as a result of both significant septal thickening and concomitant SAM of the mitral leaflets into the LVOT. Continuous-wave Doppler flow of the LVOT is characteristically dagger shaped and has a late-peaking velocity due to an increased obstruction as systole progresses. MR due to SAM is a mid-to-late systolic, eccentric, and posteriorly directed jet.

Several studies in patients with HCM have demonstrated a significant reduction in longitudinal strain (particularly in the mid-septal segment) with normal systolic twist or torsion.

Keywords

Hypertrophic cardiomyopathy Hypertrophic obstructive cardiomyopathy Left ventricular outflow tract obstruction Asymmetric septal hypertrophy Systolic anterior motion 

Abbreviations

2D

Two dimensional

3D

Three dimensional

AF

Atrial fibrillation

ASH

Asymmetric septal hypertrophy

CAD

Coronary artery disease

CMR

Cardiac magnetic resonance

CT

Computed tomography

E

Peak E-wave velocity of the mitral valve (cm/s)

E′

TDI of the mitral annulus

EF

Ejection fraction

FC

Functional class

HCM

Hypertrophic cardiomyopathy

HHD

Hypertensive heart disease

HOCM

Hypertrophic obstructive cardiomyopathy

ICD

Implantable cardioverter defivrillators

LA

Left atrium

LGE

Late gadolinium enhancement

LV

Left ventricular

LVEF

Left ventricular ejection fraction

LVH

Left ventricular hypertrophy

LVOT

Left ventricular outflow tract

NSVT

Nonsustained ventricular tachycardia

NYHA

New York Heart Association

RV

Right ventricle

SAM

Systolic anterior motion

SCD

Sudden cardiac death

STE

Speckle-tracking echocardiography

TDI

Tissue Doppler imaging

TEE

Transesophageal echocardiography

TTE

Transthoracic echocardiography

Supplementary material

Movie 22.1

(related to Fig. 22.2): ASH and SAM in para-sternal long axis view; SAM is best appreciated in the MV M-mode recordings of parasternal long-axis views (AVI 7146 kb)

Movie 22.2

MR due to SAM is a mid-to-late systolic, eccentric, and posteriorly directed jet (which is a common finding in HOCM) (AVI 808 kb)

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Anita Sadeghpour
    • 1
  • Azin Alizadehasl
    • 2
  1. 1.Professor of CardiologyEchocardiography Research Center, Rajaie Cardiovascular Medical and Research Center, Iran University of Medical ScienceTehranIran
  2. 2.Associate Professor of Cardiology, Echocardiologist, Echocardiography and Cardiogenetic Research CentersCardio-Oncology Department, Rajaie Cardiovascular Medical and Research CenterTehranIran

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