Hypertrophic Cardiomyopathy

From Ask Dr Wiki

Contents 1 Introduction 2 Types 3 Echocardiographic Assessment 4 Risk Factors for Sudden Cardiac Death

Introduction

• Most common genetic cardiovascular disease- prevalence about 0.2% ( 1 in 500) no more than 1% of OPD in routine cardiology practice
• Hypertrophic cardiomyopathy is an autosomal dominant, incompletely penetrant disease.
• The overall incidence of SCD is 2-4% in adults and up to 6% in children. It is the most common cause of SCD in young and athletes.
• First description in 1958

• Predominantly non obstructive disease-75%

• Primary sarcomere disorder

β- myosin heavy chain

cardiac troponin T

myosin- binding protein C

• Other genes

cardiac troponin I

regulatory and essential myosin light chains

titin

alpha- tropomysin

Alpha-actin

alpha myosin heavy chain

Types

• Concentric
• Free Wall
• Apical Hypertrophic Cardiomyopathy

Echocardiographic Assessment

• Check degree of septal hypertrophy
• Check for SAM and degree of Mitral Regurgitation
• Check for premature closure of the Aortic valve on M mode
• Check LVOT gradients with C_w (dagger shaped)
  • >30mm Hg resting
  • >50mm Hg with provocation

Risk Factors for Sudden Cardiac Death

• Family History of Hypertrophic Cardiomyopathy and/or Sudden Cardiac Death
• Syncope
• Severe Left Ventricular Hypertrophy (> 3.0 cm)
• Abnormal blood pressure drop with exercise
• Non Sustained Ventricular Tachycardia