2. Introduction
⚫ 2006 AHA defined cardiomyopathies as “a heterogeneous group of
diseasesof the myocardiumassociated with mechanical &/orelectrical
dysfunction that usually (but not invariably) exhibit inappropriate
ventricularhypertrophyordilatationand aredue toavarietyof causes
that frequentlyaregenetic.”
⚫ Cardiomyopathieseitherareconfined to the heartorarea partof
generalized systemicdisorders
5. Definition
⚫ Primary (idiopathic) is a disease of unknown etiology that principally
affects the myocardium leading to LVdilationand systolicdysfunction
⚫ Mostcommonof thecardiomyopathies
12. DCM - Incidence and Prognosis
⚫ Prevalence is 36 per 100,000 population
⚫ Third mostcommoncauseof heart failure
⚫ Most frequentcauseof heart transplantation
⚫ Completerecovery is rare
⚫ 50% diewithin 2yrsand 25% survive longerthan 5yrs
14. Hypertrophic cardiomyopathy
⚫ Characterised by myocardial hypertrophy,abnormal diastolic
filling,intermittentventricularoutflow obstruction
⚫ Related todefects in forcegeneration owing toaltered sarcomeric
function
⚫ Leading causeof LVH,unexplained byotherclinical/pathologiccause
⚫ Caused by mutationof genes encoding sarcomericproteins
15. Pathogenesis
⚫ Autosomal dominantwith variable penetrance
⚫ Remaining are sporadic
⚫ Mutationsare mostly missense
⚫ Mutationscausing HCM found in genes encoding β MHC,cardiac
TnT,α tropomyosin,myosin binding protein C
16. ⚫ The major abnormality of the heart
in HCM -- excessive thickening of
the muscle. Thickening usually
begins during earlyadolescence and
stops when growth has finished.
uncommon for thickening to
progress after this age
⚫ leftventricle almost always affected
⚫ Hypertrophy is usually greatest in
the septum, associated with
obstruction to the flow of blood into
theaorta
17. ⚫ Asymmetricseptal
hypertrophywith obstruction
totheoutf lowof blood from
the heart mayoccur. The mitral
valve touches the septum,
blocking the outflow tract.
Some blood is leaking back
through the mitral valve
causing mitral regurgitation
22. Restrictive cardiomyopathy
⚫Hallmark: abnormal diastolic function
⚫Rigid ventricularwall with impaired ventricularfilling ,contractile
functionsare normal
⚫ Much less common then DCM or HCM
⚫ Characterised by primary disease in ventricularcompliance resulting in
impaired ventricularfiling during diastole
