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CARDIOMYOPATHIES DR MWIINGA. Summerised Nnts

Cardiomyopathies are heart muscle diseases classified by the WHO into specific and primary categories, with specific types linked to conditions like RHD and primary types including hypertrophic, dilated, and restrictive cardiomyopathies. Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disorder often leading to sudden death, while dilated cardiomyopathy is commonly idiopathic and presents with heart failure. Restrictive cardiomyopathy is the least common type, characterized by impaired diastolic function and often associated with infiltration or fibrosis.

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20 views12 pages

CARDIOMYOPATHIES DR MWIINGA. Summerised Nnts

Cardiomyopathies are heart muscle diseases classified by the WHO into specific and primary categories, with specific types linked to conditions like RHD and primary types including hypertrophic, dilated, and restrictive cardiomyopathies. Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disorder often leading to sudden death, while dilated cardiomyopathy is commonly idiopathic and presents with heart failure. Restrictive cardiomyopathy is the least common type, characterized by impaired diastolic function and often associated with infiltration or fibrosis.

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mordecaisibulowa305
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© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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CARDIOMYOPATHIES

DR Mwiinga G N
 Cardiomyopathies ; heart muscle disease –
diseases of the myocardium associated with
cardiac dysfunction.
 WHO has classified cardiomyopathies into two
categories ( 1995 ) :
1- Specific ; secondary to RHD, ischaemic
heart disease, HHD, inflammations, general
systemic disease ( RF, Sarcoidosis ) .
2- Primary ; intrinsic to the myocardium –
hypertrophic, dilated, restrictive and
arrhythmogenic RVC and unclassified.
HCM
 Autosomal dominant disorder which most commonly
present with sudden death due to arrhythmia.
 It is caused by mutations in the genes encoding the
contractile proteins of the cardiac sarcomere.
 Characterized by heterogenous features ranging
from palpitations – syncope – exertional dyspnea to
congestive heart failure .
 Pathologically it maybe symmetric ( 1/3 of cases ) or
asymmetric ( IVS hypertrophy with sub-aortic
stenosis )
 Histologically, its hallmark is a triad of myocyte
hypertrophy, disarray and interstitial fibrosis.
Dilated cardiomyopathies
 Most commonly idiopathic presenting with progressive heart
failure.
 Characterized by globular heart shape, marked ventricular
cavity dilation, diffuse endocardial thickening, reduction in
ventricular wall thickness and atrial enlargement + thrombi
oftenly in either appendage.
 Histologically, increased myocyte length, interstitial fibrosis,
nuclear hypertophy and pleomorphism + increased
interstitial T- lymphocytes and macrophages + extensive
myofibrillary loss.
 1/3 of cases are inherited, caused by gene mutations in
structural proteins of the myocytes.
 Other causes are ; several genetic diseases ( e.g myotonic
dystrophy ) – cardiotoxins ( doxorubicin ) – alcohol –
puerperal state .
 Some cases were found to be associated with auto immune
etiology ( auto antibodies against cardiac specific antigens )
and others secondary to viral myocarditis ( +ve serology,
viral nucleic acid evidenced by PCR analysis ).
Restrictive cardiomyopathies
 The least common type.
 Characterized by impaired diastolic function or
relaxation.
 Classified into primary – endomyocardial fibrosis
( EMF ) , idiopathic. And secondary ( more common )
due to myocardial disease stiffening the heart by
infiltration ( e.g amylodosis )or fibrosis.
 Inflammatory CM ; acute myocarditis with acute onset
of progressive heart failure caused by viruses or
protozoa ( arrhythmia with concomitant febrile
illness ).
 Histologicaly, diffuse interstitial inflammatory cell
infiltrate ( T- lymphocytes + myocyte loss & damage ).
Overview
 In HCM, specific gene mutations are
associated with different disease
severity and prognosis.
 The management of
cardiomyopathies is that of the
presenting features e.g arrhythmia,
palpitations, dyspnea, failure and
management of underlying cause
when appropriate .
Thank you

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