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Definition Disorders of the cardiac muscle of unknown aetiology

Specific types: 

Specific types Infective/viral Metabolic/infiltrative (cardiac amyloidosis) Systemic/multistystem disease Familial neuro-muscular disorders eg muscular dystrophy Sensitivity or toxic reactions eg. Alcohol (dilated) and chemotherapy

Infective : 

Infective Viral eg. Coxackie virus (dilated) Tuberculosis (restrictive) HIV/AIDS ( dilated) Chagas disease (restrictive) - South America hypereosinophilic syndrome - could be parasitic, mostly unknown (restrictive - iron deposits)


Metabolic diseases Primary cardiac amyloidosis - restrictive (protein deposits) cardiac sarcoidosis - cardiac granulomas hemochromatosis


Classification Hypertrophic HCM HOCM Obliterative Dilated (Congestive) Restrictive

Hypertrophic Cardiomyopathy: 

Hypertrophic Cardiomyopathy

Hypertrophic Cardiomyopathy: 

Hypertrophic Cardiomyopathy Pathological changes are of muscular hypertrophy associated with fibrosis Muscle fibres hypertrophy Myocardial disarray - some parts of the heart coronary arteries tendancy - large calibre

Myocardial Disarray: 

Myocardial Disarray Normal Muscle Structure Myocardial Disarray (Parallel alignment) (Disorganised alignment)

Types Hypertrophic Cardiomyopathy: 

Types Hypertrophic Cardiomyopathy HCM or HOCM Asymmetric septal (ASH) - without obstruction Asymmetric septal (ASH) - with obstruction Symmetric hypertrophy - concentric Apical hypertrophy


HCM Mitral Valve in normal position


HOCM Mitral valve presses against septum MR


SYMMETRIC symmetric or concentric


APICAL Small cavity remains Apical Hypertrophy


Symptoms Dyspnoea angina palpitation dizzy spells syncope


Gene is present from birth linked to growth hormone Myopathy can develop at any age - mostly under age 25, but not always diagnosis at early age increases risk of poor prognosis


Prognosis Adults - 2-3% SCD per year Adolescents - 4-6% SCD per year Infants (less than 1 yr old), mortality = 50%

Identification of patients at risk: 

Identification of patients at risk Diagnosis in childhood/adolescence Family history of HCM or SCD Syncope

Mechanism of death: 

Mechanism of death Mostly unknown ?VT or VF AF with fast ventricular response Any arrhythmia or increased obstruction causing Haemodynamic compromise

Dilated Cardiomyopathy: 

Dilated Cardiomyopathy

Dilated Cardiomyopathy: 

Dilated Cardiomyopathy Dilated left ventricle poorly contracting little difference between systole and diastole low cardiac output


Difficult to distinguish between dilated cardiomyopathy & dilated left ventricle due to severe CAD Usually post MI/CAD - local dysfunction Dilated myopathy - global dysfunction

Right ventricular dilatation: 

Right ventricular dilatation Uhl’s disease RV dysplasia or myopathy congenital associated ventricular arrhythmias

Restrictive Cardiomyopathy: 

Restrictive Cardiomyopathy

Restrictive Cardiomyopathy: 

Restrictive Cardiomyopathy Idiopathic Infiltrative eg – amyloidosis,sarcoidosis, mucopolysacharidosis, malignancies or Radiation Glycogen storage disease Hemochromatosis Fabrys disease Cardiac transplant rejection


Characteristics Abnormal compliance of the left ventricle short relaxation time Dilated atria with normal ventricular dimensions.

Summary : 

Summary various types diagnosis ECHO BLOOD TESTS LINK TO MEDICAL CONDITIONS Classification difficult discrepancies between countries (terminology & nomenclature)

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