cardiomyopathy

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cardiomyopathy

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Presentation Transcript

Cardiomyopathy: 

Cardiomyopathy

Definition: 

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)

Metabolicdiseases: 

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

Classification: 

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: 

HCM Mitral Valve in normal position

HOCM: 

HOCM Mitral valve presses against septum MR

SYMMETRIC: 

SYMMETRIC symmetric or concentric

APICAL: 

APICAL Small cavity remains Apical Hypertrophy

Symptoms: 

Symptoms Dyspnoea angina palpitation dizzy spells syncope

Slide16: 

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: 

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

Slide22: 

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: 

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)