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)