Hypertrophic cardiomyopathy (HCM)
HCM is characterised by thickened but nondialated left ventricle in the absence of another cardiac or systemic condition.HCM is a genetic disorder inherited in an autosomal dominant way.
HCM is the most common heritable cardiomyopathy manifesting in 1 in every 500 adults.HCM is diagnosed based on medical history (symptoms and family history),physical examination and echocardiography results.
Mutation or change in some of the genes in heart muscle proteins
-High blood pressure
- Chest pain or pressure in the chest after exercise or physical activity.
- Shortness of breath( dyspnoea) especially after exertion
- Fainting (irregular heart rhythms or abnormal response of blood vessels during exercise.
- Palpitations (fluttering in the chart)due to arrhythmias such as atrial fibrillation or ventricular tachycardia.
- Sudden death
Diagnosis is typically done by echocardiogram .In some cases TEE can be done.
- Cardiac MRI
- Stress tests
- Holtyerband event monitor
- Genetic testing
In some cases cardiac catheterisation and coronary angiography can be done
It is a primary myocardial disease.The cause of dilated cardiomyopathy is still unknown.About one third of the people who have dilated cardiomyopathy inherit it from their parents. DCM is characterised by dilated left ventricle with systolic dysfunction that is not caused by ischemic or valvular heart disease.Investigating a patient with DCM ,a full history,including risk factors for coronary artery disease should be acquired.Personal history is very important therefore if possible spouse’s input is very valuable.
- Idiopathic (50%)
- Ischemic heart disease(9%)
- Infiltrative disease(5%)
- Peripartum (7%)
- Hypertension (4%)
- HIV infection(4%)
- Connective tissue disease(3%)
- Substance abuse (3%)
- Doxorubicin (1%)
- Others (10%)
- Heart failure like symptoms like shortness of breath (dyspnea).
- Reduced ability to exercise because of swelling in the ankles and legs.
- Chest pain
- Heart murmurs
- Men (20-50yrs)> Women
- Heart attack
- Family history
- Immune system disorders like lupus
- Muscular dystrophy
- Heart failure
- Heart valve regurgitation
- Fluid build up(edema)
- Sudden cardiac death
Prevention And Treatment
- Healthy lifestyle habits can help you prevent or minimise effects of DCM
- Medical management
- Cardiac resynchronisation by biventricular pacemaker.
- ICD (implantable cardioverter defibrillator.These are suggested for people at risk for life threatening arrhythmias.
- Surgery -in case of coronary artery disease or valve disease.
- Heart transplant -only for end stage heart failure
- Restrictive cardiomyopathy (RCM) :
RCM’S is a heterogenous group of diseases characterised by non dilated left ventricle often with well preserved ejection fraction .The predominant manifestation is a diastolic dysfunction as a result of myocardial disease.
- Hemochromatosis :
Too much iron build up and deposition of extra iron can damage the organs.
- Sarcoidosis :
A disease that causes inflammation and can affect various organs in the body .Abnormal immune response can cause sarcoidosis.
Some cancer treatments such as radiation and chemotherapy
Arrythmogenic right ventricular dysplasia.