Cardiomyopathy ( Braoken Heart Syndrome )


Cardiomyopathy ( Braoken Heart Syndrome ) refers to diseases of the heart muscle .In cardiomyopathy, the heart muscle becomes enlarged, thick or rigid.

As cardiomyopathy worsens, the heart becomes weaker.Its less able to pump blood through the body and maintain normal electrical rhythm. This can lead to heart failure or irregular heartbeats called arrhythmias. In turn heart failure can cause fluid build up in the lungs, ankles, feet, legs or abdomen. It could be acquired or inherited. Many times the cause of cardiomyopathy is not known.

Braoken Heart Syndrome

Types

  • Hypertrophic
  • Dilated
  • Restricted
  • Arrythmogenic right ventricular dysplasia
  • Others (Takotsubo cardiomyopathy)
  • Ventricular non-compaction
Cardiomyopathy

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.

Causes

  • Inherited
    Mutation or change in some of the genes in heart muscle proteins
  • Non-inherited
    -Aging
    -High blood pressure
    -Diabetes
    -Thyroid disease

Symptoms

  • Chest pain or pressure in the chest after exercise or physical activity.
  • Shortness of breath( dyspnoea) especially after exertion
  • Fatigue
  • 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

Diagnostic tests

Diagnosis is typically done by echocardiogram .In some cases TEE can be done.

Other tests

  • ECG
  • Cardiac MRI
  • Stress tests
  • Holtyerband event monitor
  • Genetic testing

In some cases cardiac catheterisation and coronary angiography can be done

Dilated cardiomyopathy(DCM)

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.

Causes

  • Idiopathic (50%)
  • Myocarditis(9%)
  • 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%)

Symptoms

  • Fatigue
  • 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

Risk Factors

  • Men (20-50yrs)> Women
  • Heart attack
  • Family history
  • Immune system disorders like lupus
  • Muscular dystrophy

Complications

  • Heart failure
  • Heart valve regurgitation
  • Fluid build up(edema)
  • Arrhythmia
  • Sudden cardiac death
  • Emboli

Prevention And Treatment

  • Healthy lifestyle habits can help you prevent or minimise effects of DCM
  • Medical management

Intervention

  • 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

Other Cardiomyopathies

  • 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.

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