Category Archives: Cardiomyopathy

Broken Heart Syndrome (Stress Cardiomyopathy – takotsubo syndrome)

 

The above Video Explains this Rare But interesting Broken Heart Syndrome

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Restrictive Cardiomyopathy (Infiltrative Cardiomyopathy)

Restrictive (infiltrative) cardiomyopathy is a disorder of the heart muscle, in which the stiffened walls of the ventricles become resistant to adequate filling up with blood, when the heart relaxes (diastole) after every contraction, leading to heart failure.

  • Restrictive cardiomyopathy is the result of heart muscle being replaced by scar tissue, often due to the damage caused to it by the infiltration and accumulation of harmful substances.
  • A physical examination followed by echocardiography and cardiac catheterization help diagnose restrictive cardiomyopathy.
  • Treatment is focused mainly on treating the cause, as direct treatment of restrictive cardiomyopathy is not usually feasible.

Restrictive cardiomyopathy is the rarest form of cardiomyopathy and its cause is also rarely known. There are two types of restrictive cardiomyopathy.

In one type of restrictive cardiomyopathy, the heart muscle (myocardium) is gradually replaced by scar tissue possibly due to injury to the myocardium resulting from exposure to radiation during the treatment of cancer. But often, no such apparent cause can be attributed to this abnormal formation of scar tissue in the myocardium.

In the other type, certain substances infiltrate the heart muscle and accumulate there. When body has an excess of iron, as in the condition hemochromatosis, it may accumulate in the heart muscle too.

Amyloidosis, is a condition in which Amyloid protein accumulates in tissues, especially in older people. When amyloidiosis occur in the heart, restrictive cardiomyopathy may result.

Hypereosinophilic syndrome is a condition found in tropical countries, where a type of white blood cells called eosinophils , infiltrate the heart muscle.

Sarcoidosis is a disease where small granules of waste products are formed in various organs. This type of granuloma tissue can form in the heart muscle, causing restrictive cardiomyopathy.

Endocardial fibroelastosis, a congenital abnormality in which the left ventricle is lined by a thick layer of fibrous tissue, can usually be the cause of restrictive cardiomyopathy in children.

Symptoms

Restrictive cardiomyopathy exhibits the same symptoms of heart failure such as, shortness of breath and swelling due to fluid retention (edema). Chest pain may not be felt, but awareness of heartbeats (palpitations), due to abnormal heart rhythms are often felt on exertion. The heart can pump blood adequately during rest, because more than the pumping action, it is the heart’s capacity to fill up with blood, which is affected by the stiffening of the heart muscle. Hence, restrictive cardiomyopathy is usually asymptomatic at rest. However, symptoms appear during exercise, as the stiff heart is unable to pump efficiently to keep up with the body’s increased demand for blood and oxygen.

Diagnosis

Restrictive cardiomyopathy is diagnosed with the help of various tests.

Electrocardiography (ECG) may not help in proper diagnosis as it mainly shows the abnormalities in the electrical activity of the heart.

Echocardiography images of the structure and functioning of the heart can show whether the heart function is normal only during contraction (systole) and whether there is any enlargement of the atria.

Magnetic resonance imaging (MRI) is ideal as it can show changes in the texture of the heart muscle due to infiltration of substances such as iron and amyloid.

Cardiac catheterization helps remove a sample of the heart muscle for biopsy to detect the infiltrating substance.

Prognosis

Prognosis is bleak, as only 30 % people with restrictive cardiomyopathy survive beyond 5 years from the time symptoms appear.

The main reason for the poor prognosis is that many drugs used for treating heart failure are counteractive in the case of restrictive cardiomyopathy. Diuretics, which are the mainstay of the treatment of heart failure, help relieve leg swelling and reduce lung congestion. However, they worsen restrictive cardiomyopathy, since they decrease the volume of blood reaching the heart. Other main drugs used to treat heart failure such as angiotensin-converting enzyme (ACE) inhibitors, which help reduce the workload on the heart, also lowers blood pressure. It adversely affects those with restrictive cardiomyopathy, as it results in inadequate blood supply to the rest of the body. Often, Digoxin also doesn’t help.

Treatment

Treating the cause of the restrictive myopathy is the key to damage control. Even a partial reversal may be possible in certain cases.

  1. When infiltration of myocardium by iron is found to be the cause of restrictive cadiomyopathy, periodic blood-letting (phlebotomy) or removal of excess iron through chelation help reduce the amount of iron stored in the heart, thus reversing the condition.
  2. When sarcoidosis is the cause, corticosteroids may help clear up the granuloma tissue in the heart.

However, in many cases, the treatment of restrictive cadiomyopathy is difficult or even impossible, especially since the cause is often unknown.

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Hypertrophic Cardiomyopathy (Hypertrophic Obstructive Cardiomyopathy)

Hypertrophic cardiomyopathy is a disorder of the heart muscle, in which the walls of the ventricles thicken (hypertrophy) and become stiff, without any additional work load on the heart.

  • Hypertrophic cardiomyopathy usually results from an inherited genetic defect.
  • Chest pain (angina), shortness of breath, and palpitations and fainting bouts are the usual symptoms.
  • An echocardiography can confirm the initial diagnosis made through physical examination.
  • Drugs to reduce the force of the heart’s contractions help those with hypertrophic cardiomyopathy.

The incidence of hypertrophic cardiomyopathy is equal in men and women except among the older age group, where women outnumber men simply because the general life span of women is longer. In this age group, four people out of every hundred have this condition.

Causes

Congenital hypertrophic cardiomyopathy is almost always the result of a genetic defect that is inherited. Even when this heart condition develops later in life, the cause could be traced to this genetic factor.

Acquired hypertrophic cardiomyopathy may be the result of certain disorders due to over production of hormones. For example; acromegaly, resulting from an excess of pituitary growth hormone, and pheochromocytoma resulting from the excess of the hormone epinephrine, can often lead to hypertrophic cardiomyopathy. It may also be caused by another hereditary disorder called neurofibromatosis.

Symptoms

  • Fainting (syncope) during exertion is a common symptom.
  • Awareness of heartbeats (palpitations) may be present due to an abnormal heart rhythm (arrhythmia).
  • Chest pain (angina) is also very common symptom associated with this condition.
  • When the hypertrophied heart resists being refilled with blood from the lungs, it results in stagnation of blood in the lungs, leading to shortness of breath.

When the walls of the ventricle thicken, the mitral valve between the left atrium and the left ventricle may not close properly, due to which blood leaks back into the atrium.

In some cases, when the thickening of the heart muscle cause obstruction to the flow of blood out of the heart below the aortic valve, it results in a condition called hypertrophic obstructive cardiomyopathy.

Diagnosis

Hypertrophic cardiomyopathy is easy to diagnose from the sounds picked up by the stethoscope. Echocardiography is the ideal test to confirm the initial diagnosis. Chest x-ray and ECG are additional tests usually prescribed by doctors.

Prognosis

The fatality rate of hypertrophic cardiomyopathy is 4% among those who suffer from it. Most deaths tend to be sudden; an abnormal heart rhythm (arrhythmia) being the usual cause. Death due to chronic heart failure is a comparatively rare occurrence in those with this condition. Hypertrophic cardiomyopathy is the most usual cause of sudden death in young athletes.

Genetic Counseling

Since the usual cause of hypertrophic cardiomyopathy is an inherited genetic defect, family members of those who are known to have this disorder can opt for genetic testing to confirm their status. Those who are likely to inherit this disorder are advised to go for genetic counseling when they plan a family.

Treatment

Drug therapy for hypertrophic cardiomyopathy focuses on reducing the resistance of the heart to refilling with blood after every contraction.

Beta-blockers reduce the extent to which the heart muscle contracts, so that it can return to relaxed position more easily allowing the heart to fill better. When the contraction is limited, it can also improve the blood flow out of the heart, in case a thickened muscle is blocking the blood flow.

The calcium channel blocker verapamil also works along similar lines and it is often prescribed along with beta blockers. They also help slow down the heart, giving it extra time to fill. Disopyramide is another useful drug which help reduce the strength of heart contractions.

When treatment with drugs does not improve the condition, the thickened heart muscle can be surgically removed (myectomy) to improve the blood flow from the heart. Surgery may reduce the symptoms, but not the risk of death.

Cardiac catheterization is a minimally invasive procedure by which selective destruction of thickened tissue can be achieved, by injecting a small amount of absolute alcohol (alcohol ablation) into a small, carefully ear marked area. Killing the thickened tissue causing obstruction improves the blood flow. This method is preferred over open heart surgery due to the reduced risk associated.

A cardioverter-defibrillator for better control of heart beat can be implanted in people with an increased risk of sudden death due to arrhythmia.

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Dilated Cardiomyopathy

What is Dilated Cardiomyopathy ? Dilated cardiomyopathy is a disorder of the heart muscle, in which the abnormally enlarged lower chambers (ventricles) of the heart fail to pump adequate amount of blood, leading to heart failure.

  • Dilated cardiomyopathy is often caused by coronary artery disease and myocarditis.
  • The earliest symptoms are shortness of breath and fatigue on exertion.
  • Electrocardiography and echocardiography help diagnose dilated cardiomyopathy.
  • Dilated cardiomyopathy is generally treated with drugs that treat its cause.

Dilated cardiomyopathy is prevalent in the age group 20 – 60 years, but not strictly restricted to it. Men are more prone to this condition than women; and blacks, more than whites; the ratio of incidence being 3 : 1 in both cases. Every year, approximately 50 – 80 people out of every million develop dilated cardiomyopathy.

Causes of Dilated Cardiomyopathy

Coronary artery disease is the most common cause of dilated cardiomyopathy. Coronary artery disease impairs blood supply to the heart muscle, causing irreversible damage and death of the muscle, consequent to which, the blood pumping capacity of the heart becomes severely diminished. The remaining healthy heart muscle stretches (hypertrophies) to make up for the reduced capacity since stretched and taut heart muscle can pump with more force (much like a stretched rubber band). However, after a while, the hypertrophied heart muscle can no longer keep up this compensatory action, resulting in dilated cardiomyopathy leading to heart failure.

Myocarditis or inflammation of the heart muscle due to an infection can also result in dilated cardiomyopathy. When a viral infection is the cause, it is called viral cardiomyopathy. Coxsackie B virus infection is a common cause of viral cardiomyopathy in the U.S. Dilated cardiomyopathy can also result from a bacterial infection. The weakening of the heart muscle by the infection causes the muscles to stretch and thicken in a compensatory attempt, resulting in dilated cardiomyopathy, and often heart failure.

Obesity, diabetes, thyroid disease and persistent tachycardia (rapid heart rate) are also known to result in dilated cardiomyopathy.

Some substances such as, cocaine, and alcohol (especially when combined with poor nutrition), antidepressants, and a few chemotherapy drugs too cause this condition.

Extra stress to the heart during pregnancy and auto immune diseases like rheumatoid arthritis also may lead to dilated cardiomyopathy.

When dilated cardiomyopathy cannot be attributed to any identifiable cause, it is termed idiopathic dilated cardiomyopathy.

Symptoms

Shortness of breath and fatigue on exertion are the earliest symptoms of dilated cardiomyopathy. Heart failure due to the weak pumping action of the heart causes these symptoms.

A sudden fever may be the first symptom of the development of cardiomyopathy due to viral or bacterial myocarditis.

Palpitations or pronounced beating of the heart felt by the patient, is a sign of a dangerous (often fatal) abnormal heart rhythm (arrhythmias), caused by the damaged heart.

Due to the enlargement of the heart, there may be incomplete closing of the heart valves, leading to leakage and regurgitation of blood, further impairing the pumping action of the heart. Heart murmurs that can be picked up by through a stethoscope can identify this condition. The two atrioventrcular valves connecting the ventricles to atrium, namely, mitral valve and tricuspid valve are the most affected, because, it is often the ventricles of the heart that are abnormally enlarged.

The enlarged walls and the reduced pumping action of the heart, together precipitates the ideal condition for the formation of clots on the heart walls. When these clots become detached and travel through the blood vessels (emboli), they can cause partial or complete blockage of those vessels, resulting in damage to various organs. A block in the arteries supplying the brain can lead to stroke.

Irrespective of the cause of dilated cardiomyopathy, a severely damaged heart eventually precipitates symptoms of heart failure such as rapid heart rate and fluid retention in the limbs and lungs, finally culminating in death.

Diagnosis

The symptoms exhibited by the patient and physical examination can help diagnose cardiomyopathy. It is always followed by more accurate diagnostic tests to determine the cause as well as the extent of the disease.

  • Electrocardiography (ECG) measures the electrical activity of the heart and can point out any abnormality but it is inconclusive at best.
  • Echocardiography projects a real time image of the heart, giving a clear picture of its size and pumping action, making it the ideal diagnostic test for dilated cardiomyopathy,
  • Magnetic resonance imaging (MRI), may help to identify the cause of dilated cardimyopathy as it gives very accurate and detailed pictures of the heart.
  • Cardiac catheterization is a procedure in which a thin catheter is inserted into a major vein and guided into the heart. It can help assess more accurately, the pumping action of the heart as well as the pressure existing in the chambers of the heart. It can also collect a sample of the heart muscle for biopsy to determine whether the cardiomyopathy is due to any viral or bacterial infection. The extent of damage caused to the heart by coronary heart disease also can be correctly determined by this procedure.

Prognosis

Prognosis is very bleak, with less than 30% of those who suffer from dilated cardiomyoparthy surviving beyond five years from the onset of the disease. Also, half of the fatalities are sudden deaths, caused due to an abnormal heart rhythm which suddenly worsens. Statistics indicate that women survive for twice as long as men and whites twice as long as blacks.

Treatment Of Dilated Cardiomyopathy

    1. The main focus of the medical treatment is on treating the underlying cause.
    2. Drugs usually prescribed include ACE inhibitors, angiotensin II receptor blockers, beta-blockers, spironolactone or eplerenone, and low-dose digoxin. They help reduce symptoms and improve heart function thus prolonging life.
    3. Antiarrhythmic drugs such as beta blockers and Amiodarone are used to prevent abnormal heart rhythm. They are given in small doses, gradually increasing the dosage by small amounts, as an overdose could have an adverse effect on the pumping action of the heart, further affecting the rhythm.
    4. Pace makers may help correct abnormal electrical circuitry in the heart. Patients at greater risk of sudden death can get a cardioverter-defibrillator pacemaker implanted to regulate their heart function.
    5. Warfarin, an anti coagulant, always forms an integral part of the treatment since the overly stretched walls of a sluggish heart is an ideal site for clot formation.
    6. Avoiding physical strain and mental stress which may worsen heart function is a part of general treatment. Cutting sodium intake and use of diuretics may not prolong life but may improve the quality of life by reducing swelling and discomfort due to excess fluid collection in the lungs.
    7. In spite of the risks involved in heart transplantation, it is often considered a viable option for persons with dilated cardiomyopathy, as this condition is almost always fatal, unless its cause can be correctly identified and successfully treated before severe heart failure occurs.


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What Is Cardiomyopathy

What Is Cardiomyopathy ? Cardiomyoparthy is a degenerative disease of the muscular walls of the heart or myocardium, impairing the heart’s ability to pump blood.

Cardiomyopathy can develop due to several disorders of the heart, but its cause is not always easy to identify, especially in children.

The main types of cardiomyopathy are:

Cardiomyopathies often exhibit symptoms similar to heart failure. Chest pain (angina) and fainting may also be an indication. Sudden death due to cardiomyopathy is not rare.

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