PCCN Restrictive Cardiomyopathy

PCCN Restrictive Cardiomyopathy

Restrictive cardiomyopathy, the rarest form of cardiomyopathy, is a condition in which the walls of the lower chambers of the heart (the ventricles) are abnormally rigid and lack the flexibility to expand as the ventricles fill with blood.

The pumping or systolic function of the ventricle may be normal but the diastolic function (the ability of the heart to fill with blood) is abnormal. Therefore, it is harder for the ventricles to fill with blood, and with time, the heart loses the ability to pump blood properly leading to heart failure.

Signs and Symptoms

Many people with restrictive cardiomyopathy have no symptoms or only minor symptoms, and live a normal life. Other people develop symptoms, which progress as heart function worsens.

Symptoms occur at any age and may include:

• Shortness of breath (at first with exercise; but over time it occurs at rest)
• Fatigue (feeling overly tired)
• Inability to exercise
• Swelling of the legs and feet
• Weight gain
• Nausea, bloating, and poor appetite (related to fluid retention)
• Palpitations (fluttering in the chest due to abnormal heart rhythms)

Causes

Restrictive cardiomyopathy is not usually inherited and its cause is often unknown. Known causes include:

• Build-up of scar tissue (idiopathic is the most common cause)
• Build-up of abnormal proteins (amyloidosis) in the heart muscle
• Chemotherapy or chest exposure to radiation
• Excess iron (hemochromatosis) in the heart
• Other systemic diseases (sarcoidosis)

Treatment

The condition causing the cardiomyopathy is treated when it can be found.  Few treatments are known to work well for restrictive cardiomyopathy.  The main goal of treatment is to control symptoms and improve quality of life.
The following treatments may be used to control symptoms or prevent problems:

• Blood thinning medicines
• Chemotherapy (in some situations)
• Diuretics to remove fluid and help improve breathing
• Medicines to prevent or control abnormal heart rhythms
• Steroids or chemotherapy for some causes
• A heart transplant may be considered if the heart function is very poor and symptoms are severe.