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Restrictive Cardiomyopathy: Research Paper

Alyssa Vincent

Dixie State University

Nurs 3820: Nursing Care Across the Lifespan II

Professor Dean Brereton

March 11, 2022

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Restrictive Cardiomyopathy: Research Paper

Cardiomyopathies are a group of diseases of the myocardium related to an electrical or

mechanical dysfunction. They either show hypertrophy or dilation of the ventricles, and are

classified as genetic, nongenetic, or both. This paper will go over restrictive cardiomyopathy

which is classified in the mixed (genetic and nongenetic) category. Restrictive cardiomyopathy is

a “… rare heart muscle disease in which ventricular filling is restricted because of excessive

rigidity of ventricular walls,” (Norris et al., 2020). Signs and symptoms can be difficult to

pinpoint, and it is often mistaken for constrictive pericarditis or respiratory related diseases.

There are several different causes of this disease ranging from genetic to acquired. Treatment of

this disease can be difficult and extensive.

Restrictive cardiomyopathy can be caused by either genetic or acquired factors.

Researchers have discovered that, “More than 60 genes have been described to cause inherited

cardiomyopathy,” (Lu et al., 2018). However, genetic screening for this disease can be, “labor-

intensive and expensive,” (Lu et al., 2018). Since diagnosis of the disease is difficult it causes

prevention and teaching about the disease to be delayed. A common acquired cause of restrictive

cardiomyopathy is amyloidosis. Amyloidosis is a buildup of protein in the heart. Exposure to

radiation like chemotherapy can also cause this disease. Sarcoidosis is a disease that causes the

heart to scare, and this can increase the hardening of the ventricles of the heart.

Hemochromatosis can be another cause where there is an abnormal increased amount of iron in

the heart.

There is no one sure way to prevent restrictive cardiomyopathy especially when it is

genetic. The best ways would be to follow up and get regular checkups with a doctor, lifestyle
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changes and taking medications that help with blood flow. As long as the disease is diagnosed

early on, there are ways to help with complications.

Signs and symptoms are often difficult to pinpoint. They are commonly mistaken for

respiratory issues. Many patients with restrictive cardiomyopathy may have, “… recurrent upper

respiratory infections; dyspnea on exertion; or more ominously, may present with palpitations,

syncope, or sudden death,” (ZANGWILL & HAMILTON, 2009). Clinical manifestations are

often related to the diastolic dysfunction. Diastolic dysfunction is when the heart is unable to

relax. This can lead to hypertension, pulmonary edema, and decreased myocardial reserve. The

heart may present as tachycardic and dysrhythmic. This tachycardia can be a major issue due to

the underlying disease and its progression. It can cause thromboembolic disease, worsened

cardiac output, and sudden death. Blood clots in itself are extremely dangerous. This is why it is

extremely important that this disease is diagnosed early.

Diagnosis of restrictive cardiomyopathy is very difficult and can take a long time. Many

of the diagnostic testing is to rule out other possible diseases. The main condition health care

providers are looking for is diastolic dysfunction. “Echocardiography-based 2-dimensional and

Doppler are essential for determining diastolic dysfunction…” (Muchtar et al., 2017).

Echocardiograms are good for ruling out constrictive pericarditis and cardiac sarcoidosis. They

show how the heart is pumping and functioning, which is extremely important when looking for

diastolic dysfunction. Electrocardiograms are used to check the heart rhythms and chest x-rays

can look at the size of the heart. Cardiac magnetic resonance can also help but its not always

used. They can also use an endomyocardial biopsy.

Many times, the diagnosis of restrictive cardiomyopathy is in the late stage of the disease.

Restrictive cardiomyopathy happens when the chambers in the heart stiffen. The heart is able to
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squeeze but it cannot relax causing the heart difficulty filling with blood. This causes the body to

build up fluid often backing up into the lungs. The prognosis of restrictive cardiomyopathy often

depends on when the diagnosis is made.

Restrictive cardiomyopathy can be diagnosed anywhere from childhood to adulthood.

Often children’s first signs are failure to thrive. Failure to thrive is when children’s, “… weight

or rate of weight gain is significantly below that of other children of similar age and sex,” (Johns

Hopkins Medicine, Failure to thrive). Children with failure to thrive may have delayed physical

abilities, and mental and social skills. They also are extremely tired and have syncope. In severe

cases children can have edema, ascites, increased blood pressure, and liver problems. Some

children never show any symptoms and are at risk of cardiac arrest. This is why it is important to

diagnosis restrictive cardiomyopathy early (Medlineplus, 2020).

There is usually no treatment available for patients. The main treatment of restrictive

cardiomyopathy is to stop the disease from getting worse. Health care professionals want to

control the underlying factors causing the disease and decrease complications like cardiac arrest.

They can be given supportive care, but the biggest and most effective thing would be a heart

transplant. Medications given are to treat symptoms. “Diuretics may be useful to treat symptoms

related to pulmonary edema with the understanding that aggressive volume depletion may be

counterproductive,” (Lu et al., 2018). Beta blockers are also given. Many patients are given

implanted defibrillators.

Restrictive cardiomyopathy is a dangerous disease and there are several complications

that can come from it. Heart failure and sudden cardiac arrest are two of the biggest

complications. There is also a possibility of a blood clot forming in the heart. It can make its way

to the brain and cause a stroke. Abnormal heart rhythms are often inevitable with restrictive
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cardiomyopathy. There are several complications that can occur, and lifestyle changes can help

prevent them (Restrictive cardiomyopathy 2021).

Lifestyle changes are usually a big part in treating or preventing restrictive

cardiomyopathy. The best things are a healthy diet and physical activity. A healthy diet includes

fruits, vegetables, and whole grains. People should also watch for fats, sodium, and sugar. The

DASH diet (dietary approaches to stop hypertension) has been shown to help with blood

pressure. It has also been shown to avoid alcoholic beverages or drink in moderation. Other

major lifestyle changes include stop smoking, lose weight, get adequate rest, and reduce stress

(Prevention and treatment of cardiomyopathy 2022). Anything that can help with improving

cardiac function is encouraged.

When it comes to patient teaching as a nurse it is important to constantly remind patients

with restrictive cardiomyopathy about things they should and should not do with this disease.

Patients should restrict the amount of salt they have in their diet. Heart issues keep fluid retained

in the body causing edema in the extremities or crackles in the lungs. Sodium also retains fluid

so it is important to not do anything that would keep more fluid than the body already has.

Patients should do exercises their health care provider has approved of. They also need to know

the signs of worsening perfusion issues like, chest pain, excessive swelling, fainting, or shortness

of breath (Carle, Managing your restrictive cardiomyopathy – symptoms & treatment).

There are several types of cardiomyopathies. There is dilated, hypertrophic, and

restrictive. Dilated cardiomyopathy is when the chambers of the heart increase in size,

weakening the walls of the heart. Hypertrophic cardiomyopathy is when the heart muscle

becomes thick. Overall restrictive cardiomyopathy is a dangerous disease. It can lead to sudden

cardiac arrest and heart failure. The only sure way to treat it is by getting a heart transplant. Until
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then, health care professionals treat the symptoms and potential complications of restrictive

cardiomyopathy. Prognosis is not good for patients and this disease usually requires a heart

transplant.
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References

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Lu, C., Wu, W., Liu, F., Yang, K., Li, J., Liu, Y., Wang, R., Si, N., Gao, P., Liu, Y., Zhang, S., &

Zhang, X. (2018). Molecular analysis of inherited cardiomyopathy using Next Generation

Semiconductor Sequencing Technologies. Journal of Translational Medicine, 16(1).

https://doi.org/10.1186/s12967-018-1605-5

Managing your restrictive cardiomyopathy – symptoms & treatment. Managing Your Restrictive

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March 11, 2022, from https://www.heart.org/en/health-

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Manuals Professional Edition. Retrieved March 11, 2022, from

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