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Cardiomyopathy in Pediatric Patients

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Cardiomyopathy in Pediatric Patients

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It is a rare chronic condition in children, where the muscles of the heart get thickened and enlarged, leading to failure of blood circulation to the heart.

Medically reviewed by

Dr. Rakesh Radheshyam Gupta

Published At July 20, 2022
Reviewed AtJuly 17, 2023

What Is Cardiomyopathy in Pediatric Patients?

Worldwide there are almost one in 100,000 children who suffer from cardiomyopathy, and usually, during the first two years of life, the chances of developing this condition are higher. It is a heterogeneous group of disorders leading to fatal conditions in children like heart failure. Even though medicine is very advanced these days, conditions like this still have a high mortality rate. Few of the cases show no symptoms, but some of them show symptoms like heartburn, rapid heartbeat, fatigue, and heart failure.

Cardiomyopathy is differentiated into ischemic and non-ischemic or primary and secondary conditions. An ischemic condition is a congenital abnormality of a coronary artery that causes a decrease in oxygen levels in the blood. Non-ischemic condition is when there is structural damage or functional defects of the heart muscle causing the symptoms like a heart attack. Also, primary cardiomyopathy is a heart failure caused by genetic disorders, infectious disease, and inflammation of the muscles of the heart. On the contrary, when there are secondary complications affecting other organs along with heart failure, it is called secondary cardiomyopathy.

What Are the Types of Cardiomyopathy in Pediatric Patients?

There are five forms of cardiomyopathy, such as:

  • Dilated Cardiomyopathy (DCM): It is a muscle disorder where the ventricle is too stretched to pump out the blood to the rest of the body. This type of cardiomyopathy usually occurs due to infections, complications in the last trimester of pregnancy, immunocompromised conditions, obesity, hypertension, arrhythmias (irregular rhythm of the heart), or heart valve diseases. The symptoms of the disease include fatigue, shortness of breath, edema of the ankles and belly, poor growth, chest discomfort, persistent cough, and rapid heartbeat. Also, there are some complications associated with dilated cardiomyopathy, such as leaky heart valves, heart failure, sudden cardiac arrest, and blood clots. The primary goal of treatment is to manage cardiac function in children. Additionally, medications like beta-blockers and ACE inhibitors can be prescribed according to the age and condition of the infant.
  • Hypertrophic Cardiomyopathy (HCM): It is a large heterogeneous group of disorders that rarely affects children. Even if it is very rare, its mortality rate is very high. The childhood HCM is caused by inherited metabolic defects, malformation syndrome, and neuromuscular and mitochondrial diseases. HCM is often present without any symptoms or signs in the body in the early stages, so a high suspicion index must be made in those patients. In most cases, it goes undiagnosed till the adulthood of the patient. Some symptoms of hypertrophic cardiomyopathy; are light-headedness, shortness of breath, chest pain, palpitations, excessive sweating, and difficulty feeding. Furthermore, sometimes taking a detailed family history, physical examination, or electrocardiogram can reveal the underlying condition. Drug therapy like beta-blockers and calcium channel blockers help to relax the cardiac muscles. Moreover, patients at high risk of cardiac arrest are prescribed implantable cardioverter-defibrillator and antiarrhythmic medicines.
  • Restrictive Cardiomyopathy (RCM): When cardiac muscles become stiff and filled with blood in a relaxation position, it causes a condition like restrictive cardiomyopathy. Due to this condition, there are symptoms like shortness of breath, persistent cough, abnormal heartbeat, poor growth, sweating, and fussiness seen in children. In almost 25 to 30 percent of cases, the underlying cause is a genetic factor, and other than that, excessive iron in the heart, systemic diseases, abnormal protein in heart muscles, or any inherited diseases can cause restrictive cardiomyopathy.
  • Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC): It is a rare condition where the heart muscle of the bottom right chamber- the right ventricle, is preoccupied with fat or fibrous tissue, and because of that, the heart is not able to pump the blood. Owing to this condition, there are symptoms like irregular heart rhythms, dizziness, fluttering of the chest, or sudden death due to heart failure. Even though there is no medications or treatment with 100 percent success, antiarrhythmic drugs can help to manage the symptoms.
  • Left Ventricular Non-Compaction Cardiomyopathy (LVNC): A heart muscle is made of sponge-like muscle fibers; however, with a condition like left ventricular non-compaction cardiomyopathy, the piece of muscle growth inside the left ventricle is called trabeculations. With the development of the heart muscle, trabeculations also developed, making the heart muscle smooth and solid. The symptoms of this disorder include dizziness, abnormal heartbeat, weight gain, shortness of breath, sweating, and poor feeding. Primarily the medication to manage the symptoms and prevent cardiac damage is given. Additionally, blood thinners like Aspirin are given along with beta-blockers.

What Are the Causes of Cardiomyopathy in Pediatric Patients?

Usually, the cause of cardiomyopathy is unknown, but research shows that genetic abnormality can trigger the condition in infants. The defective gene can be inherited from any one of the parents, or it can be a result of a mutation of genes. Moreover, recessive gene disorder occurs when both parents pass the same defective gene to their child. On the contrary, when a child has one abnormal gene inherited and one normal gene, it means that they remain a carrier of the disease but without any signs and symptoms and can pass it along. On the other hand, X-linked disorder is the result of non-working genes on the X-chromosome.

Dilated cardiomyopathy is the result of a generalized genetic disorder and muscular dystrophies. Most inherited genes responsible for dilated cardiomyopathy are either autosomal dominant traits, recessive or x-linked. Hypertrophic cardiomyopathy is the result of either a genetic mutation that is passed onto future generations as an autosomal dominant gene or there is no family history or reason behind the condition. When a patient already has systemic disorders such as sarcoidosis (collection of inflammatory cells causing tiny bumps) or hemochromatosis (extra absorption of iron from food), there are also chances of developing restrictive cardiomyopathy. Additionally, it can also be the result of post-operative open-heart surgery scarring or radiotherapy.

Apart from inherited factors, cardiomyopathy can be caused by a viral infection, toxins, chemotherapy, and metallic and mitochondrial diseases.

What Are the Symptoms of Cardiomyopathy in Pediatric Patients?

  • Shortness of breath.

  • Tachycardia - Increased blood pressure.

  • Dizziness.

  • Chest pain.

  • Fatigue.

  • Abdominal swelling.

  • Sweating.

How To Diagnose Cardiomyopathy In Pediatric Patients?

  • Family history (specifically, look for a genetic abnormality that runs in the family).

  • Physical examination.

  • Computed tomography.

  • Radiography of chest.

  • Electrocardiography.

  • Cardiac magnetic resonance imaging.

  • Radionuclide ventriculogram.

  • Cardiac catheterization.

How to Manage Cardiomyopathy in Pediatric Patients?

According to the type, severity, and age of the infant, the treatment plan proceeds. Although there is no cure for cardiomyopathy, their symptoms can be managed.

  • Medication: There are some drugs that treat the symptoms; for instance, arrhythmia can be treated with antiarrhythmic drugs, cardiac arrest can be treated with beta-blockers and ACE inhibitors, and for blood clots, Aspirin can be given with age-appropriate dosages.

  • An Implantable Cardioverter Defibrillator: It is used to treat the arrhythmia by regulating the heartbeat in children.

  • A Heart Transplant: In case of a severe condition, where all the medication and other treatments are ineffective, this mode of treatment is followed.

  • Myectomy: This type of surgical treatment is performed in the case of hypertrophic cardiomyopathy (muscle thickening).

Conclusion

Children with this disorder usually face many other complications throughout their life. However, with the support of medication and surgeries, this chronic condition can be managed. Moreover, the caregivers or parents should follow a few restrictions with regular follow-up visits to the hospital with restrictions on a diet, playing sports, or any other activity that requires strength.

Frequently Asked Questions

1.

What Is the Treatment of Cardiomyopathy in Children?

Cardiomyopathy is a rare and chronic condition characterized by the thickening and enlargement of the heart muscles leading to abnormal blood circulation to the heart. Medication alone can work in many pediatric cardiomyopathy cases. Some might require surgery for the improvement of valve functions. A heart transplant can be indicated in severe cases of cardiomyopathy.

2.

What Is the Primary Treatment of Cardiomyopathy?

Cardiomyopathy is a rare heart condition in which the heart muscles are thickened and enlarged. The person encounters shortness of breath, fatigue, and heart palpitations. The treatment for cardiomyopathy depends upon the severity and nature of the condition. The primary treatment includes medications such as anti-arrhythmic drugs, ACE inhibitors, or beta-blockers.

3.

Which Medication Is Prescribed for Pediatric Cardiomyopathy?

The doctors prescribe the treatment option for cardiomyopathy in children according to the age of the infant, and the type and severity of the condition. The medication is prescribed based on the symptoms the child is encountering. So, anti-arrhythmic drugs for abnormal heart rhythms can be given. In addition, Beta-blockers and ACE inhibitors can be prescribed for cardiac events. In age-appropriate form, blood thinners such as Aspirin can be given.

4.

What Are the Four Primary Signs of Cardiomyopathy?

Cardiomyopathy is a disorder of the muscles of the heart that makes it a problem for the heart to pump enough blood. So, it disrupts blood circulation, leading to heart failure. The common signs of cardiomyopathy include the following.
- Shortness of breath with activity or at rest.
- Fatigue.
- Heart palpitations.
- Swelling of the ankles, feet, and legs.
- Chest discomfort or pressure. 

5.

How Long Can a Child With Cardiomyopathy Live?

Pediatric cardiomyopathies are considered rare but severe heart conditions occurring with the problem of the muscles of the heart. It affects about one in 100,000 pediatric population in the USA. The clinical findings show that about 40% of children with cardiomyopathy in symptomatic form have heart transplantation surgeries. Also, cardiomyopathy in children dies within two years from such cardiac complications, in otherwise a long span.

6.

What Age Is Critical for the Diagnosis of Cardiomyopathy?

Cardiomyopathy is a serious heart condition that can occur at any age. It can be acquired or hereditary. Nevertheless, most people get the diagnosis in middle age. It can be diagnosed right from birth to more than eighty years. There prevail even new diagnoses of cardiomyopathy in the ninety ages.

7.

Which Is the Latest Treatment Option for Cardiomyopathy?

The treatment of cardiomyopathies is more about addressing the heart condition and the symptoms of the condition. It can be medication, septal myectomy, or a heart transplant. The new and first disease-specific treatment is Mavacamten, a new drug class for the treatment of hypertrophic cardiomyopathy. The drug also received Food and drug administration (FDA) approval.

8.

How Can One Naturally Get Rid of Cardiomyopathy?

Cardiomyopathy is a rare yet serious heart disease that requires appropriate and effective means of intervention. But, still, certain natural ways can help reduce the effects of cardiomyopathies. The effective natural ways include the following.
- Reduce excess weight.
- Quit smoking.
- Avoid taking alcohol and other substance.
- Reduce stress.
- Get an adequate amount of sleep.
- Physical exercises.
- Eat greens.
- Address and treat underlying conditions such as hypertension and diabetes. 

9.

Which Treatment Is Considered the Alternative Option for Cardiomyopathy?

The alternative means of treatment of cardiomyopathies and heart-related concerns include the following.
- Mind-body interventions such as meditation, hypnotherapy, yoga, tai chi, and biofeedback.
- Biological-related treatment includes the supplements such as vitamins, herbals, and minerals.
- Manipulative therapies such as chiropractic or massage therapy.
- Magnetic therapy. 

10.

How to Confirm the Condition of Cardiomyopathy?

The diagnosis of cardiomyopathy in children and adults relies on physical examination, family history, computed tomography (CT) scan, electrocardiography, cardiac catheterization, magnetic resonance imaging (MRI), and radionucleotide ventriculogram. Moreover, the heart or myocardial biopsy is used to confirm the diagnosis o cardiomyopathy.

11.

Can Cardiomyopathy Condition Be Stopped?

Cardiomyopathy is a disorder of the heart muscles preventing healthy or normal blood circulation. Cardiomyopathy can be hereditary or acquired, occurring at any age. The inherited or genetic forms of cardiomyopathy cannot be stooped or prevented. But certain lifestyle changes and effective interventions help prevent the risk and complications occurring with cardiomyopathy.

12.

Can One Recover From Cardiomyopathy?

Cardiomyopathy is a rare yet severe heart condition. Many people with cardiomyopathy do not encounter major problems, but some people do experience cardiovascular events and even heart failure. Cardiomyopathy acquired from alcohol last only a few months and it can be reversed with the help of chemotherapy. In contrast, the other types of cardiomyopathy require a long span of treatment.

13.

Can Cardiomyopathy Be a Temporary Condition?

Many forms of cardiomyopathy are long-term, leading to heart complications. Nevertheless, Takutsubo cardiomyopathy is a temporary or short-term form of a heart condition. Physical stress or severe degrees of emotional stress induce this type of cardiomyopathy. The symptoms seem similar to a heart attack with shortness of breath and chest pain.

14.

Can One Lead a Normal Life With Cardiomyopathy?

Though cardiomyopathy is a serious heart condition that can cause heart failure in chronic cases, many people can lead long and normal life with appropriate care and attention. The coping strategies include medications, lifestyle support, implantable devices, procedures, and surgeries.
Dr. Rakesh Radheshyam Gupta
Dr. Rakesh Radheshyam Gupta

Child Health

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