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Pediatric Cardiomyopathy

Pediatric cardiomyopathy is a rare heart condition that affects infants and children. Specifically, cardiomyopathy means disease of the heart muscle (myocardium). Several different types of cardiomyopathy exist and the specific symptoms vary from case to case. In some cases, no symptoms may be present (asymptomatic); in many cases, cardiomyopathy is a progressive condition that may result in an impaired ability of the heart to pump blood; fatigue; heart block; irregular heartbeats (tachycardia); and, potentially, heart failure and sudden cardiac death.

Cardiomyopathy may be termed ischemic or nonischemic. Ischemic cardiomyopathy refers to cases that occur due to a lack of blood flow and oxygen (ischemia) to the heart. Such cases often result from hardening of the arteries (coronary artery disease). Nonischemic cardiomyopathy refers to cases that occur due to structural damage or malfunction of the heart muscle. Nearly all cases of pediatric cardiomyopathy are nonischemic. This report deals with nonischemic pediatric cardiomyopathy.

Cardiomyopathy may also be termed primary or secondary. Primary cardiomyopathy refers to cases where cardiomyopathy occurs by itself or for unknown reasons (idiopathic). Secondary cardiomyopathy refers to cases where the disease occurs secondary to a known cause such as heart muscle inflammation (myocarditis) caused by viral or bacterial infections; exposure to certain toxins such as heavy metals or excessive alcohol use; or certain disorders that affect the heart and/or additional organs systems. According to the Pediatric Cardiomyopathy Registry, approximately 79 percent of pediatric cardiomyopathy cases occur for unknown reasons (idiopathic).

Nonischemic cardiomyopathy may be further divided into four subtypes based upon the specific changes within the heart. These subtypes are: dilated, hypertrophic, restrictive and arrhythmogenic right ventricular dysplasia

Cardiomyopathy is a disease of the heart muscle. It affects people of all ages and is mostly inherited. It is not curable but can usually be treated successfully, with most of those affected going on to lead a long and full life.

There are several different types of cardiomyopathy three main types of cardiomyopathy – hypertrophic, dilated and arrhythmogenic right ventricular.

Early diagnosis

Increased awareness of the condition in recent years and recognition of subtle symptoms has helped early diagnosis and therefore early management. Without doubt, this can change the outcome of the condition and help to prevent complications. With the knowledge that the disease can be inherited, family screening has also contributed a great deal to early diagnosis. With knowledge of an existing or potential problem, lifestyle modifications and medication can be applied on time. Young people can be given appropriate advice when undergoing preventative screening and this is much better than treating an already severe condition.

Understanding of the condition

Understanding about the mechanisms and progression of heart dysfunction has significantly increased in the last few years. Studies have shown a number of mechanisms are activated when the heart function starts to go down and doctors are now trying to use these mechanisms in a beneficial way for the patient. In the context of a weak heart, the body’s reaction triggers compensatory mechanisms that can be helpful short term but damaging in the long term. Although it was previously thought that a weak heart needs medication that will make it beat more strongly, contemporary cardiology has been able to apply treatment that interferes with the more basic mechanisms.

An example of this is medication that prevents the heart condition from deteriorating further by keeping the heart rate and function mildly suppressed and under reasonable control. This is because studies have shown that the more you activate the failing heart, the worse it becomes in the long run. Whereas the more you protect the heart from its own compensatory reaction, the safer it is for the heart in the long term. So the philosophy in the management of heart dysfunction has changed over the years and is focusing on the prognosis and prevention of complications alongside the immediate reduction of symptoms.


Congenital Heart Disease

Congenital heart disease is a category of heart disease that includes abnormalities in cardiovascular structures that occur before birth. These defects occur while the fetus is developing in the uterus and may affect approximately 1 in 100 children. Congenital heart defects may produce symptoms at birth, during childhood, or not until adulthood. Other congenital defects may cause no symptoms.

What Causes Congenital Heart Disease?

The cause of congenital heart disease is unknown. However, there are some factors that are associated with an increased chance of having congenital heart disease. These risk factors include:
Genetic or chromosomal abnormalities in the child, such as Down syndrome
Taking certain medications or alcohol or drug abuse during pregnancy
Maternal viral infection, such as rubella (German measles) in the first trimester of pregnancy

The risk of having a child with congenital heart disease may double if a parent or a sibling has a congenital heart defect..

What Types of Congenital Heart Problems Are There?

The most common congenital heart problems include:
Heart valve defects. These can result in a narrowing or stenosis of the valves, or a complete closure that impedes or obstructs forward blood flow. Other valve defects include leaky valves that don’t close properly, thereby allowing blood to leak backwards.
Defects in the walls between the atria and ventricles of the heart (atrial and ventricular septal defects). Holes or passageways between the heart’s different chambers may allow abnormal mixing of oxygenated and unoxygenated blood between the right and left sides of the heart.
Heart muscle abnormalities that can lead to heart failure.

What Are the Symptoms of Congenital Heart Disease in Adults?

Congentital heart disease may be diagnosed before birth, right after birth, during childhood, or not until adulthood. It is possible to have a defect and no symptoms at all. In adults, if symptoms are present, they may include:
Shortness of breath
Limited ability to exercise

Congenital Heart Defects in Children

There are several congenital heart defects that are detected and treated early in infancy. Most of them are abnormal connections among the veins, as well as other arteries of the heart (aortic and pulmonary). These abnormal connections can allow unoxygenated blood to flow to the body instead of to the lungs, or allow oxygenated blood to flow to the lungs instead of to the body. They may also cause heart failure. Some examples of congenital heart disease in infants and children include:
Patent ductus arteriosus (when blood bypasses the lungs, preventing oxygen from circulating throughout the body)
Tetralogy of Fallot (four different heart defects that occur together)
Transposition of the great vessels (blood from the left side of the heart and right side of the heart intermix because the large artery connections are incorrect)
Coarctation of the aorta (a pinched aorta)
Heart valve problems

What Are the Symptoms of Congenital Heart Disease in Infants and Children?

The symptoms of congenital heart disease in infants and children include:
Cyanosis (a bluish tint to the skin, fingernails, and lips)
Fast breathing and poor feeding
Poor weight gain
Recurrent lung infections
Inability to exercise

How Are Congenital Heart Defects in Children Treated?

Some congenital heart defects will require surgery or an interventional procedure to repair the problem. Children with congenital heart disease may also need treatment with medication to improve heart function.
Children and adults with congenital heart disease should be treated by a cardiologist who specializes in congenital heart disease. Some types of disease may require a team approach as the child grows into an adult.


Atrial Fibrillation

Atrial fibrillation (say “A-tree-uhl fih-bruh-LAY-shun”) is an irregular heart rhythm (arrhythmia) that starts in the upper parts (atria) of the heart. Atrial fibrillation is the most common type of persistent irregular heartbeat (arrhythmia).

Normally, the heart beats in a strong, steady rhythm. In atrial fibrillation, a problem with the heart’s electrical system causes the atria to quiver, or fibrillate. The quivering upsets the normal rhythm between the atria and the lower parts (ventricles) of the heart. The lower parts may beat fast and without a regular rhythm.

Atrial fibrillation is dangerous because it greatly increases the risk of stroke. If the heart doesn’t beat strongly, blood can collect, or pool, in the atria. Pooled blood is more likely to form clots. If the heart pumps a clot into the bloodstream, the clot can travel to the brain and block blood flow, causing a stroke. Atrial fibrillation can also lead to heart failure.

What causes atrial fibrillation?

Conditions that damage or strain the heart commonly cause atrial fibrillation.
These include:

  • High blood pressure.
  • Coronary artery disease (CAD).
  • Heart attack.
  • Heart valve disease, especially diseases of the mitral valve.

What are the symptoms?

Symptoms may include:

  • Feeling dizzy or lightheaded.
  • Feeling out of breath.
  • Feeling weak and tired.
  • A feeling that the heart is fluttering, racing, or pounding (palpitations).
  • A feeling that the heart is beating unevenly.
  • Chest pain (angina).