Congenital heart disease is a heart condition you are born with. The word congenital means “present at birth.” Congenital heart disease can range from very minor conditions which never cause problems, to more serious conditions that require treatment.
A congenital heart defect happens when the chambers, walls or valves of your heart – or the blood vessels near the heart – don’t develop normally before birth. There are many different types of defects listed below.
1. Holes in the heart (septal defects)
When a baby is born with an abnormal opening in the wall that separates the right and left chambers of the heart (the septum), blood can leak between the chambers instead of flowing normally to the rest of the body. This may cause the heart to become enlarged.
The most common holes in the heart are:
Patent foramen ovale (PFO) is one type of atrial septal defect. The hole between the left and right atria usually closes within the first few years of life. Even if it doesn’t close, the hole may not cause any complications unless you have a second heart defect. PFOs are very common and many people will never know they have one.
2. Obstruction of blood flow
Stenosis is a narrowing or obstruction in heart valves, arteries or veins that affects the flow of blood. Atresia is when a passageway in the body is abnormally shut or has not formed properly. Different types of stenosis and atresia can partly or completely block blood flow in the heart.
Medication in the first few weeks of life can either close (or keep open) the ductus arteriosus. As your baby gets older and if the medication isn’t working, the ductus can be closed off using a cardiac catheterization procedure. This will restore normal circulation.
Other defects between the right and left sides of the heart often co-exist with transposition of the great arteries. An atrial septal defect, ventricular septal defect or ductus arteriosus can actually help oxygenated blood circulate to the body.
Children with this condition often have an atrial septal defect as well (see above).
Kawasaki disease causes inflammation or swelling of the blood vessels. Kawasaki disease can affect any medium-sized artery in the body but primarily affects the coronary arteries. The coronary arteries are special blood vessels that carry blood and oxygen into the heart muscle. If there is a problem with the coronary arteries, the heart will not get enough blood and oxygen, making it unable to work properly.
An inflammatory disease that, among other things, affects blood vessels in the body, particularly the coronary arteries.
The diagnosis of Kawasaki disease is made when a child has at least five consecutive days of fever and at least four out of the other five symptoms mentioned above. In some cases, a child will have fewer than four symptoms. Kawasaki disease often mimics other diseases such as common childhood infections. These factors make the diagnosis of Kawasaki disease more difficult.
Kawasaki disease is a rare illness. It usually affects children under the age of five, but older children can also be affected.
Information taken from: https://www.aboutkidshealth.ca/Article?contentid=915&language=English
Pompe disease is a rare, inherited neuromuscular disorder that causes progressive muscle weakness and loss of muscle tissue.
Pompe disease goes by many different names:
Pompe disease can occur between infancy and adulthood, and affects both men and women equally. Approximately one-third of people with Pompe disease are infants (infantile-onset) while the other two thirds are children or adults (late-onset).
Pompe disease is caused by mutations in a gene that makes an enzyme called acid alpha-glucosidase (GAA). The job of this enzyme is to break down glycogen, a form of sugar stored in muscle cells throughout the body. In people with Pompe disease, this enzyme is either missing or in short supply.
The symptoms and severity of Pompe disease can vary widely from person to person. Symptoms associated with the infantile-onset form may include:
Symptoms associated with the late-onset form may include:
Enzyme replacement therapy (ERT) has the ability to treat the underlying cause of the disease. Though ERT is not a cure, providing the missing enzyme may slow the progression of muscle weakness and improve muscle function.
Information taken from; http://muscle.ca/discover-md/types-of-neuromuscular-disorders/
What is valvular heart disease?
The heart has four chambers. The two upper chambers are called the left and right atrium, and the two lower chambers are called the left and right ventricle. The four valves at the exit of each chamber maintain one-way continuous flow of blood through the heart to the lungs and the rest of the body.
The four valves are the tricuspid valve, pulmonary valve, mitral valve and aortic valve.
In between each step, the valve closes to prevent blood from flowing backwards and mixing oxygen-poor blood with oxygen-rich blood. The one-way continuous flow of blood delivers oxygen throughout your body.
Heart valve disease occurs when one or more of the heart valves do not open or close properly. When it affects more than one heart valve, it is called multiple valvular heart disease.
Heart valve disease can be classified as mild, moderate or severe. It can lead to an enlarged heart or heart failure. Heart failure is a serious medical condition where the heart cannot pump enough blood to meet the body’s need for oxygen.
Many valvular heart diseases can be treated with medication, or surgery and other procedures to repair or replace the valve.
The four valves are the tricuspid valve, pulmonary valve, mitral valve and aortic valve.
In between each step, the valve closes to prevent blood from flowing backwards and mixing oxygen-poor blood with oxygen-rich blood. The one-way continuous flow of blood delivers oxygen throughout your body.
Heart valve disease occurs when one or more of the heart valves do not open or close properly. When it affects more than one heart valve, it is called multiple valvular heart disease.
Heart valve disease can be classified as mild, moderate or severe. It can lead to an enlarged heart or heart failure. Heart failure is a serious medical condition where the heart cannot pump enough blood to meet the body’s need for oxygen.
Many valvular heart diseases can be treated with medication, or surgery and other procedures to repair or replace the valve.
Information taken from https://www.heartandstroke.ca/heart/conditions/valvular-heart-disease
Why is a tilt-table exam done?Tilt tests are especially useful if you have been fainting without any explanation. These tests help doctors understand how your body posture affects your blood pressure. The goal is to find out if different drugs or different body positions will trigger an arrhythmia (abnormal heartbeat) or symptoms.
Information take from https://www.heartandstroke.ca/heart/tests/tilt-table-exam
Click HERE to learn more about a tilt table test/exam
What is an Echocardiogram?
Echo is the short way of saying echocardiogram. An echo is another test that lets doctors see inside your body. The echo is special since it only takes pictures of your heart. It can show the shape and size of your heart. Just like the name, the echo uses sound waves to take the pictures; it bounces invisible sound waves off of your heart to create a picture of what it looks like. You will not feel or hear these sound waves in your body.
Why do I need to have an Echo?
The doctor needs to see a picture of your heart and the best way to take the picture is to use the echo machine. A regular X-Ray cannot see the heart, so an echo has to be used.
What does an Echo look like?
An echo machine looks like a computer. The technologist (the person taking the picture) uses a smooth flat wand and a special gel to take the picture. The gel and wand lets the computer hear the sound waves from your heart. Once the pictures have been taken, they are printed and shown to the radiologist. He is a special doctor who looks at the pictures.
What happens when I have an Echo?
A porter will come to bring you to the special room to have the echo. A technologist (the person who will take the pictures) will greet you and explain what will happen.
The technologist will give you a gown to wear, this looks like a backward housecoat, you may already be wearing one. You will lie down on a bed beside the computer. The technologist usually turns the lights down so that they can see the pictures on the computer better. Next the technologist will put gel on your chest over the area where your heart is. He or she will move the smooth, flat wand on your skin where the gel is. The wand is attached to the computer. While the technologist moves the wand over your chest, you will be able to see a wavy and fuzzy image on the screen. While the technologist is moving the wand, he or she will also be touching buttons on the computer keyboard.
It is important to remember to stay still, if you move a lot, the picture will not be clear and the test will take longer. The test is usually short and takes about 15-30 minutes. Once the pictures are done, they will be printed off to show the doctor. The technologist will wipe the gel off with a soft towel and then you will be able to go back to your room.
What will the Echo feel like?
The echo will not hurt. The gel may feel a little cool and sticky. The wand may press down onto your skin a little bit, but it will not hurt. Remember to lie still so that the technologist can get a clear picture. Thinking about your favourite thing to do may help you stay still.
Preparing for the test
Your nurse will give you the information you need to help you get ready to have your echo. Sometimes you may have to do nothing to get ready.
Remember
If you have any questions about the test, always ask!
Valvular Stenosis
Anatomy of the Heart
The heart has four chambers. The two upper chambers are called the left and right atriums, and the two lower chambers are called the left and right ventricles. There is a valve at the exit of each chamber that ensures one-way continuous flow of blood through the heart.
The four valves are the tricuspid valve, pulmonary valve, mitral valve and aortic valve. These valves open and close to prevent blood from flowing backwards.
Stenosis
Stenosis is when the valve opening becomes narrow and restricts blood flow.
Causes
Valvular heart disease can develop before or at birth (congenital causes) or normal valves may become damaged during one’s lifetime (acquired causes). The cause of valvular heart disease is not always known.
Symptoms
Many people do not notice any symptoms until their blood flow has been significantly reduced by valvular heart disease. Symptoms can include:
If you don’t have many symptoms or if they are mild and not affecting you too much, your doctor may choose to monitor your condition carefully and wait until it is necessary to treat your symptoms. It is important to understand that the symptoms of valvular heart disease may not necessarily reflect the seriousness of the problem. Regular check-ups are recommended.
Treatment
Treatment will depend on the severity of your disease! If it’s minor, you may not need treatment at all. You and your doctor will discuss your options based on your condition.
Options include: