|
 |
|
|
|
|
|
cyanotic heart disease Images (Click to view larger image)
Alternative Names
congenital cyanotic heart disease
Definition
Cyanotic heart disease refers to certain kinds of birth defects, or congenital heart disease, that affect the heart. Cyanotic heart disease causes cyanosis, or a blue skin colour.
What is going on in the body?
Cyanosis usually means there is a severe lack of oxygen in the blood. Certain birth defects in the heart can produce cyanosis, and there can be other causes. Cyanosis caused by birth defects often shows up at birth, or shortly afterward. The low oxygen levels in the blood can lead to organ failure and death in severe cases.
What are the signs and symptoms of the disease?
Symptoms of cyanotic heart disease can vary, depending on the underlying congenital heart disease. All children with cyanotic heart disease have constant or intermittent bluish discolouration of the skin, known as cyanosis. Other symptoms may include: - abnormal breathing, either rapid or periodically absent, or with flaring of the nostrils
- fatigue
- decreased movement and response to stimulation
- failure to thrive, or failure to grow and develop properly
Later in childhood, exercise may worsen symptoms and bring about "spells." These can result in anxiety, laboured breathing, and worsening cyanosis.
On examination, a doctor may notice: - clubbed fingertips, whose altered shape is caused by prolonged cyanosis
- cold limbs
- abnormal or absent pulses
- abnormal heart sounds, or heart murmurs
- abnormal chest wall movements
- difficulty breathing, or shortness of breath
What are the causes and risks of the disease?
Several congenital heart defects can cause cyanosis. Some defects prevent enough blood from getting to the lungs, where oxygen is normally put into the bloodstream. Others allow blood to get to the lungs, but the oxygenated blood is then prevented from being pumped into the body's regular circulation.
Two examples of heart defects that can cause cyanosis are: What can be done to prevent the disease?
Good antenatal care is an important way to help prevent cyanotic heart disease. This includes regular checkups, proper vaccinations, and avoidance of alcohol. Many times nothing can be done to prevent cyanotic heart disease.
How is the disease diagnosed?
The physical examination often makes the doctor suspect cyanotic heart disease. Blood tests, chest x-rays, a heart tracing or ECG, and other imaging tests may be done. These tests can both confirm the diagnosis and determine the type of heart defect.
A test called an echocardiogram uses ultrasound waves to look at the heart while it is beating. It shows its size, structure, shape, blood flow, and motion.
Cardiac catheterisation can also be used to examine the heart defect. In this test, a tube, or catheter, is inserted through the skin and into an artery, usually in the groin. A contrast agent is then injected through the tube. Pictures can then be taken of the heart as the contrast agent is pumped through it. This allows defects to be seen clearly.
What are the long-term effects of the disease?
If left untreated, severe cyanotic heart disease can lead to permanent organ damage or death.
What are the risks to others?
Cyanotic heart disease poses no risk to others. Parents who have had one child with a heart defect that causes cyanosis have a slightly increased risk of having a second affected child. Genetic counselling can be useful in this case.
What are the treatments for the disease?
Treatment varies, depending on the underlying defect that causes the cyanotic heart disease. Open heart surgery is often needed to fix the heart defect. In some cases, emergency surgery right after birth may be the only hope of survival. In other cases, surgery may be delayed for a year or more after birth. Oxygen can be given to help improve symptoms. The use of heart medications and other medications depends on the underlying heart defect.
What are the side effects of the treatments?
Surgery carries a risk of bleeding, infection, and allergic reaction to anaesthesia. All medications can have side effects. Heart medications may cause allergic reactions, stomach upset, or irregular heartbeats.
What happens after treatment for the disease?
If the cyanotic heart disease can be treated with surgery or medications, the outcome can be very good. More severe heart defects require lifelong treatment and monitoring.
How is the disease monitored?
Follow-up care is based on the underlying heart defect. Routine visits to the doctor are made to check on status of the heart, the healing of the heart after surgery, and to adjust medication if needed. The child's growth and development will be carefully monitored. Any new or worsening symptoms should be reported to the doctor.
Author:
Reviewer: HealthAnswers Australia Medical Review Panel
Editor: Dr David Taylor, Chief Medical Officer HealthAnswers Australia
Last Updated: 1/10/2001
Contributors
Potential conflict of interest information for reviewers available on request |
|
|
| |
|
|
|
|
|
|
|
|
