Congenital heart diseases represent spectrum of diseases which are present
since birth although may manifest later in life. Congenital heart disease is
usually suspected in children who have recurrent chest infections, heart failure,
failure to gain weight etc. Any deviation from the normal development of heart
while in womb, results in the congenital heart disease.
What are various types of congenital heart diseases?
Congenital heart diseases broadly speaking could be described as those, which
cause mixing of pure (oxygenated) and impure (deoxygenated) blood (examples
being atrial septal defects, ventricular septal defects, patent ductus arteriosus
etc), those associated with obstruction to flow of blood from heart (aortic
stenosis, coarctation of aorta etc) and those associated with cyanosis (bluish
discoloration).
The cause of congenital heart disease is largely unknown. About 8 in 1000 newborns
will be born with some type of congenital heart disease. Most newborns with
congenital heart disease now live into adulthood. Medical and surgical treatments
have greatly improved the long-term outlook and quality of life for people born
with a congenital heart defect.
Very few congenital heart problems are ever considered to be completely "cured."
In some cases, surgery may not be possible or not yet needed. Lifelong medical
follow-up is very important for most adults with a congenital heart defect.
The purpose of follow-up is to prevent any possible problems from developing.
Some congenital heart defects may not be found until adulthood. Further tests
are often needed to determine the type of treatment and whether surgery is needed.
Experience has shown that problems related to a heart condition in childhood
are not the same as problems that arise in adulthood. Health care for adults
with congenital heart disease has become a specialty. An adult with a congenital
heart defect needs to see a heart doctor (cardiologist) who cares for patients
with congenital heart problems.
Lifestyle guidelines for patients with Congenital Heart Disease
Below is a listing of common concerns and recommendations that apply to most
people with a congenital heart defect.
- Bacterial endocarditis. A heart defect places a person at greater
risk for developing an infection of the inside lining of the heart and of
the heart valves. When a person has dental work or certain surgeries, bacteria
or germs that can cause a heart infection (endocarditis) may get into the
blood and be carried to the heart. Special prevention guidelines called bacterial
endocarditis prophylaxis should be followed by patients with heart defects
both before and after surgery. To prevent a heart infection, antibiotics should
be taken 1 hour before dental work or other surgeries that might allow bacteria
to get into the blood and again 6 hours later. It is also important to maintain
good dental hygiene and carry a special wallet card printed by the American
Heart Association that indicates when antibiotics are needed and the type
and amount that should be given. You can get this card from your cardiologist.
- Diet, alcohol, smoking, and high blood pressure. All adults should
control the amount of fat in their diets to help prevent coronary artery disease.
Fewer than 30% of the calories eaten should be from fats; food labels provide
helpful information. Patients with heart failure or high blood pressure need
to limit the amount of salt in their diet. Being overweight causes an increased
workload on the heart. Excess alcohol intake weakens the heart muscle. Smoking
is particularly harmful to the heart because smoking limits the amount of
oxygen available. Smoking also damages the coronary arteries and may lead
to a heart attack. High blood pressure increases the workload of the heart
and damages the coronary arteries. High pressure does not make a person feel
sick. If your doctor has told you that you have high blood pressure, be sure
to have your blood pressured checked periodically and take your medication.
- Activity. Most patients with congenital heart disease are able to
do all normal activities and can participate in most recreational sports.
Some type of physical exercise is important to maintain good health. Exercise
helps prevent coronary artery disease and heart attacks. Use common sense
when exercising; do not push yourself beyond fatigue or uncomfortable shortness
of breath. Some patients may be advised to avoid isometric exercises, which
are heavy push-pull exercises that rapidly raise the blood pressure. In some
cases, patients may need to avoid certain jobs or careers and team sports
that require excessive physical endurance. Talk with your cardiologist about
recommended activity guidelines for your case.
- Family planning and pregnancy. Patients often ask whether their heart
defect can be passed on to their child. Although the risk is higher than for
the general population, the chances of a child having a congenital heart defect
are generally less than 4%. The question of whether a woman with congenital
heart disease should become pregnant can only be answered by the individual.
If the woman is not experiencing any symptoms from her heart defect, she should
be able to carry a pregnancy without problems. Consult your cardiologist before
becoming pregnant and before taking medications for birth control.
- Insurance. Congenital heart disease is considered a pre-existing
condition. Insurance companies may deny coverage altogether or deny coverage
related to disorders of the heart. Often they offer insurance at a higher
rate or require a waiting period without treatment before medical care relating
to the defect can be covered.
Cyanotic Congenital Heart Disease
Cyanosis is a blue discoloration of the skin that occurs when the blood
is not completely filled with oxygen. This change in color is especially noticeable
around the lips, the tips of the fingers and toes, and the outer rims of the
ears.
Cyanotic congenital heart disease refers to heart defects that allow oxygen-poor
blood to mix with oxygen-rich blood. When this happens, some of the oxygen-poor
blood is pumped out to the heart before the blood gets to the lungs. Too much
oxygen-poor blood in the body causes the skin to look bluish or dusky.
Symptoms of cyanosis
Cyanosis results in a decreased supply of oxygen for the body. People with
cyanosis tire more easily with physical activity. During physical exertion,
the amount of oxygen being used by the body increases, causing the degree of
cyanosis to increase too. Cyanotic patients may also develop a rounded and thickened
appearance of their nails called clubbing.
When a person has cyanosis for a prolonged time, the body will compensate by
making more red blood cells to carry more oxygen. The body may overproduce red
blood cells, making the blood too concentrated. Although uncommon, this can
produce headaches, eyesight changes, prolonged bleeding, muscle aches, joint
pains, and marked fatigue. These cyanotic patients need to have periodic blood
tests. A blood test called a hematocrit measures the percentage, or amount,
of red blood cells.
Blood clotting is also impaired by an increase in the amount of red blood cells.
Patients with cyanotic heart disease tend to bruise and bleed more easily. Although
rare, patients may cough up blood. As a result, special precautions are needed
with certain medications, surgical procedures, and injuries.
How is cyanosis measured?
Oxygen can be measured as a percentage of red blood cells that are saturated
with oxygen. Oxygen saturation is usually measured by a painless instrument
called a pulse oximeter. This instrument is placed on the tip of a finger; it
uses light to detect the amount of oxygen in the red blood cells. Occasionally,
blood must be taken from an artery to directly measure the amount of oxygen
in the blood. The red blood cells are normally more than 93% saturated with
oxygen. A person usually appears cyanotic when the red blood cells are less
than 87% saturated with oxygen.
Living with cyanosis
- Surgical treatment. In most cases, cyanosis is corrected by surgical
repair the heart defect. However, it is not always possible to completely
repair some defects. Alternative surgical procedures call shunts are sometimes
used to direct more blood into the lungs and decrease the degree of cyanosis.
- Oxygen. Some people think that cyanosis will go away by breathing
more oxygen. However, additional oxygen may only slightly improve the degree
of cyanosis caused by congenital heart disease and in most cases is not recommended.
Some patients, however, may need additional oxygen during air travel. Higher
altitudes should usually be avoided. Ask you heart doctor or nurse specialist
for specific travel and altitude guidelines.
- Activity. Patients with cyanosis often express frustration in not
being able to perform all desired activities. Patients should try to pace
themselves and alternate easy and hard activities. They should allow themselves
extra time to walk somewhere or to climb stairs. They should try not to put
themselves in positions that require extreme or prolonged physical demand.
Heart doctors or nurse specialists can provide individual vocational guides
that are tailored to the patient's needs. Activities should be limited during
hot, smoggy days. Extremely cold temperatures and extremely hot showers, baths,
or hot tubs should be avoided. Hot water will lower blood pressure and cause
an increased amount of oxygen-poor blood to flow out into the body. Patients
should not be hard on themselves. They should find an enjoyable hobby or outlet
and focus on the things they can do.
- Medications. Aspirin is a common medication that slows clotting and
should not be taken by cyanotic patients. Many cold and flu medications contain
aspirin; their labels should be read carefully. The heart doctor should be
consulted before anti-inflammatory medications such as ibuprofen are taken.
Iron or vitamin supplements containing iron should not be taken unless advised
to do so by the heart doctor. Iron supplements may cause the number of red
blood cells to become too high.
- Smoking. Do not smoke; avoid second-hand smoke. Smoke from cigarettes
binds to the red blood cells and limits their ability to carry oxygen.
- Medical follow-up and special precautions. Patients should keep their
medical and lab appointments. Patients should always let their heart doctor
know when they plan to have an operation or a special medical procedure. Cyanotic
patients are more sensitive to dehydration. They should drink plenty of fluids,
especially in hot weather or times of fever, diarrhea, or vomiting. If blood
is coughed up, the patient should remain calm, rest in bed, and call the heart
doctor. Patients should know their normal hematocrit level.
Birth control.
Because of the low amount of oxygen in the blood, women with cyanosis should
not become pregnant. Birth control pills generally are not recommended for women
with cyanosis. Patients can consult the heart doctor or nurse specialist about
other types of birth control.
Diagnosis of congenital heart diseases?
Several tests can show what kind of heart disease baby/adult has. Here are some
tests
- ECG (short for "electrocardiogram")- An ECG is a drawing of the
baby's heart beat. It shows how well the heart is working.
- Pulse oximetry-This test shows how much oxygen is in the baby's blood.
- Echocardiogram-This test gives the doctor an ultrasound "picture"
of the baby's heart.
- Chest x-ray-This can show how well the heart is growing and if your baby's
lungs have fluid in them.
- Cardiac catheterization-This test puts dye in the heart to give the doctor
a clear picture of the heart problem.
